Avoidable Medical Mishaps - Society to Improve Diagnosis in
Transcription
Avoidable Medical Mishaps - Society to Improve Diagnosis in
Avoidable Medical Mishaps: A Patient Guide By Michael H. Kelleher, MD Reprinted with permission of the author. No endorsement by the Society to Improve Diagnosis in Medicine is made. The author is solely responsibile for the accuracy of the contents. Copyright Statement: Copyright registration 2015, Michael Kelleher M.D. This work may be reproduced and redistributed, in whole or in part, without alteration and without prior written permission, solely by educational individuals and institutions, for nonprofit educational purposes, provided that all copies contain the following statement: ”Copyright 2015, Michael Kelleher M.D. This work is reproduced and shared solely for nonprofit educational purposes.” Introduction The title of this book focuses not on the term “Medical Error” but on the broader term “Medical Mishap,” which captures more completely the full spectrum of causes for medical adverse events. When we speak of a medical error the implicit assumption is that the medical professional involved in the case was guilty of substandard care, with an error in clinical judgment or a defect in technical skill. But the malpractice insurance companies which track medical lawsuit data have long recognized that two other factors are frequently root causes for medical adverse events, namely system defects and patient contributions. The case vignettes and patient education summaries that have been compiled in this book, therefore, focus on those system defects (especially communication and information gaps) and patient behaviors that can help to prevent medical mishaps. Webster's dictionary defines a mishap as "an unfortunate accident", implying both preventable and unavoidable accidents. But this medical mishap discussion will focus on preventable cases, highlighting opportunities for improving patient safety. Most of the patient safety media coverage over the past 16 years has focused on hospital care, triggered by the publication in 1999 of the Institute of Medicine’s seminal text, “To Err is Human…” That document famously noted that 44,000 to 98,000 patients die each year in US hospitals as a result of medical errors. Much attention has been devoted to hospital safety in the wake of the IOM report, and many system improvements have been accomplished in hospitals. Examples include significant reductions in hospital acquired central line infections, improvements in operating room safety, and adherence to treatment guidelines for conditions like pneumonia, heart failure, and heart attacks. At the same time, however, it has become apparent that outpatient medical adverse events, those occurring outside the hospital walls, represent an equally large opportunity for improvement. The Journal of the American Medical Association published in June 2011 a summary of more than 10,000 medical malpractice suits from across the country. That summary revealed that 43% of cases involved no hospital care and another 9% included both hospital and outpatient components, so most litigated medical adverse events (52%) do involve outpatient care. Additional evidence for a large outpatient safety opportunity is available from a study co-funded by the US Agency for Healthcare Research and Quality and CRICO, the largest professional liability company in Massachusetts. That study was titled "Malpractice Insurance Medical Error Surveillance and Prevention Study" and carefully reviewed more than 1400 claims from across the country. Although surgical claims accounted for the largest number of cases, diagnosis-related cases accounted for the highest total dollar values. These diagnosis-related cases also account for most of the paid claims reported to the National Practitioner Databank each year, and the majority involve failure to diagnose cancer or heart disease or other conditions which represent missed opportunities at the doctors office, not at the hospital. The AHRQ/CRICO study also revealed that 75% of cases involved system problems outside the control of the individual clinician, 33% involved patient behavior or patient compliance problems, and 17% involved medication problems which contributed to the outcome. The National Patient Safety Foundation has also recently highlighted the impact of diagnostic errors, an under-recognized cause of medical mishaps, affecting 5-15% of all healthcare episodes. The NPSF references the need for “greater patient engagement, better use of information technology, and focused interventions to avoid specific, known pitfalls”. These large opportunities represent fertile ground for the outpatient case studies that are summarized in this book. It is not difficult to understand why medical mishaps are frequent events in our fragmented healthcare system (or non-system as many have suggested). Primary care physicians (PCPs) are the first line of defense for patient safety, functioning in ideal circumstances as the quarterback for the healthcare team, coordinating all aspects of care and communicating with all specialists who may see the patient. But there is a growing shortage of PCPs in most states, with long waits for adult PCP appointments. Also the PCPs at most offices no longer go to hospitals or skilled nursing facilities, relying on hospital-based physicians to provide this care. PCP office congestion also results in many patients seeking same-day care at freestanding urgent care facilities or emergency departments, creating more opportunities for communication gaps and follow-up failures. Electronic medical records within individual group practices can help to improve coordination of care, but these systems typically do not connect with nearby hospitals or with other group practices, another barrier to safe and coordinated care. In the context of this fragmented care system, patients can reduce the likelihood of a serious medical mishap by becoming more actively engaged in their care. This engagement requires commitment to more effective communication with all clinicians, and can be facilitated by means of medical information resources like this book that are translated into laymen terms, and easily accessed. Patient information websites such as WebMD and Medicinenet can be a starting point for patients seeking medical guidance, but these sources typically provide only superficial coverage of medical topics, and do not have a specific focus on practical patient safety opportunities. This book adopts the unique approach of focusing only on those medical mishaps which are common and preventable, drawing on my medical leadership experience over the past 34 years, reviewing and analyzing more than 7,000 adverse medical events in several healthcare systems. Some of the events I reviewed were not preventable and would not have benefited from actions on the part of the patient and family. But a surprising number of cases could have generated a more favorable outcome if the patient had been able to collaborate more effectively with the care team, with improvements in communication and medical follow-up. These practical lessons comprise the first 10 chapters of this book, each of which focuses on a specific category of mishap. The last chapter takes a different tack, addressing the three patient habits that account for more disability and death than the first 10 chapters combined. The language is concise by intent, with no wasted words, and with online resources identified at the end of each chapter to provide more information on the topic of interest. Although the primary goals here are enhanced patient safety and improved patient outcomes, an important secondary outcome should be the avoidance of medical expenses that are preventable or unnecessary. Third party payers have continued to shift costs to patients, with increasing copay and deductible obligations, so patients can make use of many of these recommendations to avoid care that has no value or negative value, with substantial financial benefit. A single preventable hospital admission can represent savings of several thousand dollars for a patient with standard Medicare coverage, or typical “Bronze” or “Silver” ObamaCare coverage. I have included sample patient case histories for each of the major categories of preventable events, drawn from actual cases I have encountered in the past, but with changes to avoid any identification of actual patients. If this text proves useful to readers, it can certainly be expanded in future editions to include more examples of opportunities for patients to steer their care in safer directions. It has been said that patients are the most underutilized resource in our healthcare system, so readers may consider this book to represent a modest effort to correct that longstanding oversight. MK Disclaimer: This book contains educational information about medical conditions, but is not intended to substitute for personal diagnostic or treatment advice, which must be obtained in all cases from your personal healthcare professionals. The medical information in this book is presented “as is,” without any representations or warranties, express or implied, and without any guarantee that the information is complete, up-to-date, or free of errors. Table of Contents Avoidable Medical Mishaps Preface Author’s Introduction Disclaimer Foreword - Medical Safety Lessons From The Agency for Healthcare Research and Quality (AHRQ) 20 Practical Tips for Engaged Patients and Families Chapter 1 - Common Diseases Often Missed or Mismanaged The Evaluation of Rectal Bleeding Osteoporosis: Major Missed Opportunity for Pain and Disability Prevention Celiac disease: Long Delays in Diagnosis are Preventable Iron deficiency: Causes Must be Explored Carefully Hemochromatosis: Delays in Diagnosis are Common for this Inherited Disease Painful Kidney Stones: Preventive Treatments are Under Used Adrenal Failure: Often Misdiagnosed, with Many Cases Related to Prednisone Usage Chapter 2 – Diagnostic Challenges in Reproductive Health Von Willebrand Disease and Heavy Menses Ectopic Pregnancy Diagnostic Pitfalls Follow-up Lapses with New PAP/HPV Protocol for Cervical Cancer Screening Big Uterine Fibroids Can Hide Other Pelvic Problems Chlamydia STDs Are Often Silent…Can Masquerade as Bladder Infection Chapter 3 - Infectious Diseases Antibiotics: Three Cautions Regarding Complications Lyme disease: Cautions Regarding Prevention and Diagnosis Hepatitis C: The “Baby Boomer Disease” Requires Careful Surveillance Necrotizing Fasciitis: Diagnostic Delays are Common and Deadly Chapter 4 - Medication Interactions and Hazards Opioids: Three Cautions Regarding the Tragic Overdose Epidemic NSAIDs: Three Cautions Regarding the Most Dangerous of All OTC Meds Hyperkalemia: Drug Interactions That Can Prove Fatal Beta Blockers: No Longer Preferred for Blood Pressure Control Insulin: Essential but Hazardous Therapy Diuretic risks: Commonly Overlooked Pitfalls Amiodarone/Cordarone: Controls Heart Rhythm, But Requires Close Monitoring Medication Hazards During Pregnancy Chapter 5 - Risks with Transitions In Care Hospital Discharge Plans: Hand-offs Are Often Mishandled Incidental CT Findings: A Frequent Source of Lawsuits Specialist “Hop Scotch”: Can Lead to Mishaps The Transition to Medical Frailty: When Gravity Starts to Win Hospice and Care at The End of Life: “The Ultimate Transition” Chapter 6 - Self-Monitoring Opportunities Home BPs: Can Help to Prevent Unnecessary Meds and Visits Home Glucose Monitoring: Best Way to Insure Good Diabetes Control Home INR Monitoring: Safer and More Convenient Home Peak Flow Monitoring: An Asthma Early Warning Yardstick Chapter 7 - Self-Triage Tips Headache: Which Ones Need Prompt Evaluation Stroke: How to Benefit from Clot-Busting Drugs Chest Pain: When to Call 911 Abdominal Pain: Which cases Need Prompt Surgical Evaluation Back Pain: The Danger Signs which Should Trigger Early Action Obstructive Sleep Apnea: Under Diagnosed and Under Treated Chapter 8 - Unnecessary Procedures Stress Tests for Low Risk Patients: Why These Can Be Misleading Avoidable Procedures: How I Almost Lost My Mother-In-Law The Choosing Wisely Campaign: Physicians Designate Low-Value Care CRP syndrome: Ill-Advised Surgery Can Make Matters Worse Chapter 9 - Visual Symptom Alerts Cranial Arteritis: Blindness Can Result From Delays in Diagnosis Visual Floaters and Flashes: Which Ones Need Prompt Attention Glaucoma: When Halos Are Not Good The Red Eye: Can be Misdiagnosed as Conjunctivitis Chapter 10 - Cancer Screening Breast Lumps: Mammograms Can Be Misleading Cervical Cancer and HPV: Major Missed Vaccine Opportunities Lung Cancer: Effective Screening Is Finally Available, But…. Colon Polyps: One-Size-Does-Not-Fit-All for Follow-up PSA Testing: Not for All Men…Shared Decision-Making is Key Chapter 11 – Injuries That Are Often Misdiagnosed Navicular Fractures of the Wrist Hip Fractures in Patients Who Can Still Walk Missed Achilles Tendon Ruptures Spinal Fluid Leaks: When a Runny Nose Can Be Life-Threatening Chapter 12 – Our Deadliest Habits Tobacco Smoke: The Only Legal Substance With No Safe Dose Caloric Excess: The Obesity Epidemic and Its Consequences Alcoholism: Off the Front Page, But Still a Potent Killer About the Author Michael H. Kelleher, MD Foreword Medical Safety Lessons From The Agency for Healthcare Research and Quality (AHRQ) Twenty Practical Tips for Engaged Patients and Families The US Agency for Healthcare Research and Quality (AHRQ) provides funding for research projects focusing on patient safety and quality of care, and collects information related to adverse medical events. The agency has also posted on its website a summary of practical suggestions for patients and families to become more involved in their personal healthcare to reduce the risk for adverse events. Many of these recommendations relate to gaps in communication and follow-up, which are all-too-common in our current healthcare system. The following information is in the public domain, without copyright protection, and is reproduced below as a prelude to the specific healthcare scenarios addressed in this book. This information, which can be shared freely with others, is also available online at www.ahrq.gov/patients-consumers/careplanning/errors/20tips/index.html: What You Can Do to Stay Safe The best way you can help to prevent errors is to be an active member of your health care team. That means taking part in every decision about your health care. Research shows that patients who are more involved with their care tend to get better results. Medicines 1) Make sure that all of your doctors know about every medicine you are taking. This includes prescription and over-the-counter medicines and dietary supplements, such as vitamins and herbs. 2) Bring all of your medicines and supplements to your doctor visits. "Brown bagging" your medicines can help you and your doctor talk about them and find out if there are any problems. It can also help your doctor keep your records up to date and help you get better quality care. 3) Make sure your doctor knows about any allergies and adverse reactions you have had to medicines. This can help you to avoid getting a medicine that could harm you. 4) When your doctor writes a prescription for you, make sure you can read it. If you cannot read your doctor's handwriting, your pharmacist might not be able to either. 5) Ask for information about your medicines in terms you can understand—both when your medicines are prescribed and when you get them: What is the medicine for? How am I supposed to take it and for how long? What side effects are likely? What do I do if they occur? Is this medicine safe to take with other medicines or dietary supplements I am taking? What food, drink, or activities should I avoid while taking this medicine? 6) When you pick up your medicine from the pharmacy, ask: Is this the medicine that my doctor prescribed? 7) If you have any questions about the directions on your medicine labels, ask. Medicine labels can be hard to understand. For example, ask if "four times daily" means taking a dose every 6 hours around the clock or just during regular waking hours. 8) Ask your pharmacist for the best device to measure your liquid medicine. For example, many people use household teaspoons, which often do not hold a true teaspoon of liquid. Special devices, like marked syringes, help people measure the right dose. 9) Ask for written information about the side effects your medicine could cause. If you know what might happen, you will be better prepared if it does or if something unexpected happens. Hospital Stays 10) If you are in a hospital, consider asking all health care workers who will touch you whether they have washed their hands. Hand washing can prevent the spread of infections in hospitals. 11) When you are being discharged from the hospital, ask your doctor to explain the treatment plan you will follow at home. This includes learning about your new medicines, making sure you know when to schedule follow-up appointments, and finding out when you can get back to your regular activities. It is important to know whether or not you should keep taking the medicines you were taking before your hospital stay. Getting clear instructions may help prevent an unexpected return trip to the hospital. Surgery 12) If you are having surgery, make sure that you, your doctor, and your surgeon all agree on exactly what will be done. Having surgery at the wrong site (for example, operating on the left knee instead of the right) is rare. But even once is too often. The good news is that wrong-site surgery is 100 percent preventable. Surgeons are expected to sign their initials directly on the site to be operated on before the surgery. 13) If you have a choice, choose a hospital where many patients have had the procedure or surgery you need. Research shows that patients tend to have better results when they are treated in hospitals that have a great deal of experience with their condition. Other Steps 14) Speak up if you have questions or concerns. You have a right to question anyone who is involved with your care. 15) Make sure that someone, such as your primary care doctor, coordinates your care. This is especially important if you have many health problems or are in the hospital. 16) Make sure that all your doctors have your important health information. Do not assume that everyone has all the information they need. 17) Ask a family member or friend to go to appointments with you. Even if you do not need help now, you might need it later. 18) Know that "more" is not always better. It is a good idea to find out why a test or treatment is needed and how it can help you. You could be better off without it. 19) If you have a test, do not assume that no news is good news. Ask how and when you will get the results. 20) Learn about your condition and treatments by asking your doctor and nurse and by using other reliable sources. Ask your doctor if your treatment is based on the latest evidence. Editorial comments from MK: Recommendation number 18, referenced on the AHRQ list above, makes the point that “More care is not always better care.” This is a theme that resonates with many of the chapters of this book, as related for example to screening tests, antibiotic overuse, and the “Choosing Wisely Campaign” which is referenced in Chapter 8. Although not mentioned specifically in the 20 tips above, it is also helpful for patients to obtain and retain copies of their medical records. After all, we retain copies of the maintenance records for our car, and our bodies should be no less important to us. Review of your personal medical records also affords the opportunity for correction of factual errors and omissions, as well as for the smooth transitioning of care to a new primary care office as needed. From a broader perspective, these 20 tips represent generic medical safety strategies that can be applied by patients to all of the clinical situations addressed in the following pages. Chapter 1 Common Diseases Often Missed or Mismanaged Section 1 The Evaluation of Rectal Bleeding Small amounts of rectal bleeding are usually caused by benign rectal disease such as hemorrhoids and rectal fissures, but a significant number of these cases prove to be due to cancer. For that reason it is important for physicians to adhere to national practice guidelines in the assessment of patients with rectal bleeding. Failure to follow these guidelines can have serious consequences for the patient, as revealed in the following actual case summary: “Mr. D, a 56-year-old banker, presented to his PCP with a 10-day history of small amounts of blood with bowel movements. He felt well otherwise with no abdominal pain, and had undergone a normal colonoscopy at age 50. His PCP performed examination of the rectum with an anal scope, and found several hemorrhoids, one of which appeared to be irritated, with a bit of bleeding caused by the scope. Mr. D was told that the bleeding was just due to hemorrhoids, and was advised to use metamucil and suppositories, with no colonoscopy suggested at that time. Unfortunately, one year later Mr. D returned with increased rectal bleeding and a sigmoid colon cancer was found to have spread to his liver, with poor prognosis.” This case makes the point that hemorrhoids are very common, detectable in almost half of all adults, so it is not wise to assume that they are the sole cause of rectal bleeding in adults over age 50. The American Society for Gastrointestinal Endoscopy (ASGE) recommends that full colonoscopy be performed in patients over age 50 with rectal bleeding, even if a hemorrhoid source is suspected, unless the patient has had a normal colonoscopy within 2-3 years. We know that colon cancers can develop within 5 years after a colon exam, so the evaluation performed in the case of Mr. D as described above was inadequate for a patient with rectal bleeding. The ASGE guideline referenced above goes on to state that full colonoscopy can be avoided for patients with small amounts of rectal bleeding if the patient is under age 50 with no risk factors for colon cancer, provided that a sigmoidoscope exam (shorter scope as compared to colonoscopy) reveals a suspected benign cause for the bleeding. All of these comments apply only to patients with small amounts of bright red rectal bleeding. The patient who experiences large amounts of bleeding, or black stools, or maroon colored stools requires more aggressive evaluation, usually in the hospital setting. Likewise patients who have colon polyps or a strong family history of colon cancer, or chronic alternative colitis may qualify for more frequent colonoscopy exams regardless of age or rectal bleeding. It is also important to note that all patients over age 50 should have a screening test for colon cancer performed on a periodic basis regardless of whether any rectal bleeding has been noted. The preferred test is colonoscopy every 10 years, but some patients, in consultation with their PCP, may choose to have annual test for hidden blood in the stool, or other screening tests that have been approved. More information on this topic is available at: www.asge.org (check the clinical guidelines tab for “role of endoscopy in the patient with lower GI bleeding”) www.rmf.harvard.edu/Clinician-Resources/Guidelines-Algorithms/2014/CRCscreening-with-symptoms (This guideline from the Harvard Risk Management Foundation is essentially identical to the ASGE version above) www.cghjournal.org/article/S1542-3565(13)01048-3/pdf (This study from a high quality group practice in eastern Massachusetts revealed that only 57% of patients over age 40 with rectal bleeding were appropriately evaluated within 90 days with colonoscopy or sigmoidoscopy) Chapter 1 Common Diseases Often Missed or Mismanaged Section 2 Osteoporosis: Much Pain and Disability Can be Prevented Osteoporosis is defined by significantly decreased bone density or weak bones, as measured by sophisticated bone densitometry testing. Standard x-rays can confirm osteoporosis in its late stages, but are not a reliable method to diagnose the problem for most patients. The US Preventive Services Task Force recommends that all women over age 65 be screened with a bone density test, but studies reveal that fewer than 45% of eligible women have been tested in the US. This is perhaps the most under-diagnosed and under-treated disabling disease of our time. The health impact of osteoporosis is staggering, with more than 1.5 million Americans suffering annually with a bone fracture related to osteoporosis. The lifetime risk for osteoporotic fracture in females is about 40%, and for males 25 to 33%. Hip Fractures are the most devastating, with a death rate near 30%, and with one fourth of hip fracture patients requiring nursing home placement, with loss of their independence. Spinal compression fractures are even more common, and can cause disabling pain as well as deformity of the spine, known in the past as the dreaded “dowagers hump.” Despite the abundant data documenting the impact of this disease, the medical system’s track record with respect to screening and treatment is very disappointing. Recent efforts have focused on the promotion of bone density testing in older women, with physician quality incentives tracked by Medicare and by Medicare HMOs. But the best outcomes won’t be achievable until senior women become more engaged in their own care, demanding bone density testing at the same rate as their requests for flu shots. Bone density testing is a covered preventive service for all Medicare plans, and patients with osteoporosis risk factors are covered for bone density testing at younger ages as well by commercial insurance companies. Patient should be aware of the factors that increase the risk for osteoporosis as follows: Prednisone treatment, at least 5 mg per day for three or more months Body weight less than 127 pounds, or with BMI less than 20 Premature menopause Men with testosterone deficiency, including those treated for prostate cancer with Lupron or Eligard Current smokers Alcohol users with three or more drinks per day Malabsorption of food due to any cause, including gastric bypass surgery Rheumatoid arthritis Diet deficient in calcium or vitamin D Age over 65 Caucasian or Oriental race Sedentary lifestyle Prior hip fracture or low velocity fracture (excludes auto accidents and falls from a height, as examples) The risk factors for each patient can be plugged into a risk predictor software product referred to as the “FRAX” tool, which generates a 10-year probability for osteoporotic fracture for that patient. Facilities that perform bone density testing usually have access to this tool, and many physician offices also have this information available. The FRAX calculation can identify those patients who qualify for osteoporosis medication treatment when the bone density is in the “borderline “ range, and also identifies patients at very high risk for whom treatment is essential. In general it is appropriate to request bone density testing for following patients: All women over 65 Postmenopausal women under 65 with other risk factors Patients with prior low velocity fractures All patients on prednisone tablets, at least 5 mg per day for three or more months Patients with osteoporosis noted on standard x-rays of the spine For patients identified with osteoporosis there are multiple medications that have proved effective for the prevention of future fractures, and some of these medications are needed just once a week or once a month. There is even an injectable osteoporosis medication that can be administered just once a year. Most of these medications are in the “bisphosphonate“ category, which helps add mineral strength to the bone structure. Unfortunately most patients discontinue treatment within a year and remain unprotected from the pain and disability associated with fractures of their weak bones. There seem to be at least three major reasons for this poor compliance: 1) Cost….but generic versions of alendronate are available for as little as $9 cash per month at Walmart and other discount chains, and generic Reclast requires just one injection per year at a drug cost of less than $60. 2) Side Effects…including heartburn and indigestion with the oral meds. These can be minimized with the once a week and once a month versions, and those patients with persistent symptoms can switch to injected meds once every 3 to 12 months to avoid the GI side effects. 3) Fear of two rare complications which have received media attention: a) Atypical femoral shaft (thigh) fractures…seen annually in only 3-4 of every 10,000 patients using these meds more than 4 years. But this risk is minimal compared to the annual reduction in risk for osteoporotic fracture which exceeds 40 for every 10,000 patients on treatment with these “bisphosphonate” meds. b) Osteonecrosis of the mandible…a degeneration of the jawbone which requires oral surgery intervention. But 95% of these bisphosphonate related cases are due to high dose intravenous therapy for cancer and Pagets disease. For patients who use these meds in tablet form for osteoporosis the risk is much lower…less than 1 case per thousand patients who use the meds up to 5 years. In order to reduce the impact of cost, side effects, and these rare complications many physicians now recommend that patients take a one year holiday from their osteoporosis medication every 4-5 years. Ongoing research should help to clarify the potential advantages of this approach. For those patients who begin medication treatment for osteoporosis it is appropriate to recheck the bone density test after 2 years to assess progress. Likewise those patients who have multiple risk factors or borderline bone density, also called “osteopenia”, it is reasonable to recheck the bone density test in 2 years. On the other hand there is no reason to repeat the bone density test if the first result is normal and the patient has a low risk history. All diagnosed patients should also include adequate calcium in their diet, using calcium supplements such as calcium carbonate 600 mg twice daily. For those patients who note constipation or indigestion on calcium carbonate tablets it is often helpful to switch to calcium citrate, also available over the counter. Daily vitamin D supplements are also recommended, with 800 units representing an appropriate dose for most senior patients. Once the diagnosis of osteoporosis is established the treating physician is also obligated to search for any underlying metabolic disorders that might be causing the problem, such as kidney failure, an overactive thyroid gland or a parathyroid disorder. This can be accomplished with blood tests and a careful history and physical exam. Patients should also be encouraged to remain active with a regular exercise program matched to their age and physical condition. More information on this topic is available at: National Institutes of Health: Osteoporosis www.niams.nih.gov/Health_Info/Bone/Osteoporosis Mayo Clinic: Osteoporosis www.mayoclinic.org/diseases-conditions/osteoporosis/basics/risk-factors/con20019924 Chapter 1 Common Diseases Often Missed or Mismanaged Section 3 Celiac Disease: Long Delays in Diagnosis are Common but Preventable Celiac Disease, also known as “Gluten Sensitive Enteropathy” affects at least 1% of the US population, with the highest rate in Caucasians. The disease is caused by sensitivity to the gluten protein, which is found in wheat, barley, and rye. The body’s immune system is triggered by gluten to attack the lining of the intestine, causing intermittent abdominal discomfort and diarrhea in most patients. The disease can cause poor absorption of food, leading to weight loss, anemia, and bone disease including osteoporosis. The disease is curable with a gluten-free diet, but that diet is not easy to maintain, so it is essential that the diagnosis be confirmed before undertaking a lifelong commitment to this very restrictive diet. Unfortunately the diagnosis of celiac disease is often delayed by many years, despite frequent medical visits for a variety of symptoms. Consider the following typical story: Ms. D began experiencing bloating after meals and intermittent loose stools at age 34. Her physician ordered gallbladder and colon tests which were normal, and then recommended a lactose free diet, which helped only a little. The diagnosis of irritable colon was offered, and Ms. D was advised to use Metamucil and a high-fiber diet. Her symptoms never improved, and she was evaluated five years later by different physician for a pre-op exam before tubal ligation. At that time, she was noted to have iron deficient anemia, and the second physician astutely recognized the possibility of celiac disease as an explanation of the chronic intestinal symptoms and the iron deficiency. The diagnosis was confirmed with a blood test and intestinal biopsy, and all symptoms resolved promptly with a gluten-free diet. This pattern of a long delay before correct diagnosis is unfortunately typical for celiac patients. Survey studies in the US and Sweden confirm an average time lag of 9 to 11 years (!!) from symptom onset to diagnosis, in part because the symptoms often mimic irritable colon which is a common benign disease. The problem of under-diagnosis was further defined by the US National Health and Nutrition Survey of 2010, which performed blood tests on 4,000 randomly selected adults. The study found 35 cases of Celiac Disease, but only 6 of those patients were aware of the diagnosis! Surprising also was the finding that most patients adhering to a glutenfree diet did not have Celiac disease, so self-diagnosis is not reliable. The correct diagnosis can be suspected on the basis of a single positive blood test for antibodies to “Tissue Trans-Glutaminase” or “TTG”. This test is about 95% accurate, but positive patients should also have a small bowel biopsy to confirm the diagnosis. It is also important for patients not to begin a gluten-free diet until after the tests are performed, in order to avoid misleading false negative results. The American College of Gastroenterology published in 2013 (see first link below) an updated guideline for the diagnosis and management of celiac disease. This guideline recommends TTG testing for patients at increased risk, including those with the following symptoms or findings: Chronic diarrhea with weight loss and signs of malnutrition Unexplained iron deficiency Unexplained liver function test abnormalities Unexplained osteoporosis Patients with type one diabetes plus chronic G.I. symptoms Irritable bowel syndrome with doesn’t respond well to treatment Unexplained chronic abdominal pain, especially if the family history is positive for celiac disease It is reasonable for patients with any of these problems to discuss TTG testing with their physician, since Celiac Disease is completely curable with a gluten-free diet. Patients who are actively engaged in their care, asking appropriate questions, are less likely to experience a long and uncomfortable lag time before the diagnosis of Celiac Disease, which can cause disabling symptoms without the correct treatment. Children are also at risk for Celiac Disease, so well-informed parents can also play an important role as advocates for timely testing. More information on this topic is available at: American College of Gastroenterology: Diagnosis and Management of Celiac Disease www.gi.org/guideline/diagnosis-and-management-of-celiac-disease National Institutes of Health: Delay in Diagnosis and Implications www.ncbi.nlm.nih.gov/pmc/articles/PMC3233515/#!po=79.4118 Chapter 1 Common Diseases Often Missed or Mismanaged Section 4 Iron Deficiency: Pitfalls in Diagnosis and Treatment Mrs. B, 55-year-old teacher, was noted to have fatigue and Iron deficient anemia at the time of her annual check up. She also had occasional diarrhea. Her physician noted that there was no blood in her stool and ordered a colonoscopy and upper G.I. endoscopy that proved to be normal. Mrs. B was advised that the anemia was likely related to years of menstrual periods and she was treated with iron supplements, which partially corrected the anemia. Two years later she returned with weight loss and back pain that proved to be due to osteoporosis and compression fractures of the spine. Her anemia had also worsened despite the iron supplements. Further evaluation with a blood test and a biopsy confirmed the diagnosis of celiac disease as the cause of all of her symptoms due to poor iron absorption from the gut. The symptoms resolved completely with a gluten-free diet (see also last section on Celiac Disease). The key point here is that Iron deficiency in adults requires careful assessment. Colon and stomach evaluation is part of that workup, but other causes should also be considered, including malabsorption of food and urinary blood loss. On a related theme, for those women who have early iron deficiency caused by menstrual periods, there are now several research studies that support the use of iron supplements before the development of anemia. The blood “ferritin” test is used to diagnose Iron deficiency, with iron therapy usually reserved for patients with levels below 20. Recent studies indicate however that women with levels below 50 can benefit from Iron supplements with improvement in fatigue and energy levels even if their red cell count is normal (see also first two links at the end). This is not surprising, since iron is required not just for red blood cells, but also for the cytochrome enzymes which provide energy production in all the cells of the body. A few tips regarding iron supplement options are also worth mentioning. Most patients use over-the-counter Ferrous Sulfate, which can cause abdominal pain and constipation. Ferrous Gluconate is also available over-the-counter and is less often associated with side effects. It is important to note also that tea does reduce iron absorption and should be avoided for several hours before and after the iron supplements. Vitamin C at the dose of 250-500 mg improves iron absorption from the gut and also provides other benefits, so many experts recommend combining iron supplements with vitamin C. More information on these topics is available at: National Institutes of Health: Iron Supplements for Women with Fatigue www.ncbi.nlm.nih.gov/pubmed/12763985 CMAJ: Effect of Iron Supplementation on Fatigue www.cmaj.ca/content/early/2012/07/09/cmaj.110950.long American Society of Hematology: Iron-Deficiency Anemia www.hematology.org/Patients/Anemia/Iron-Deficiency.aspx Chapter 1 Common Diseases Often Missed or Mismanaged Section 5 Hemochromatosis: Delays in Diagnosis are Common for this Inherited Disease The last section in this chapter addressed the topic of iron deficiency and its appropriate evaluation. This summary will focus on the opposite problem: Iron overload due to an inherited disease known as hemochromatosis. Patients who are affected by this disease have inherited a defective gene from each of their parents, so the disease is found to cluster in affected families. This is one of the most common inherited diseases, with about 9% of American Caucasian adults harboring one dose of the defective gene, and just under 1% with two doses. Not all patients with the double gene defect develop the disease however, so the overall frequency of this disease is about one in 200 Caucasians, with lower rates in other races. Patients with hemochromatosis gradually accumulate an overload of iron in all of the body’s tissues, with particularly high levels in the liver. Early in the disease there are no symptoms, but by age 40 to 60 these patients develop abnormal liver tests, Type II diabetes, low sex hormone levels, arthritis, and in late stages heart failure and liver failure. In Asian and Caucasian patients there may also be abnormal skin color, described as a bronzed or tanned appearance, even with no sun exposure. Women are less likely to show signs of hemochromatosis, even if they have the abnormal genes, because of iron lost during the menstruating years. If this disease is not correctly diagnosed the patient can develop advanced heart disease and cirrhosis of the liver, as well as the complications of advanced diabetes. So it’s important for the engaged patient and his/her physician to consider this genetic disease when patients are found to have unexplained abnormal liver tests, or a new diagnosis of diabetes in combination with any of the other signs mentioned above. The key step is to think about the possibility of hemochromatosis, because in many cases there are substantial delays in diagnosis. Consider for example the following common story: Mr. H presented to his PCP at age 52 with symptoms of new onset diabetes, as well as bilateral knee arthritis and abnormal liver function tests. He was advised by his PCP that all these problems were likely caused by his obesity, since his BMI was 32 with a weight of 230 pounds. He was then treated for several years with medications, and developed impotence, which was found to be related to testosterone deficiency. An endocrine specialist was consulted, who astutely noted the possibility of hemochromatosis as an explanation for all of Mr. H’s findings. That diagnosis was confirmed with a liver biopsy, and the patient was successfully treated with therapeutic phlebotomy. This story is typical for hemochromatosis patients, and many cases remain undiagnosed. For example the average panel of adult patients assigned to a PCP is more than 2000, which translates to at least 10 expected patients with hemochromatosis. However, very few PCPs have confirmed this diagnosis for more than one or two patients in their practice. The initial diagnostic test is a blood sample for iron, iron binding capacity, and ferritin. If these tests show abnormally high iron levels the diagnosis can be further assessed with MRI of the liver. Biopsy of the liver provides the most reliable confirmation of diagnosis, with actual measurement of the iron content of liver tissue. When the diagnosis is confirmed treatment is initiated with periodic one pint blood donations, which translates to “therapeutic phlebotomy” in medical terms. Depending on the severity of the case this phlebotomy usually starts at a frequency of once or twice weekly. After iron levels have returned to normal the frequency of phlebotomy can be reduced to once every 2 to 4 months. If treatment is begun early enough the patient can enjoy a normal lifespan, particularly if excess alcohol is avoided. Some patients are unable to tolerate phlebotomy because of anemia due other causes or because of heart disease. These patients can be treated with socalled chelation therapy, using injected or oral medications that remove iron through the kidneys. Since this is an inherited disease is essential that family members also be informed and identified for testing. Within the family of a diagnosed hemochromatosis patient there is a 25% chance that each of his/her siblings is also affected. Genetic counseling is often helpful to explain the rationale and the potential consequences for each patient who is considering the screening process. Take home messages for patients: Discuss hemochromatosis testing with your primary clinician if you are found to have unexplained abnormal liver tests Likewise discuss possible testing if you are diagnosed with type II diabetes plus any of the following: unexplained abnormal liver tests, heart failure, sex hormone deficiency, arthritis of the knees and hips, or unusual tanning of the skin without sun exposure. More information on this topic can be found at: www.cdc.gov/ncbddd/hemochromatosis www.nhlbi.nih.gov/health/health-topics/topics/hemo/ www.mayoclinic.org/diseases-conditions/hemochromatosis/basics/testsdiagnosis/con-20023606 Chapter 1 Common Diseases Often Missed or Mismanaged Section 6 Painful Kidney Stones: Preventive Treatments are Under Used Painful kidney stones are common, affecting at least 10% of men and 7% of women according to the most recent data from The National Health and Nutrition Survey. For those individuals who suffer through the unassisted passage of a stone the experience is memorable, often compared to childbirth by those women who’ve experienced both adventures. Most small stones can be passed spontaneously in the urine, sometimes with the assistance of medications such as calcium channel blockers. Modern medical science has also developed effective techniques to deal with stubborn stones that do not pass spontaneously. High tech shockwave therapy can be used for some of the stones, and urology specialists can also use special basket retrieval or lasers for other stones. Fortunately, there are many helpful interventions that are available to get the patient through the painful emergency phase of this illness. However at least 50% of patients can expect more stones to pass within 7 to 10 years after the first episode. Many patients have multiple painful episodes so there is great interest in prevention strategies. The American Urology Association recently published an updated guideline titled “Medical Management of Kidney Stones,” cited as the first link at the end of this summary. This authoritative guideline, based on 46 carefully conducted studies of kidney stone treatment, offers hope for the kidney stone sufferer, who has likely been exposed to many popular misconceptions about prevention. For example, many stone formers attempt to restrict their intake of calcium and milk products, based on the false premise that this will prevent stones. Others were advised to increase their fluid intake and responded by increasing their beer consumption to a six-pack daily, not what the doctor had in mind. There are three key pieces of information required to plan effective preventive therapy for patients with recurrent kidney stones. The first is chemical analysis of the saved stone, the second is a 24-hour urine sample to determine urine chemistries, and the third is blood testing to assess for underlying disorders. Unfortunately these three critical diagnostic steps are not completed for many patients with recurrent stones. I continue to be amazed to hear patients tell me that they have endured 10 or more episodes of painful stone passage, without a stone analysis and without ever collecting a 24-hour urine sample to determine effective treatment. In fact the last person to tell me that story was the CEO of a healthcare organization! And my own brother is in the same category!! Some patients may have been asked by their physician to collect the 24-hour urine sample but never complied with that request. But there are also many instances where the treating physicians didn’t make the request, for example when the patient sees only emergency department physicians who don’t have accountability for longterm followup. Stone patients are usually given urine strainers by the emergency department staff, with instructions to save any stones that pass into the urine. But patients are often not consistent or compliant with the use of these pesky little devices. Patients may also save the stone but never get it analyzed through their physician’s office, another opportunity for process errors. The stone analysis and 24-hour urine tests are critical for recurrent stone formers, because there are effective preventive treatments that are determined by stone type and by urine chemistry results. All kidney stone patients should increase their daily fluid intake (not beer) to approximately 3 quarts daily, which translates to nine 10 ounce glasses or six of the typical 1 pint water bottles. It’s also helpful to avoid soft drinks in the “cola” category, which contain phosphoric acid. But more specific treatment is also required for those patients who continue to pass stones despite a high fluid intake, as dictated by the results of urine testing and stone analysis. The following comments summarize the main treatment options from the Urology Association guideline described earlier: For calcium oxalate stones, the most common type, sodium intake should be limited to 3 grams daily with a low salt diet, and calcium intake should be normal, at 1000 to 2000 mg daily, without calcium supplements. In these patients if the urine calcium content is high it can be reduced with thiazide type diuretics such as HCTZ, which reduce the risk of future calcium stones by about 50%. If the urine calcium content is low or normal the patient can benefit from prescription potassium citrate, which reduces the risk for future stones by about 75%. Potassium citrate is less convenient, with dosing 3 times daily for most patients, and more frequent side effects, so some patients with calcium stones are treated with HCTZ or another similar diuretic regardless of their urine calcium levels. The success rates quoted above for medication therapy reflect all patients enrolled in the published studies, including those who didn’t continue the meds, so patients who stick faithfully to the treatment program can expect even better results. For patients with recurrent calcium oxalate stones and high levels of uric acid in the urine a medication called allopurinol can help to prevent future stones. For patients with uric acid stones or cystine stones the recommended initial treatment is with potassium citrate supplements. Some patients with uric acid stones and gout may also benefit from the medication allopurinol. Note also that uric acid stones are usually not visible on routine X-rays, making followup tracking a bit more challenging. For the rare patients with so-called “struvite” stones (containing calcium, magnesium, and ammonium) the cause is usually chronic urine infection, so these patients require chronic antibiotics in some cases, but it’s also important to remove any large stones in the kidney that may be harboring the infection. Individually tailored therapy using these well-proven guidelines can reduce the frequency of future kidney stones substantially, so it’s disappointing to see patients who continue to suffer without the benefit of these effective therapies. There’s little doubt that our fragmented healthcare system is at least partially responsible for this treatment gap, since the typical kidney stone episode involves three or four separate categories of providers: the on-call call primary care clinician (PCP) who takes the initial call from the patient, the ED staff who perform the initial assessment, the urologist who gets the referral from the emergency department, and the PCP who owns the follow-up care. In many cases the patient may not return to the PCP to close the loop for urine testing and for stone analysis, reducing the chances for future stone prevention. The patient with recurrent kidney stones who reads this summary is now aware of the information needed to ensure that inappropriate diagnostic evaluation is performed, including the important 24-hour urine collection a few weeks after the stone has passed. Even if the stone can’t be analyzed (sometimes the stone breaks up into tiny fragments during passage and can’t be saved) the blood tests and 24hour urine tests are usually sufficient to determine the best treatment plan. A second 24-hour urine collection may also be helpful to confirm the response to treatment. Initial radiology studies of the kidneys are also helpful in detecting residual stones which are at risk for passage in the future. In some cases, these tests will also suggest the possibility of an underlying disease such as an overactive parathyroid gland or primary kidney disease. Further medical evaluation can assess these problems in proactive fashion, preventing more serious future complications. Gastric bypass surgery for obesity is another potential cause for kidney stones which has become much more common in the last 25 years, related to increased oxalate and low citrate levels in the urine. This can be corrected with potassium citrate prescriptions, and with avoidance of high oxalate foods such as black tea, spinach, nuts, and chocolate (see also the third reference link at the end). Take-Home Messages for Patients with Recurrent Kidney Stones: Be sure to have at least one stone analyzed through your treating physician Be sure to have 24-hour urine collection as well as blood testing to determine causes Do not restrict your calcium intake Drink 3 quarts of nonalcoholic fluids daily Discuss possible preventive medications with your treating physician More information on this topic is available at: www.auanet.org/education/guidelines/management-kidney-stones.cfm www.ncbi.nlm.nih.gov/pubmed/351268 and www.ncbi.nlm.nih.gov/pmc/articles/pmc2831413 Chapter 1 Common Diseases Often Missed or Mismanaged Section 7 Adrenal Failure: Often Misdiagnosed, with Many Cases Related to Prednisone Usage “Adrenal Crisis” is defined as cardiovascular collapse due to dangerously low levels of hormones produced by the adrenal glands. The patient deteriorates rapidly with very low blood pressure, kidney failure, and profound abnormalities of blood chemistry values. Rescue requires immediate recognition of the correct diagnosis, followed by urgent infusion of intravenous hormone replacement with cortisone. Adrenal crisis can be triggered by any acute illness, such as pneumonia or kidney infection, in a patient who has underlying failure of the adrenal gland due to one of three causes: Addison’s Disease, an auto-immune disease affecting the adrenal glands, made famous by the illness of president John F Kennedy. “Secondary” adrenal insufficiency, due to failure of the pituitary gland that controls the adrenal glands “Tertiary “ adrenal insufficiency, which is seen in patients treated long-term with prednisone and similar medications Addison’s disease and secondary adrenal gland failure are relatively rare, affecting perhaps one or two of every 20,000 persons in the United States. A brief review of clues to this diagnosis is still of value to laypersons, because these patients are often misdiagnosed initially. Let’s address that issue first and then discuss the more common scenario, which is adrenal insufficiency caused by prescriptions for longterm prednisone use. Addison’s disease is caused by antibodies in the blood that attack and eventually destroy the adrenal glands, which are the sole source of cortisol and other critical hormones in the body. This “auto-immune” disease progresses gradually with symptoms that are often vague and nonspecific, such as fatigue, loss of appetite, dizziness on standing, depression, and nausea. As the disease progresses, the patient may develop darkening of the skin, abdominal pain, anemia, and abnormal blood chemistry tests. It is common for patients to consult many physicians before the correct diagnosis is considered. A study published in the American Journal of Medical Science in 2010 reviewed the pathway to diagnosis for 216 patients with Addison’s disease. Overall 68% of patients were labeled initially with the wrong diagnosis, and fewer than 50% of patients were correctly diagnosed within six months, despite consulting three or more physicians (see citation at end of section). Fatigue, depression, and decreased appetite are common complaints and many patients have life related stresses, which may account for these chronic symptoms. There are, however, some objective clues which can help physicians and patients consider the correct diagnosis as follows: Increased skin and mucous membrane pigmentation without sun exposure Unexplained anemia, without iron deficiency Blood pressure that decreases more than 10 mm on standing Low sodium or high potassium levels in blood, sometimes with craving of salt Weight loss that is not intentional Low blood glucose values Any of these findings, in combination with a history of progressive fatigue should trigger blood tests to confirm the diagnosis. The evaluation can start at the PCP office with measurement of blood cortisol and ACTH levels in the morning. If the cortisol level is greater than 14 and the ACTH level is normal the diagnosis of adrenal failure is essentially excluded. For lower levels of cortisol it’s necessary to proceed with an ACTH stimulation test, with cortisol levels measured before and after injection of ACTH hormone. This stage of the testing process is best supervised by a certified endocrinology specialist, who may also order 24-hour urine collections to check specific hormone levels. In some cases, the adrenal gland failure is due to decreased production of ACTH hormone by the pituitary gland, so-called secondary adrenal insufficiency. This diagnosis requires further evaluation of the pituitary gland by brain MRI to look for pituitary tumors. Regardless of whether the adrenal failure is primary or secondary it is possible to return the patient to normal health with the prescription of hormone supplements in tablet form. It’s critical to note however that these patients require increased stress doses of their cortisone supplements if they need surgery or if they become acutely ill due to an unrelated illness such as pneumonia or heart attack. Failure to do this can result in potentially fatal adrenal crisis, described in the first paragraph of the section. All of the foregoing comments relate to the rare causes of adrenal failure, with diseases directly affecting the adrenal and pituitary glands. However, the most common trigger of a acute adrenal crisis is medication related, caused by the longterm use of prednisone and related corticosteroid medications. The following case summary is typical in this regard: “Mr. J, 65-year-old man with temporal arteritis had been using prednisone at 20 mg daily for 6 weeks to control severe headaches. Unfortunately he was involved in a serious car accident and was brought to the local ED with multiple fractures. As he is being prepared for surgery his blood pressure becomes dangerously low with agitated confusion. His medical bracelet however alerts the staff to his prednisone therapy, and the attending physician treats him with 200 mg of IV cortisone, with prompt return of his blood pressure to safe levels.” For these patients on chronic prednisone therapy an adrenal crisis can be triggered one of the following events: The patient who has been using at least 20 mg of prednisone daily for three or more weeks stops the drugs suddenly or becomes very sick with an unrelated illness or surgery A patient using lower doses of prednisone but at least 5 mg daily for three weeks becomes very sick with an unrelated illness or surgery In these situations the patient can crash quickly with low blood pressure and multiple organ failure, all reversible with prompt intravenous infusion of cortisone supplements. This therapy can be life saving, so patients who have adrenal disease or use long-term prednisone medication should consider purchasing a medical alert bracelet to inform healthcare providers regarding their risk for adrenal crisis. Some of the disorders treated with long-term prednisone include the following: severe arthritis, polymyalgia, polymyositis, cranial arteritis also known as temporal arteritis, ulcerative colitis, Crohn’s disease, COPD or emphysema, severe asthma, and pemphigoid skin disease. These patients need to be aware of the risk for medication related adrenal suppression if they meet the criteria listed above. There is also evidence that high doses of inhaled steroids for asthma and COPD patients may cause some degree of adrenal suppression, so these patients often require booster doses of prednisone when they experience attacks of breathing distress or other severe illness. One final comment of interest about individuals diagnosed with autoimmune Addison’s disease. These patients have increased genetic risk for other types of autoimmune disease, including thyroid failure, type I diabetes, and gluten sensitive disease, so testing for these disorders should be considered if any related symptoms appear. More information on this complex topic is available at: www.mayoclinic.org/diseases-conditions/addisons-disease/home/ovc-20155636 The reference regarding misdiagnosis of Addison’s disease is: Bleiken et al, The American Journal of the Medical Sciences, vol 339, no 6, pages 525-531; 2010 Chapter 2 Diagnostic Challenges in Reproductive Health Section 1 Von Willebrand Disease and Heavy Menses Von Willebrand disease is the most common inherited bleeding disorder, caused by deficiency of a blood protein that enables normal blood clotting. The abnormal gene can be detected in about 1% of the general population, but among women with very heavy menstrual flow the disease has been diagnosed in 10- 20% of patients. Unfortunately there’s often a long delay in diagnosis for these women. In fact the Center for Disease Control reports that the average delay from onset of heavy menses to the correct diagnosis is 16 years! The following case summary is typical: “Mrs. B had noted heavy periods since her teen years and reported this problem to her physicians, who were able to decrease the flow somewhat with birth control pills. However she continued to use twice the typical number of pads, and also noted occasional nosebleeds causing her physician to advise avoidance of Advil and aspirin. She became pregnant at age 29, but her delivery was complicated by severe bleeding that required ICU care and multiple blood transfusions. Blood tests at that point revealed the diagnosis of Von Willebrand disease. She began treatment with a nasal inhaler containing DDAVP medication, and her menstrual flow returned to normal for the first time since age 15, with no further nosebleeds.” The most common form of Von Willebrand disease is an inherited disorder with each child of an affected parent having a 50% chance of inheriting the defective gene, so the family history can be an important clue to the correct diagnosis. Females are most likely to show symptoms because of the bleeding expected during menstrual cycles and obstetric delivery. Many affected women also develop iron deficiency anemia due to the heavy bleeding, so this form of anemia can be another clue to the diagnosis. Patients with Von Willebrand disease are also at risk for other forms of bleeding such as nosebleeds or hemorrhage after dental extractions and surgical procedures. The diagnosis can be confirmed by measurement of blood proteins including Von Willebrand factor antigen and ristocetin cofactor. These tests require specialist expertise for interpretation, and should be combined with tests for other bleeding disorders so the evaluation is best performed by certified physician hematologist. These specialty referrals must however be triggered by the primary care team or gynecologist, so patients with the symptoms described above should discuss with their clinicians the possibility of von Willebrand disease, especially if there is also a family history of heavy bleeding. The treatment of Von Willebrand disease depends on the specific genetic mutation, but many patients can be treat treated successfully with a nasal inhaler which delivers the synthetic hormone DDAVP, also known as desmopressin. Some women can use this approach just before and during their menstrual periods or for a planned surgical procedure. There is also some documented benefit from the use of contraceptive pills, especially those with progestin ingredients as well as the progestin containing IUD devices. For affected women with more serious bleeding episodes it may be necessary to use intravenous infusions of clotting factors or more potent drugs. All of these treatments are best supervised by qualified hematology specialist. When a patient is confirmed to have Von Willebrand disease, it is also important to consider the screening of immediate family members, particularly those with any history of abnormal bleeding. As indicated above, there is a 50% chance that siblings and children of an affected patient also have the most common type of abnormal gene. Relatives identified with the defective gene can benefit from this information even if they have never experienced heavy bleeding, in particular if they contemplate a major surgery procedure in the future, for which special precautions may be appropriate. Hopefully the sharing of this information can help women with excessive bleeding to communicate effectively with their clinicians to reduce the long delays in diagnosis that are typical for this disorder. Since effective treatment is available for most patients, it is important to consider the diagnosis before dismissing the bleeding as a burden to be endured. More information on this topic is available at: www.mayoclinic.org/diseases-conditions/von-willebranddisease/basics/definition/con-20030195 Chapter 2 Diagnostic Challenges in Reproductive Health Section 2 Ectopic Pregnancy: Diagnostic Pitfalls Etopic pregnancy occurs when the fertilized egg grows outside the uterus, usually within the fallopian tube. These ectopic pregnancies may cause intermittent vaginal bleeding, which can be confused with the normal menstrual cycle. The big concern with an undiagnosed ectopic pregnancy is that the abnormal fetal sac can rupture into the abdomen, causing severe pain and life-threatening bleeding. The following case story is not unusual for this high-risk situation: “Mrs. E, a 40 year old mother of two, presents to her primary care physician with a three day history of pain in her left lower abdomen, with no urinary symptoms but with some associated constipation. She reports that her menstrual period the week prior was a few days late and lighter than usual. Her PCP requests a urine pregnancy test which is negative, and he advises laxatives with instructions for the patient to call back if not better in a few days. No pelvic exam was performed. Four days later the patient presented to the local emergency department with severe pain in her abdomen and a fainting spell. She is found have a very tender abdomen and dangerously low blood pressure. Surgery reveals a ruptured ectopic pregnancy and multiple blood transfusions are needed to save her life.” This is not a rare scenario for ectopic pregnancy, with failure to make the correct diagnosis at the first medical evaluation. In this case there were two factors that contributed to the misdiagnosis: failure to perform a pelvic exam, and sole reliance on a urine pregnancy test. The pelvic exam is important because it will often reveal tenderness in the neighborhood of the ovaries and uterus, a clue that this patient’s pain was not due to constipation. A urine pregnancy test is not ideal for the diagnosis of ectopic pregnancy, which is associated with levels of HCG hormone that are lower than would be expected in the first weeks of a normal pregnancy. Urine tests in early ectopic pregnancy are also less reliable than blood tests because urine can be very diluted in those women who drink large amounts of coffee or soft drinks, making it more difficult to identify low levels of HCG hormone. The Medline review article cited at the end of this section assessed the accuracy of several types of urine pregnancy tests in women proven to have ectopic pregnancy. Overall these tests missed 5-10% of the ectopic pregnancies which were verified by blood tests and final obstetric outcome. So the preferred approach is to use a blood pregnancy test in this situation, increasing the likelihood that low levels of HCG can be detected. From a practical perspective it’s also reasonable to do the quick urine test in the office first, followed by expedited blood testing if the urine test is negative for a suspected ectopic pregnancy. When ectopic pregnancy is suspected on the basis of exam findings for pregnancy testing it is essential for the patient to be promptly evaluated by a qualified obstetrician, who will arrange for urgent transvaginal ultrasound testing to assess for an abnormal fetal sac. The obstetrician will also usually arrange to follow serial HCG hormone levels, which don’t increase as rapidly as normal in ectopic pregnancy. All ectopic pregnancies must be terminated, either spontaneously or by intervention with medication or surgery. The most commonly used medication for this purpose is methotrexate, which is also used for treatment of some types of arthritis and cancer. Patients who require surgical termination can usually be successfully treated with a minimally invasive approach through a laparoscope. This is much safer and less stressful than emergency surgery for a ruptured ectopic pregnancy that has been misdiagnosed. Take Home Points: Women in their reproductive years who present to a PCP office with lower abdomen pain should have an evaluation that includes pelvic examination and appropriate pregnancy testing, unless pregnancy is known to be impossible on the basis of sexual history or prior hysterectomy. In this regard no contraceptive method should be regarded as 100% effective. With respect to prevention of ectopic pregnancy it has been confirmed that smoking and STD related pelvic infections such as chlamydia do increase the risk. These risk factors are addressed in subsequent sections of this text. More information on this topic is available at: www.mayoclinic.org/diseases-conditions/ectopic-pregnancy/basics/testsdiagnosis/con-20024262 www.scirp.org/journal/PaperDownload.aspx?paperID=48488 Chapter 2 Diagnostic Challenges in Reproductive Health Section 3 Follow-up Lapses with New Pap/HPV Protocol for Cervical Cancer Screening Since 2012, the United States Preventive Services Task Force and the American College of OB/GYN have recommended a new approach to traditional Pap smear screening for cancer of the cervix. This new protocol recognizes that HPV virus is the cause of more than 90% of cervical cancer cases. Accordingly, it is now agreed that women between the ages of 30 and 65 may undergo testing every five years with Pap smears plus HPV testing. The prior practice was to offer pap smears alone for these women every one to three years. The new protocol is certainly more convenient and less expensive for patients, but also has created some patient confusion and risk regarding the recommended follow-up for abnormal tests. It also means that the patient who misses a single 5year follow-up appointment could go many years before her next screening visit. Follow-up problems may surface, for example, when a Pap test is reported as normal but the HPV test is positive, indicating increased future risk for cervical cancer. In this situation, the patient is usually advised to schedule repeat Pap and HPV testing in 12 months, or if high risk HPV subtypes are detected the patient may be advised to have a colposcopy procedure, with possible biopsies depending upon magnified views of the cervix. Colposccopy is also often recommended when the HPV test is repeatedly positive. Some patients are confused by this advice: “Why do I need more tests if my Pap shows no signs of cancer, just a virus?” Other patients may be reluctant to proceed with colposcopy because of cost, with high deductibles for their insurance coverage. The following case history is not unusual: “Ms. C undergoes Pap and HPV testing at age 35 and receives a letter from her PCP stating that the Pap is normal but the HPV test is positive for the second time with high risk subtype virus number 18. Her doctor advises a colposcopy procedure but Ms C learns that her Bronze health insurance deductible will hold her accountable for the $800 dollar procedure cost. She reasons that she can wait another year for a repeat Pap since her last exam revealed only a virus. But her next Pap and HPV are actually performed two years later, at which point invasive cancer cells are found.” The Center for Disease Control (CDC) and the American Cancer Society have posted online educational tools to help patients understand the updated screening guidelines and the importance of HPV follow-up (See citations at the end of the section). However, the longer time intervals between screening visits and concerns about out-of-pocket costs have created some challenges for this important screening process. The appropriate screening interval can range from one year to five years depending on the patient’s risk factors and prior screening results, so it is not surprising that patients are often confused about their follow-up schedule. The new guidelines do clarify to situations where cervical cancer screening is no longer needed. This applies to women with prior complete hysterectomy including cervix removal, as well as women over age 65 whose prior cervical screening tests have been normal. Most gynecologist still recommend however that all adult women have periodic pelvic exams to exclude other GYN disorders, but are respectful of those individuals who make an informed decision to discontinue pelvic exams, for example after age 65. Patients who have questions about their cervical cancer screening schedule or test results should not hesitate to discuss these issues with their medical clinicians at the primary care office or the GYN office. The value of HPV testing is beyond dispute at this point and all women should have timely follow-up of positive HPV tests regardless of Pap results More information o this topic is available at: www.cancer.gov/types/cervical/pap-hpv-testing-fact-sheet www.acog.org/Patients Chapter 2 Diagnostic Challenges in Reproductive Health Section 4 Big Uterine Fibroids Can Hide Other Pelvic Problems Uterine fibroids are benign growths that can develop at any age from the muscle tissue of the uterus. As they enlarge they can cause abnormal bleeding, lower abdominal pain, increased urine frequency, and constipation. Very large fibroids that extend from the left or right side of the uterus can also be difficult to distinguish from tumors of the ovary, which are more likely to be malignant. Most uterine fibroids can be easily defined as benign on the basis of ultrasound imaging, but some large ones are more difficult to diagnose, especially when the ovaries are not separately identified on the images. This can create risk for the patient as demonstrated in the following case summary: “Ms. O, a 52-year-old executive, was noted by her PCP to have a suspected mass in the left side of her pelvis on routine pelvic exam. This was evaluated by a gynecologist who performed transvaginal ultrasound, revealing a large complex suspected uterine fibroid that obscured the left ovary. The doctor discussed with the patient a recommendation for further evaluation with MRI scanning, but the patient reported claustrophobia with a prior MRI attempt and bargained for a follow-up ultrasound instead. That ultrasound was performed six months later and revealed fluid in the pelvis as well as several new masses in the abdominal cavity which proved to be widespread ovarian cancer.” Take Home Points Follow: Most uterine fibroids can be easily defined as benign on the basis of ultrasound imaging, but some complex masses are more difficult to diagnose, especially when the ovaries are not separately identified on the ultrasound images. MRI is the preferred procedure for evaluation for those pelvic masses that are of uncertain origin on ultrasound, because it provides more reliable diagnostic information as compared to CT scanning. Many facilities now have open magnet MRI scanners, which can be more tolerable for patients with claustrophobic problems. These patients may also benefit from mild sedation for the procedure. MRI imaging of the uterus may also reveal that some lesions identified as fibroids on ultrasound are actually adenomyosis, caused by uterine lining cells trapped within the muscle layer. This disorder can cause severe pain with menses and may require hysterectomy for relief. Although the primary purpose of this text is to share information to help patients prevent medical mishaps is also worth commenting here about some of the exciting new treatments for symptomatic uterine fibroids. These new methods offer the opportunity to avoid the potential complications of traditional hysterectomy. For many years women have had the option of a laparoscope procedure to remove superficial fibroids with a procedure called myomectomy. Likewise there is also a relatively long track record for endometrial ablation of those fibroids that are attached to the inner wall of the uterus, inside the uterine cavity. This involves placing a probe up the vagina and through the cervix in order to cauterize or vaporize these benign tumors. More recently it has become possible to treat large uterine fibroids with “embolization” techniques. This is accomplished by injecting tiny inert particles into a segment of the uterine artery, thereby depriving the fibroid of its blood supply. Shrinkage of the benign tumor follows, with improvement of pain and bleeding symptoms. It is also now possible to shrink fibroids by means of a focused beam of ultrasound energy that is guided by MRI images. This is the least invasive of available procedures, with no skin incision and no arterial catheter. However there is limited availability of this procedure and the long-term outcome studies are still in progress. Patients with longstanding symptoms related to their uterine fibroids are encouraged to discuss with their treating gynecologist these new treatment options, which can improve symptoms without major surgery. More information on these topics is available at: www.acog.org/Patients Chapter 2 Diagnostic Challenges in Reproductive Health Section 5 Chlamydia STDs Are Often Silent, But Can Masquerade as Bladder Infection Chlamydia infection is the most common treatable sexually transmitted disease (STD) in America by a wide margin, with the CDC estimating about 1.8 million infected individuals as of 2013. Sexually active females age 14 to 25 have the highest prevalence, estimated at 5% overall and with significantly higher rates in black and hispanic females. The untreated disease in women can cause severe pelvic pain, infertility, and ectopic pregnancy. In early stages it can also cause frequent and urgent urination, mimicking the symptoms of a bladder infection. Misdiagnosis of this problem can have serious consequences for the patient. The following case summary is not unusual: “Ms. S, a 20 year old sexually active college student presented to an urgent care center with a two-day history of burning on urination and increased vaginal discharge. The nurse practitioner at urgent care diagnosed a bladder infection and started treatment with a sulfa antibiotic. The burning subsided over a few days but the patient then developed severe lower abdominal pain and fever. The patient returned to the emergency department and was diagnosed with pelvic inflammatory disease that required treatment with injected and oral antibiotics. A cervical test at that time confirmed chlamydia infection.” This young patient was sexually active and had vaginal discharge as well as burning on urination so it would have been appropriate to test her for chlamydia at the first visit. This can be accomplished with a dipstick urine test, with results available in a few minutes. The antibiotics used for bladder infection such as sulfa drugs are not effective for chlamydia so this patient proceeded to develop a more serious preventable pelvic infection, which places her at risk for future infertility and ectopic pregnancy. It is also clear that many patients with chlamydia infection, both female and male, have no symptoms of infection so current medical guidelines from the CDC recommend annual screening tests for high-risk patients in the following categories: Patients in any of these high-risk groups who develop bladder symptoms should also request chlamydia testing as part of their evaluation, to avoid the outcome described in the vignette above. When a patient is diagnosed with chlamydia it’s also important to identify and treat sex partners from the prior 60 days in order to limit community spread of the disease. The preferred treatment is a single dose of azithromycin 1 g by mouth, which helps to assure high treatment compliance rates. Although chlamydia is the primary focus of this section a brief update is in order for the disappointing syphilis story. The incidence of this sexually transmitted disease had decreased in the United States to very low frequencies by the year 2000, but the number of reported cases essentially doubled between 2003 and 2013. The majority of these cases are seen in men who have sex with other men. Significant racial disparities also persist with syphilis rates being several- fold higher among black and Hispanic patients as compared to caucasians. This surge of syphilis cases creates significant diagnostic challenges for US physicians, because many younger physicians have never seen a case, and because syphilis is known as the “great imitator”. This disease can mimic many unrelated skin conditions and neurologic disorders so it presents diagnostic difficulty for many physicians. New syphilis cases also promote the transmission of HIV virus and AIDS because of the open sores, not good news for individuals with multiple sex partners. Given this information it becomes particularly important for patients with unexplained rash or genital sores to inform their treating physician if they have risk factors for syphilis with respect to sexual orientation or number of sex partners. This information can help increase the likelihood of accurate diagnosis and treatment. More information on the chlamydia topic is available at: www.cdc.gov/std/tg2015/chlamydia.htm And the syphilis story is summarized at: www.cdc.gov/std/syphilis/stdfact-syphilis.htm Chapter 3 Infectious Diseases Section 1 Antibiotics: Three Cautions Regarding Complications First Caution Regarding Antibiotics: Quinolone Antibiotic Therapy Appropriate antibiotic treatment can be life-saving for serious infections, but the US Centers for Disease Control has repeatedly warned us regarding the risks of antibiotic overuse. For example, the use of antibiotics for viral respiratory infections has become problematic, leading to the emergence of bacterial superbugs that are not susceptible to commonly used antibiotics Another rare but serious consequence of overuse is apparent from the following case summary: ”Mr. G is a 68-year-old patient with smoking-related lung disease, which requires long-term treatment with inhaled medications plus prednisone tablets. He presents to his primary care physician with sore throat and head congestion for two days. He requests and receives a prescription for levofloxacin, an antibiotic in the “Quinolone” category that helped him through a bout of pneumonia in the past. Five days later, he notes pain in the right knee while climbing stairs, and falls to the ground, unable to walk. Examination in the emergency department reveals complete rupture of the quadricep tendon just above the knee. He requires hospitalization and surgical repair of the tendon, with a personal co-pay of $1000 and a sevenweek painful recovery at a skilled nursing facility.” Take-home points 1) The quinolone group of antibiotics, including Levaquin (levofloxacin) and Cipro (ciprofloxacin), increase the risk for tendon rupture, especially in patients over age 60 and in those who have used prednisone recently as well as patients with kidney failure. 2) In those cases where an antibiotic is required for a patient with these risk factors, it is wise to avoid quinolone antibiotics, with many alternative choices available. 3) This patient’s initial symptoms of sore throat and head congestion did not require antibiotic therapy. With only two days of symptoms it is likely this was a viral illness so the patient and physician may have avoided the tendon rupture by holding off on antibiotics in this case. More detailed information regarding the risk of tendon rupture with quinolones is available at this link: www.ncbi.nlm.nih.gov/pmc/articles/PMC2921747/ and www.fda.gov/NewsEvents/Newsroom/PressAnnouncements/2008/ucm116919.ht m Second Caution Regarding Antibiotics: Clostridium Difficile Colitis The next topic in the antibiotic hazards series will address the risk of antibioticassociated colitis, a much more frequent and potentially life-threatening complication of antibiotic use. Colitis is inflammation of the colon, causing diarrhea, pain, and often bleeding. Consider the following story, which is typical of many cases seen daily in our acutecare hospitals: ”Mrs. N is a 70-year-old previously healthy female admitted to the hospital for evaluation of fever, cough, head congestion, and mild confusion. The chest x-ray does not show pneumonia but antibiotic injections are begun as a precaution. After three days the patient’s physical exam and x-ray continue to show no signs of pneumonia, and her fever and confusion are resolved. At that point the antibiotics are stopped and she is discharged from the hospital with a diagnosis of viral influenza. One week after hospital discharge Mrs. N develops severe diarrhea and stomach pain, and her doctor performs a stool test that reveals Clostridium Difficile colitis, prompting readmission to the hospital for intravenous fluids and Vancomycin medication. Her symptoms improve initially, but return twice over the next three months, requiring repeat treatments and causing substantial misery for Mrs. N.” Take-home points include the following: 1) Although most cases of “C Dif” colitis involve elderly hospital patients, this disease can also affect younger patients treated with antibiotics at an office or clinic. 2) Treatment failures and recurrences are common as in the case above, affecting 15 to 25% of cases, often requiring repeated hospital stays 3) Any antibiotics can trigger this complication, by killing off the normal bacteria that live in the colon, allowing overgrowth of the harmful clostridia bacteria. 4) Antibiotics should be used only when there is a clear medical indication. In this case the patient did not benefit from antibiotics, and suffered a serious complication of that treatment. Patients and their physicians should discuss the risks of antibiotic treatment in those situations where there is not good evidence for a bacterial infection. More information can be obtained on this topic at the following link: Baltimore City Local Health Improvement: Clostridium Difficile Colitis http://baltimorecity.md.networkofcare.org/ph/library/article.aspx?hwid=uf6176s pec Third Caution : Antibiotic Use in Conjunction with Coumadin (warfarin) The two previous cautions on this topic have highlighted three serious complications of antibiotic overuse: Emergence of bacterial superbugs Tendon rupture with Levaquin and Cipro, and Inflammation of the: colon caused by “C Dif” colitis The final topic in the series will focus on antibiotic drug interactions for those patients using the blood thinner Coumadin, more commonly referred to by it is generic name warfarin. Consider the following all too common scenario: ”Mrs. C is a 60-year-old patient who began treatment with warfarin four months ago because of a blood clot in her leg. Her specialist advised her not to start any new medications without authorization from her PCP, because of the risk for drug interactions. However Mrs. C notes the onset of bladder symptoms on a Saturday and decides to take just three days of trimethoprim/sulfamethoxazole (antibiotic) tablets from a prior prescription. She figures that a few days of treatment should not interfere with her blood thinner. Unfortunately she underestimates the risk and is rushed to the hospital on Tuesday with headaches and new seizures which proved to be due to a brain hemorrhage caused by excessive thinning of her blood, triggered by the combination of sulfamethoxazole with warfarin. She improves very slowly and is left with a significant paralysis of her left arm and leg.” Take-home points: 1) All antibiotics can increase the blood thinning effect of warfarin by reducing vitamin K production by bacteria in the colon 2) Several antibiotics including sulfa drugs and clarithromycin also interfere with the metabolism of warfarin in the body, and can greatly increase the blood thinning effect after just a few days of combined treatment with both drugs 3) Patients using Coumadin or warfarin should not add any new medications even for a few days without first communicating with their PCP or the nurse who manages warfarin for that PCP 4) Bladder symptoms such as burning or frequency of urination may not be related to bladder infection, so antibiotics should not be started for patients on warfarin without physician consultation to help prevent all of the complications described in this series. More information on this topic is available at www.ncbi.nlm.nih.gov/pubmed/22269622 and www.health.harvard.edu/newsletters/Harvard_Heart_Letter/2012/June/war farin-users-beware-of-antibiotics Chapter 3 Infectious Diseases Section 2 Lyme Disease: Cautions Regarding Prevention and Diagnosis Lyme disease is an infection transmitted by deer ticks that can cause short-term rash, headache, and fever as well as longer-term arthritis, meningitis, nerve injuries, and heart problems. In the United States, more than 95% of Lyme disease cases are seen in New England, New York, the East Coast states as far south as Virginia, plus Minnesota and Wisconsin. The deer tick is smaller than the common dog tick, with nymphs 1 to 2 mm in size (1/16 inch) and adult ticks 3-4 mm in length (avg. 1/8 inch) before engorgement. Experimental studies indicate that these ticks must be embedded for at least 24 hours with blood engorgement in order to transmit Lyme disease. Prevention Effective prevention in high-risk states begins with wearing long pants tucked into long socks prior to outdoor activities in thick brush or unmowed areas. The US army has learned that it’s very effective to pretreat clothing and socks with 0.5% permethrin solution, which kills ticks on contact and lasts 4-6 weeks, even after several washing cycles. This clear solution has no odor when dry and will not stain clothing. It is available from multiple well-known sources, both online and at many local market places. Using this approach an individual can prepare for an extended camping, hiking, or hunting adventure with a single treatment of clothing---much more convenient than daily application. Also recommended by Army protocol, any exposed skin should be treated prior to tick exposure with insect repellent containing 15-34% DEET, available from multiple sources and very effective also for mosquitoes. After outdoor activity in Lyme disease states, the skin should be inspected for ticks, with particular attention to the groin, waist, and armpit areas, where ticks often lodge. Embedded tick should be removed promptly, using tweezers applied near the tick’s head. It is not appropriate to attempt to “suffocate” the tick by application of Vaseline or other ointments, because the risk is reduced more reliably by immediate removal. Application of heat to the embedded tick is likewise discouraged, since it may cause the tick to regurgitate before removal, and may also cause skin burns. The removed tick should be saved in a zip lock bag for review with your physician if preventive antibiotics are desired. Adult deer ticks have a uniformly colored dark shield on their backs, unlike the multicolor shields of other ticks, but expert review may be required for precise identification. Preventive Treatments Following Exposure The Centers for Disease Control (CDC) recommends that preventive antibiotics can be considered for individuals age 8 and older after a tick bite if all the following conditions are met: 1) The patient has been exposed to ticks in one of the 13 high-risk states, or in another area with confirmed presence of Lyme disease. 2) The insect can be identified as a deer tick 3) Antibiotic treatment can be started within 72 hours of tick removal 4) The tick has been attached for at least 24 hours, as judged by the time of exposure, or by blood engorgement of the tick For those bites that qualify as above, the preferred antibiotic for preventive treatment is doxycycline, given as a single 200 mg dose, with dose adjustment for children over 8 years. The CDC does not endorse amoxicillin as a substitute for those patients unable to take doxycycline (e.g. children under age 8), because this approach is unproven, but many physicians elect this approach, using at least 7 days of amoxicillin with dosing appropriate for body weight. Proactive Monitoring Those individuals who remove a suspected deer tick should continue to watch carefully over the next 30 days for signs of early Lyme disease, such as fever, headache, or oval red blotches on the skin which enlarge, with central clearing of the blotches in some cases giving the appearance of a “bulls-eye”. This rash is called “erythema migrans” and is an important clue to Lyme disease, but in many cases the rash is absent or atypical, without the bulls-eye appearance. These tips regarding prevention of Lyme disease can spare much grief for cautious patients since, even with appropriate treatment, Lyme disease can apparently cause chronic symptoms for which there is no easy cure. Diagnosis and Treatment for Lyme Disease and its Frequent Companions In the early phase of Lyme disease, corresponding to the first 3 to 4 weeks after a tick bite, blood tests are usually negative, so physicians must rely on the history and physical exam. Patients who present with a rash consistent with erythema migrans (see part one) in a Lyme disease region should be treated for Lyme disease without waiting for blood test results, even if no tick bite was observed. If the initial blood tests are negative for Lyme disease repeat testing can be performed in 3 to 4 weeks to confirm or exclude the diagnosis. Blood testing for Lyme disease is a two-step process, with initial “enzyme immunoassay” or EIA, which detects fragments of the Lyme bacteria itself. This test doesn’t miss many cases of true Lyme disease, but it is not specific to Lyme disease so if the EIA is positive or equivocal a second “Western blot” test is needed, which is more accurate. The Western blot detects anti-bodies which the body produces to fight the infection in the early weeks, tracking so called IGM proteins and also detects the late phase IGG antibodies which are produced after the first four weeks of illness. The two-step sequence for testing may require a week for completion, and physicians may choose to begin treatment while test results are still pending. But if the test results remain negative even after four weeks of symptoms it is very unlikely that Lyme disease is the cause. It is also important to note that the CDC does not recommend testing of removed ticks for Lyme disease, in part because such testing is not reliable, but also because a tick may not have transmitted the disease even if the bacteria is found in its gut. For patients with persistent symptoms suggesting Lyme arthritis or meningitis it is possible to also perform PCR (polymerase chain reaction) testing of joint fluid and spinal fluid. The PCR test can help to confirm these diagnoses if other tests are inconclusive. But PCR testing of blood is not useful, because the test is positive in blood only briefly in early stages of the disease. The results of Western blot testing can be confusing if the patient has had symptoms for more than a month, with a positive IGM test and negative IGG testing. In such cases the diagnosis of Lyme disease cannot be confirmed, because there are frequent false positives when the IGM results are the only abnormal finding. Most of these patients have already been treated however on the basis of their IGM results. There is a syndrome similar to Lyme disease, with rash and malaise, transmitted by the “Lone Star” tick in the southwest US, but lab evidence suggests that this disease is not caused by the same Borrelia organism that is responsible for Lyme disease. So far there is no reliable lab test for this disease, which seems to respond well to doxycycline antibiotic therapy. Some patients with Lyme disease are infected at the same time by Anaplasmosis (formerly called Ehrlichiosis) or by Borrelia Miyamotoi, also carried by deer ticks. So, it is reasonable to test for these organisms when Lyme disease is suspected, since both diseases have similar symptoms and respond to the same treatment. Babesiosis is also rarely transmitted by deer ticks, but is mostly limited to the northeast coast and northern Midwest. It can be suspected when the findings include red blood cell destruction or liver disease, and when patients failed to respond to standard treatment for documented Lyme disease. The diagnosis of Babesiosis can be confirmed by blood PCR testing and microscopic examination of blood smears for the parasite. It requires dual antibiotic treatment with medications that are not used for Lyme disease. Typical Lyme disease, without meningitis, arthritis, or heart involvement, is treated with a two to three week course of oral antibiotic therapy (doxycycline, amoxicillin, or cefuroxime). Arthritis extends the treatment duration to 4 weeks. For Lyme disease affecting the brain and cranial nerves intravenous antibiotics are recommended for two to three weeks. A combination of initial intravenous and subsequent oral antibiotics is appropriate for Lyme disease affecting the heart. Published studies have revealed that there is no added value for long term (i.e., many months) antibiotics, which can lead to the development of resistant bacterial “superbugs”. More information can be found at: http://cid.oxfordjournals.org/content/43/9/1089.full and http://www.cdc.gov/lyme/ Chapter 3 Infectious Diseases Section 3 Hepatitis C: The “Baby Boomer Disease” Requires Careful Surveillance The U.S. Center for Disease Control (CDC) now recommends that blood tests be performed on all adults born between 1945 and 1965, to detect silent hepatitis viral infections which can cause liver failure and liver cancer. This news has generated significant media attention with “Baby Boomers” specifically targeted for this screening effort. The stakes are high since more than 3 million Americans are already infected with hepatitis C virus, which is the most common cause of liver cancer and liver transplants. There is been much less public discussion of two important related issues for those patients who were treated for this disease in the past. Consider the story of patient John B, a 52-year-old family man who was treated 10 years ago for hepatitis C, using the best available medications at the time. His blood virus levels responded initially, but he relapsed after completing a full year of treatment, as was the case for most treatment attempts at the time. He was then without insurance coverage for an extended period, and only recently visited his physician for fatigue and yellow eyes and was found to have cirrhosis and liver failure. A CT scan of his liver showed findings suspicious for liver cancer, which may prevent him from becoming a liver transplant candidate. Two important take-home messages are apparent here: 1) All patients with prior treatment for hepatitis C should have periodic testing to assess for liver failure and cirrhosis. When a diagnoses of cirrhosis is confirmed the patient should begin a monitoring program with liver ultrasound testing and blood “AFP” testing every six to 12 months to detect early signs of liver cancer. 2) Patients who failed prior treatment for hepatitis C, as described above for patient John B, should seek specialty consultation for consideration of second chance treatment, taking advantage of a new class of drugs called protease inhibitors. The newest of these drugs, Sovaldi for example, offer much higher cure rates, shorter treatment cycles and fewer side effects. Individuals are at increased risk for silent hepatitis C infection if they have a history of IV drug use, multiple sex partners, tattoos, or blood transfusions prior to 1986. Well-informed patients and their families are in the best position to advocate for appropriate screening and treatment for this serious disease, which is now significantly impacting the baby boomer generation. On the topic of screening for liver cancer, it should also be noted that patients with chronic viral hepatitis B or alcoholic liver disease should also be screened using the tests mentioned above if cirrhosis has been confirmed. Additional candidates for screening include those hepatitis B patients who have a family history of liver cancer, or are Asian males over 40, Asian females over 50, or Africans over age 20. Additional information on this topic can be found at: www.ncbi.nlm.nih.gov/pmc/articles/PMC3036965 and at www.webmd.com/digestive-disorders/tc/cirrhosis-treatment-overview Chapter 3 Infectious Diseases Section 4 Necrotizing Fasciitis: Diagnostic Delays are Common and Deadly Necrotizing Fasciitis (NF) is an unusual but particularly deadly infection of the soft tissues of the body, portrayed in the public media as related to "flesh-eating bacteria". The Centers for Disease Control tracks only the cases caused by a group A strep, and counts between 500 and 650 of these cases annually. But the other two types of NF, caused by clostridia and by mixtures of nasty bacteria, are more common, causing perhaps a few thousand cases annually in the US. This is the only rare disease that is addressed in this book, chosen because many cases are misdiagnosed initially, with catastrophic results for the patient. The disease is appropriately known as ”a wolf in sheep’s clothing” because in early stages the appearance is benign, without the visible clues that physicians usually see in cases of serious skin infections. There is good reason for this misleading appearance, because the primary infection is below the surface of the skin and subcutaneous fat, and tracks along the fibrous sheath covering the muscles, referenced as “fascia” in medical terms. Since the infection begins in the deeper tissues the classic presentation is with pain out of proportion to the visible findings, with no recent trauma sufficient to explain the pain. For the treating physician the picture doesn’t add up as simple skin infection, so the experienced clinician will immediately think of NF and will initiate appropriate emergency treatment. But most physicians have never seen a case of NF, and may be misled by the benign appearance, offering an erroneous diagnosis such as muscle strain and sending the patient home with some Advil. Patients with NF invariably return before long with a life-threatening progression of their infection. Another clue that can be used to identify patients with early NF is the presence of flu-like symptoms including fever, nausea, weakness, and in some cases diarrhea. These are not symptoms that should be expected in conjunction with a muscle strain, and should ring alarm bells for the treating physician who sees a patient with pain out of proportion to the visible findings. Most patients with NF also have a history of a minor break in the skin, such as an abrasion or insect bite or recent surgery. About half of these victims also have a weakened immune system due to diabetes, cancer treatment, or liver disease. But the other half are apparently healthy, including some children. A complete blood count or “CBC” test performed during early stages of NF will usually be significantly abnormal, with many immature white blood cells, and an abnormal white count which can be high or low. The following case summary depicts the typical presentation of early NF and the consequences of diagnostic error: Mrs. N was a 35-year-old type one diabetic on insulin, who scratched her right knee while doing yard work. Two days later she noted severe pain in the right thigh, making it difficult for her walk. She presented that day to an urgent care center where the nurse practitioner noted tenderness but no redness or signs of a wound infection. The patient did have a low-grade fever and nausea, so the NP performed a duplex ultrasound test looking for blood clots in the leg. That test was normal, and the patient was sent home with an antibiotic prescription for possible early cellulitis of the skin. Mrs. N returned to the nearby emergency department the next day with high fever, confusion, and low blood pressure associated with severe swelling and pain in her thigh, extending around the hip. CT scanning revealed NF, which extended from her knee to her abdomen, and she required emergency surgery with amputation of her leg at the hip. A major lawsuit was initiated by the patient, subsequently. This is a typical sequence of events for patients with NF, including the malpractice allegation. Ideal care of this patient would have included the recognition that the picture was not consistent with a minor skin infection, since the patient had severe pain and could barely walk, despite no visible redness. Alarm bells should have been ringing in the head of the clinician, and the patient and should have been sent to an emergency department promptly. As mentioned earlier however most primary care clinicians have never treated a case of NF and are not alert to this “wolf in sheep’s clothing.” The punch line here is that patients need to press for more complete assessment whenever their body is telling them that something is seriously wrong, regardless of whether the explanation is readily visible or apparent to the treating physician. With respect to NF, the cardinal warning signs need to be familiar to patients and to clinicians, as follows: First, pain out of proportion to visible findings. Some cases may show minor blisters or splotches, which still do not explain the severe pain. Second, signs of systemic illness not explained by the skin appearance (fever nausea weakness etc.) Thirdly, if available, abnormal CBC results, not explained by the skin appearance. With this pattern of findings the next step must be immediate transportation to an emergency department to be evaluated for NF, with CT or MRI scanning as well as surgical consultation. There might be a few false alarms which prove to be due to less dangerous disorders, but this urgent approach is much less likely to yield the sad outcome experienced by Mrs. N above. Patients and family members should be educated regarding this rare but deadly disease to enable them to press for appropriate emergency assessment when the key warning signs appear. Even with prompt recognition and treatment this disease can be devastating, with surgical removal of large amounts of infected tissue, and with a significant risk of death and disability. One study found that the average elapsed time to the operating room was 90 hours for fatal cases, and just 25 hours for nonfatal cases, so timely surgical intervention is critical. I have been personally involved with physician educational efforts to improve the odds for timely recognition of this disease, and would never expect patients to bear the full burden of diagnostic responsibility, but well informed patients are less likely to be content with sub-optimal management, and are more likely to collaborate with their treating clinician to reach the right conclusion regarding the need for emergency treatment for this potentially fatal disease. More information on this topic is available at: www.emedicine.medscape.com/article/2051157-obverview www.nlm.nih.gov/medlineplus/ency/article/001443.htm and a patient advocacy website with many tragic patient stories is at: www.nnff.org Chapter 4 Medication Interactions and Hazards Section 1 Opioids: Three Cautions Regarding the Tragic Overdose Epidemic First Caution: Prescription Opioid Abuse For each of the last several years in this country, deaths due to prescription opioid overdose have exceeded the combined total of deaths due to heroin and cocaine. This has been equal opportunity epidemic, with well-heeled suburban communities impacted just as often as urban slums. The common denominator is abundant availability of prescribed opioids, especially OXYCODONE (Percocet, OxyContin, Roxicet, etc.) and HYDROCODONE (Vicodin) as well as METHADONE. A few definitions should be clarified. The term narcotic is not specific to opioids and applies to any medication that dulls the senses and is a federally controlled substance. This includes opioids, cocaine, marijuana, and Valium as examples. Opioids such as heroin are derived from poppy seeds, or are created by chemists to resemble poppy extracts. With those definitions in mind consider the following tragic story, which is replayed many times daily in every state: ”Billy G is a likable 24-year-old former high school football star whose athletic career ends with a serious knee injury that required surgery. During rehab, Billy continue to request and receive Percocet for pain. The surgeon told him after six weeks that the Percocet prescriptions must stop, to prevent long-term dependence. Billy then manages to find unused Percocet tablets in his grandparents medicine cabinet and eventually starts buying tablets on the street, but can’t afford to continue that habit at $20-$40 per tablet. A friend introduces him to Heroin, which is much cheaper and far more potent. Within 3 months Billy’s entire life focuses on heroin and it is pursuit, and his doses escalate rapidly. Eventually a fatal bag of heroin laced with fentanyl proves to be Billy’s demise. His family is baffled and heartbroken.” Take home points include the following: 1) Most opioid abusers report that they started with stolen prescription drugs, often from relatives. So unused opioid tablets should never be saved. 2) Oxycodone (Percocet, OxyContin, Roxicet, etc.) is the most commonly abused prescription opioid, and all patients should be very cautious with its use, switching to non-opioid pain relievers as soon as possible. 3) Tolerance to opioids develops rapidly, with increased doses required to achieve the same effect, another good reason to limit opioid use to two weeks or less after surgery or injury. Second Caution: Risks of High Dose Opioids While it is accurate that many opioid-related deaths result from the involvement with street drugs, sadly others are caused by the overuse of prescription opioids, often in combination with alcohol or other medications. Three recent published studies have documented that the risk of death from opioid overdose increases by more than 300% for those patients using more than the equivalent of 120 mg of morphine daily. This translates to about 80 mg of oxycodone per day. Deaths can occur in patients who escalate their dose without physician authorization, or when sleeping meds or alcohol are added. In some cases medical conditions can also develop which greatly increase the risk. Consider the following scenario involving a common medical condition: Mr. J is a 48-year-old carpenter who has continued to gain weight, now at 290 pounds, translating to morbid obesity for his height 5’10”. He has chronic back pain as well with escalating doses of oxycodone, most recently up 160 mg per day. His wife notes that he snores heavily with daytime sleepiness so his physician schedules him for an overnight sleep test to look for obstructive sleep apnea (OSA). In the overnight sleep lab monitors show that the patient has “central apnea” with dangerously low oxygen levels due to long episodes with no breathing efforts. During one of those spells the patient’s heart stops requiring urgent CPR, which is fortunately successful. A similar episode at home would likely have been fatal. Take-home points include the following: 1) For noncancerous patients managed by their PCPs the dosing of chronic opioids should be limited to the equivalent of 120 mg of morphine per day when possible (see dose equivalency link at the end of this section.) 2) For patients who require higher doses the US Center for Disease Control (CDC) recommends that the care be guided by a pain specialist, who may also suggest safer approaches for pain control 3) Obese patients with signs of sleep apnea (snoring, nighttime gasping, daytime sleepiness) are at high risk for opioid related complications as described in the vignette above. Their pain symptoms should be treated very cautiously with relatively low dose opioids, pending evaluation by a sleep specialist. Patients with a confirmed diagnosis of sleep apnea who are using “CPAP” breathing devices in bed are still at high risk with opioids, because they often forget or refuse to use their CPAP device. Information on opioid dose limits can be found: http://www.nyc.gov/html/doh/html/mental/MME.html Related information can also be found: http://www.cdc.gov/mmwr/preview/mmwrhtml/mm6101a3.htm Third Caution: Opioid Dependency and Side Effects My last two vignettes on this topic highlighted death as a major complication of opioid therapy, especially for patients on high doses and those with complicating medical conditions. This edition will focus on a more complete description of opioid side effects, as a caution for those patients who are inclined to demand opioids for pain syndromes that do not respond well to these drugs. Opioids commonly cause nausea, constipation, depression, decreased sex hormone levels, and a paradoxical increase in pain levels, which is also known as the hyperalgesia syndrome. The most predictable side effect is the development of physical dependence for patients on chronic opioids. When chronic opioids are stopped abruptly the withdrawal syndrome is not life-threatening, unlike acute alcohol withdrawal, but can be compared to severe flu symptoms with chills, nausea, diarrhea, body aches and abundant misery. It is a common misconception that this syndrome is reserved for junkies and heroin addicts, but fact these symptoms can be expected by any chronic opioid patient who stops these medications suddenly, for example due to a simple oversight packing a travel bag. The following vignette demonstrates how an opioid abstinence adventure can befall any chronic user: ”Mr. J is a 48-year-old disabled former trucker with chronic backache using Percocet 60 mg per day for pain control. Returning from Florida in February, he gets stranded at the Baltimore airport because of a blizzard. He had planned to refill his prescription at home the next day, but is still stuck at the airport hotel, with no transportation options due to the storm. Within 24 hours. he begins to experience the full opiate withdrawal syndrome with whole body pain, diarrhea, and anxiety. He is unable to find a local physician willing to prescribe Percocet over the telephone, and Maryland pharmacies will not accept a Percocet telephone authorization from his physician in Massachusetts (this regulatory restriction applies in all states). He is finally able to renew his prescription at home three days later, after spending three sleepless nights which he described to his doctor as “Hell on Earth”. Take-home points include the following: 1) Long-term opioid therapy is associated with many risks so alternate methods of pain management should be attempted first, including acupuncture, customized rehab programs, alternate medications, and stress management as appropriate. 2) Medical specialists agree that opioids should be avoided for non-cancer patients with pain due to headache, abdominal distress, and fibromyalgia, because these patients are likely to fare poorly with opioids (see Mayo Clinic link at the end). Patients may be able to find a PCP willing to prescribe opioids for these problems, but all parties are likely to regret that decision the long run. 3) Chronic back pain is the most common condition for which opioids are prescribed, but published studies revealed that long-term outcomes are poor, with all the hazards described in my last three sections but with no significant improvement in functional status. For this reason many physicians decline to prescribed opioids for long-term back pain, since the potential harms appeared to exceed the benefit. More information on this topic may be found at: http://www.addictionsandrecovery.org/opiates-narcotics-recovery.htm http://www.mayoclinic.org/diseasesconditions/fibromyalgia/basics/definition/con-20019243 http://www.rheumatology.org/Practice/Clinical/Patients/Diseases_And_Condition s/Fibromyalgia/ Chapter 4 Medication Interactions and Hazards Section 2 NSAIDs: Three Cautions Regarding the Most Dangerous of All OTC Meds First Caution: Ulcer Risk Over the Counter (OTC) pain medications such as Advil, Aleve, and aspirin are used by millions of Americans without direct medical supervision, but these medications frequently cause bleeding ulcers of the stomach and intestine, as well as high blood pressure, kidney disease, heart failure, and strokes. This section will focus on the ulcer problem, which affects up to one of every four patients who use these meds over an extended period of time. These three medications are part of a much larger group of “non steroidal antiinflammatory drugs”, also called “NSAIDs”, most of which still require a prescription. Commonly prescribed NSAIDs include ibuprofen (brand names Motrin and Advil), naproxen (brand names Naprosyn and Aleve), and piroxicam (brand name Feldene). All of these drugs share a similar risk profile. A detailed listing of all NSAIDs , their costs , and doses can be found in the Consumer Reports reference at the end of this section . Although Advil, Aleve, and aspirin are available without a prescription the NSAID meds are among the most dangerous drugs on the market, causing ulcer complications that result in more than 100,000 hospitalizations each year in the US, and 7,000 to 10,000 deaths. Only the opioid category of drugs causes more deaths annually. The following true case history is typical: Mrs. J, a 68 year old smoker, began using OTC Advil for knee pain, and did well for 3 weeks, but then noted the sudden onset of bloody bowel movements, causing her to be hospitalized in the intensive care unit. She received multiple blood transfusions, and tests revealed a bleeding stomach ulcer. Emergency surgery was required to stop the bleeding, complicated by a post-op wound infection and a long rehab period. Mrs. J survived, but with a large surgical site hernia and more than $1500 in out of pocket expenses. The risk for ulcer complications such as bleeding and perforation is highest in those with one or more of the following risk factors: 1) Age over 65 2) History of prior bleeding ulcer or perforation 3) Any prior ulcers 4) Smokers, and those who ingest 3 or more alcohol beverages daily 5) Patients taking prednisone or blood thinners 6) Patients using the maximum doses of NSAIDs 7) Patients with disabling chronic illness such as emphysema/COPD, Heart failure, Rheumatoid Arthritis, or Kidney Failure In addition there is double risk for those NSAID users who also take daily aspirin because of coronary heart disease, i.e., increased risk for heart attacks as well as increased risk for ulcer complications. The non-profit Consumer Reports organization was so impressed with the risk of NSAID meds that they took the unusual step of posting online a free 27 page document outlining the hazards along with recommendations for risk reduction. The American College of Gastroenterology (medical specialty society for stomach and intestinal disorders) and the Academy of Family Practice have also posted recommendations for safer use of NSAIDs. I have referenced these documents at the end, and offer the following summary of those recommendations, sorted by patient category: 1) Healthy patients under age 65…no history of ulcers or risk factors: OK to use NSAIDs, best limited to 2 weeks with the minimum effective dose except by physician request 2) Patients with one or two risk factors from the list above: It is safest to combine the NSAID with a drug that reduces stomach acid such as OTC Prilosec once daily to prevent ulcers. 3) Patients with 3 or more risk factors from the list above, or with any previous ulcer bleeding or perforation: Avoid Advil, Aleve, aspirin, and all other NSAIDs., with the possible exception of celecoxib (brand name Celebrex), which should be combined with Prilosec if your physician approves. 4) Patients with Coronary Heart Disease on daily aspirin: The safest NSAID for coronary patients is probably naproxen/Aleve, which should be combined with Prilosec unless the pt is also on clopidogrel (brand name Ticlid)-- for stents, in which case Protonix should substitute for Prilosec. However if the patient also has 3 or more risk factors it is safest to completely avoid NSAIDs including celecoxib. NSAIDs in gel form represent another reasonable option for patients with risk factors. Voltaren 1% Gel is available by prescription in the US and multiple studies have confirmed that it is as effective as oral NSAIDs for degenerative arthritis involving the knees and hands, with much lower risk for complications such as ulcers. It is however expensive, so check first with your insurance company. In my medical director roles over the past 25 years I have dealt with dozens of cases of serious bleeding ulcers and perforations caused by these NSAID medications, so I sincerely hope that the sharing of these safety guidelines can help to prevent some of these life-threatening events. In that regard, it is also important to note that many patients who suffer these complications (including Mrs. J in the case summary above) have no warning symptoms such as abdominal pain or indigestion. The first sign of NSAID trouble can be vomiting of blood or the severe pain associated with ulcer perforation. So this is another example of a situation where a few ounces of prevention are preferable to a ton of attempted cure. More information on this topic can be found at: www.gi.org/guideline/prevention-of-nsaid-related-ulcer-complications/ www.consumerreports.org/cro/news/2013/07/treating-pain-with-nsaidmedications www.aafp.org/afp/2009/1215/p1371.html Second NSAID Caution: Strokes and Heart Attacks This risk proves to be much smaller statistically as compared to the risk for ulcers, but has attracted much media attention. The NSAIDs that demonstrate the most cardiovascular risk, i.e., diclofenac and celecoxib, may cause 2-3 strokes or heart attacks annually for every 1000 treated patients. The cardiovascular risk for Advil/Motrin/ibuprofen is even lower, perhaps one stroke or heart attack annually for every 1000 treated patients. So this risk is very small compared to the ulcer risk with NSAIDS, which is 1-2 episodes of bleeding or perforation annually per 100 treated patients, fully 10 times greater than the risk for cardiovascular complications. Given this information many patients may reasonably choose to continue to use NSAIDs for pain relief despite the small risk of stroke or heart attack, particularly since one NSAID, i.e. naproxen, appears to have very little cardiovascular risk based on studies to date. There is however a special caution required for those patients with established coronary heart disease who need to take one aspirin daily for preventive purposes. It turns out that Advil (also called Motrin or ibuprofen) blocks the preventive effects of aspirin when these drugs are ingested together. The FDA reports that this undesirable interaction can be prevented if the ibuprofen is ingested at least 8 hours before the aspirin or at least one hour later. Low dose Aleve/Naprosyn is probably also safe for these patients, regardless of the timing of ingestion. Two NSAIDs have been removed from the market by the FDA because of a particularly high risk of cardiac complication. Those discontinued drugs, both in the “COX-2” category, are Vioxx and Bextra. The only remaining drug in this class is celecoxib (brand name Celebrex), which still carries a “black box” warning from the FDA regarding cardiac risks. So far the cardiovascular risk with celecoxib seems to be similar to several other NSAIDs. This is fortunate, since celecoxib is the only available NSAID with relatively low risk for stomach and intestinal ulcers, as mentioned in Part I. All factors considered, patients should be very cautious in their use of NSAIDs, using these drugs at the lowest possible dose and for the shortest tolerable duration of treatment. Patients with previous coronary heart disease or stroke are at highest risk. These cautions are based on the risk information summarized above, plus the observation that NSAIDs also increase blood pressure and increase the risk of kidney disease, as discussed in the next section. More information on the cardiovascular risks of NSAIDs can be found at: www.medicinenet.com/script/main/art.asp?articlekey=124660 www.ncbi.nlm.nih.gov/pubmed/21224324 www.circ.ahajournals.org/content/117/17/e322 Third NSAID Caution: Risks of High Blood Pressure, Kidney Disease, and Liver Disease Many published studies now confirm that all NSAIDs, including over-the-counter Advil and Aleve, raise blood pressure levels by 3 to 5 points on average with longterm use, with much variation among individuals. Patients already on treatment for high blood pressure (a.k.a. hypertension) seem to be most sensitive to the impact of NSAIDs. Raising blood pressure by just a few points may seem like little cause for concern, but we know from outcome studies that elevations of this magnitude can increase the risk of stroke and heart attack by more than 15%. It is probably not a coincidence that the actual rates of stroke and heart attack among NSAID users are 15-30% higher than expected, as discussed previously. NSAIDs reduce the ability of the kidneys to clear salt and water from the body, and therefore interfere with the diuretic category of blood pressure medications (like HCTZ), which are the most commonly used drugs for blood pressure control. All NSAIDs have this drawback, but the impact can be limited by using these meds at the lowest tolerable doses. Patients whose control levels are not acceptable should let their physician know if they are using any over the counter NSAIDs. For patients with high blood pressure who can’t avoid the use of NSAIDs there is some evidence that the class of BP meds called “calcium channel blockers” may be least sensitive to the effects of NSAIDs, with improved BP control. NSAID medications also interfere with normal kidney function, beyond the simple reduction of salt and water excretion. These drugs reduce prostaglandin production in the kidney, and thereby reduce kidney blood flow. This reduction in blood flow has little impact for healthy individuals, but if the patient already has smoldering kidney disease, or if the patient is dehydrated or has low blood pressure because of infection, bleeding, or surgery, the NSAID may help to cause serious kidney failure. In rare cases NSAIDs may also cause inflammation in the kidneys that can lead to kidney failure due to “interstitial nephritis” NSIADs can less commonly cause serious liver disease, affecting just a few patients for every 100,000 users. These rare reactions seem to be related to unexpected activation of the immune system of the liver, but mild abnormalities of liver function are seen in 10-15% of long-term NSAID users. These low-grade abnormalities are not dangerous, but multiple (expensive) tests may be required to confirm that there is no other cause of the abnormal liver tests. Take Home Points: 1) NSAIDS can cause high blood pressure, and can impair BP control for those on treatment with commonly used BP meds. 2) Notify your PCP if you are using any over the counter NSAIDs for 10 days or more, as recommended on the package insert 3) Stop NSAIDs before surgery, and with any serious illness 4) Avoid NSAIDs completely if you have chronic kidney or liver disease, in consultation with your physician More information regarding NSAIDs and blood pressure is available at: www.ncbi.nlm.nih.gov/pubmed/9391772 www.johnshopkinshealthalerts.com/reports/hypertension_stroke/2933-1.html www.kidney.org/atoz/content/painMeds_analgesics Chapter 4 Medication Interactions and Hazards Section 3 Hyperkalemia: Drug Interactions That Can Prove Fatal Potassium is an essential element in the body, but more than 95% is contained within human cells, with very small amounts circulating freely in the blood. The heart is very sensitive to increases in blood potassium levels, and fatal slowing of the heart can be seen when blood potassium levels rise above 6.5 meq/Liter (“Hyperkalemia’). Unfortunately, there are several commonly prescribed drug combinations that can increase blood potassium to dangerous levels, as in the following actual case summary: Mrs. M, A 75-year-old patient with high blood pressure, decreased kidney function and diabetes, was prescribed enalapril (brand name Vasotec) plus a combination diuretic called HCTZ/triamterene for control of her blood pressure, and her blood potassium level was just at the upper limits of normal on this program. Two months later she noted a flare of knee arthritis for which another physician prescribed celecoxib (brand name Celebrex), a drug in the in the “NSAID” category. The following month she felt weak and had a fainting spell, which prompted an emergency department visit, where her blood potassium level was 7.0, dangerously high, with drastic slowing of her heart rate. Physicians were able to reduce the potassium to safe levels, and she was advised to stop the celecoxib and switch the diuretic to HCTZ, to avoid future similar problems. This story is typical for patients who get into trouble with high potassium levels, a complication usually triggered by two or more of the following risk factors: 1) Blood pressure drugs in the ACE (like Zestril, Lisinopril, Vesotec, Enalapril, captopril) or ARB category (like Cozaar or Valsartan) 2) Potassium sparing diuretics (like Dyazide, Maxzide, Triamterene, Spironolactone) 3) Potassium supplements (like KCL, Kaon CL, etc) 4) Diabetes 5) Decreased kidney function 6) High potassium diet (lots of tomatoes, spinach, bananas, meat, etc.) 7) Arthritis “NSAID” medications (Advil, Aleve, Motrin, Celebrex, ibuprofen, naproxen, etc.) It is common for patients like Mrs. M to percolate along with mildly elevated potassium levels on their interacting drugs until another factor is introduced. In her case the arthritis drug was the tipping point that pushed her level to the danger zone. In other cases, the cause could be a decrease in kidney function due to dehydration or surgery or infection, as examples. The second reference link at the end of this summary describes a European study that confirmed that two-thirds of serious cases of hyperkalemia in hospitals were caused by drug interactions, and 75% of these patients also had decreased kidney function. Once the potassium level rises above 6.5 the heart can fail quickly unless physicians recognize the problem and administer emergency treatment. Hyperkalemia is the likely cause of many cases of unexplained sudden death in the hospital, since patients who require CPR are expected to have high potassium levels because of early cell death and acidosis. The third resource linked at the end of this summary describes a case very similar to Mrs. M, where physicians were able to recognize the problem and save the patient. Other patients on these drug combinations may not be as fortunate. Patients who have two or more of the risk factors listed above should be attentive to the potassium results shared by their physicians, with discussion of possible modifications of their medications when potassium values climb into the high normal range, above five. At these levels there’s very little safety margin, if the patient becomes sick in any fashion causing dehydration, or if the patient starts taking over-the-counter arthritis pills. Kidney function can also decrease gradually with aging, causing one of the risky drug combinations to be less well-tolerated, with rising potassium levels. These drug safety adjustments can usually be easily accomplished by changing to a different diuretic medication, or by stopping potassium supplements. In other cases it may be sufficient for the patient to make dietary adjustments, avoiding overuse of bananas, tomatoes or spinach, as examples. In summary, this is a situation where engaged patients can work with their physicians to improve the safety of their medication program, avoiding hyperkalemia, which is one of the most serious of all medical mishaps. More information this topic is available at: www.ncbi.nlm.nih.gov/pubmed/24150532 www.ncbi.nlm.nih.gov/pmc/articles/PMC1413606 www.ncbi.nlm.nih.gov/pubmed/12113843 Chapter 4 Medication Interactions and Hazards Section 4 Beta Blockers: No Longer Preferred for Blood Pressure Control Beta blocker medications, such as atenolol, propranolol, and metoprolol, are very useful drugs that have been shown to improve medical outcomes for patients with heart attacks, heart failure, and heart palpitations due to rapid heartbeat. The most frequent reason for beta blocker use however is the management of high blood pressure, a practice which has fallen out of favor in the updated guideline for blood pressure treatment from the Joint National Committee on Hypertension, Version Eight (“JNC-8”). (see the first reference linked at the end) High blood pressure, also known as hypertension, is caused by an increase in resistance to bloodflow through the body’s small blood vessels. But beta blockers do not decrease this resistance to bloodflow. In fact many beta blockers actually increase the resistance, causing cold extremities or bluish finger discoloration. These drugs decrease blood pressure by decreasing the pumping function of the heart, with less blood flowing out to the body’s arteries. Elderly patients who are relatively inactive usually tolerate this decrease in cardiac output without difficulty, but younger patients often note fatigue and lack of energy, especially with activity, as demonstrated in this typical case history: Mr. T, at age 35, was started on the medication atenolol by his PCP for persistent high blood pressure. His BP decreased along with his pulse rate, but he noted extreme fatigue, and had difficulty completing his normal exercise sessions at the gym. In addition he noted that his fingers tended to become cold and dusky colored during winter months. These symptoms all resolved when his PCP switched him to lisinopril for control of his blood pressure. Studies of the treatment of high blood pressure also confirm that beta blockers are not as effective as other medication categories, including water pills (diuretics like HCTZ), ACE inhibitors (like lisinopril), calcium channel blocker (like diltiazem and felodipine), and ARB drugs (like valsartan). So the new JNC -8 guideline recommends that beta blockers be used for high blood pressure only if these other four categories of drugs fail to achieve good blood-pressure control for the patient. Those patients who have already achieved good blood-pressure control using a beta blocker, without significant side effects, do not need to consider changing their medication. They need to be aware however the beta blockers can also cause other side effects such as constipation, sleep problems, and worsening of the symptoms of asthma or COPD. With respect to the asthma and COPD symptoms it is possible to decrease these side effects by switching to a beta blocker which does not affect the lungs, such as atenolol, metoprolol, or bisoprolol. For patients who encounter problems with fatigue or exercise intolerance on beta blockers it is also possible to switch to a combination drug with beta blocker and alpha blocker properties. Such drugs include labetolol and carvedilol. These medications are less likely to cause fatigue but must be taken twice daily. Many patients require a combination of several drugs to control their blood pressure. The combination of a beta blocker plus diltiazem or verapamil, both calcium channel blockers , turns out to be dangerous because all of these drugs tend to decrease the pulse rate. Some patients on a beta blocker plus verapamil or diltiazem suffer very low heart rates which can cause fainting spells and falls. So this particular drug combination is best avoided. (see also the third reference link at the end) Although I describe above some drawbacks related to the use of beta blockers for blood pressure, it is important to recognize the value these medications provide for patients with heart failure and heart attacks. For these conditions beta blockers provide protection to the heart, reducing the strain associated with activity and stress, with proven reduction in death rates. In similar fashion beta blockers have also improved outcomes for patients at high risk for heart disease who need major surgery. So the only situation where physicians are moving away from beta blockers is for management of high blood pressure in patients without heart disease, particularly when fatigue, lack of energy, or shortness of breath prove to be side effects of treatment. More information on this topic is available at: www.ncbi.nlm.nih.gov/pubmed/24352797 www.ncbi.nlm.nih.gov/pubmed/11406056 www.cvpharmacology.com/vasodilator/CCB.htm Chapter 4 Medication Interactions and Hazards Section 5 Insulin: Essential but Hazardous Therapy Diabetes is caused by failure of the pancreas gland to produce enough insulin to meet the needs of the body, causing blood glucose or “sugar” levels to rise to dangerous levels. So insulin therapy can be life-saving for diabetics, and experts recommend that insulin injections be started sooner than was customary in past years, to prevent the complications of diabetes. However insulin can also cause dangerous drops in blood glucose levels (“hypoglycemia”) if not used properly, as noted in the following actual case summary: Mr. N is a 56-year-old obese diabetic whose diabetic pills were no longer keeping his blood glucose levels below 200. His physician began insulin therapy, initially with a single bedtime dose and later with three daily doses, following patient education sessions with a nurse. Three months later Mr. N was having a hectic day, and decided to skip lunch. One hour later he became shaky, sweaty and faint, causing him to drive his car off the road into a tree. EMTs at the scene noted his diabetic bracelet and checked his blood glucose, finding it to be very low at 35. Mr. N suffered a broken leg and ruptured spleen as a result of this accident, requiring surgery and prolonged recovery. The take-home message here is that insulin therapy requires careful adherence to treatment plans and a strict meal schedule, with the use of glucose supplements for symptoms of low blood sugar or hypoglycemia. Patients on insulin therapy should keep with them at all times emergency glucose supplements, either tablets or gel as recommended by the physician, since it is not always possible to stick with a strict meal schedule. Hypoglycemia can also become a problem when the patient injects insulin in the thigh or upper arm and then engages in vigorous exercise, which speeds the absorption of insulin from the skin. Low blood glucose can also happen if the patient confuses insulin types (long-acting versus short acting) or if the patient loads too much insulin in the syringe by mistake. Prevention of these errors is best accomplished with careful patient education when insulin is started, preferably with a certified diabetic nurse educator, who will also reinforce the importance of home glucose measurement and tracking. As part of this training process each diabetic who starts insulin should also be provided with “sick day rules”, with instructions for insulin dosing and home glucose measurement to handle those days when illness or medical procedures prevent normal eating patterns. It’s difficult to prevent all cases of human error, so family members can prepared to deal with possible episodes of hypoglycemia by learning how to use an emergency syringe containing prescription glucagon. This kit can be life-saving if the blood glucose is so low that the patient is unconscious are having seizures, preventing the use of oral glucose tablets, gel, or juice. Use of the glucagon kit requires training in a medical office, since the solution must be mixed before injection. Glucagon itself is safe for use by laypersons, and will not cause injury to the patient even if the blood glucose was higher than expected before the injection. Although I have described some serious potential complications of insulin therapy, it is important to recognize that insulin is still the correct choice for all type one diabetics and for those type two diabetics for whom oral medications are no longer providing good glucose control. For these patients insulin is the only way to prevent irreversible end stage complications of diabetes including blindness, kidney failure, painful nerve damage, and leg amputations. More info on these topics can be found at: http://www.diabetes.org/living-with-diabetes/treatment-and-care/blood-glucosecontrol/hypoglycemia-low-blood.html?loc=lwd-slabnav http://www.mayoclinic.org/diseases-conditions/diabetichypoglycemia/basics/prevention/con-20034680 Chapter 4 Medication Interactions and Hazards Section 6 Diuretic risks: Commonly Overlooked Pitfalls The following scenario describes an avoidable medical mishap that is seen frequently in community emergency departments: Mrs. D is a 75-year-old active woman whose physician manages her blood pressure with two medications, lisinopril and hydrochlorothiazide (HCTZ). The first medication works by adjusting the level of a hormone that controls blood pressure, and the second is a water pill or diuretic that increases the flow of urine from the kidneys. Her blood pressure is well controlled on this program with no side effects, and she continues those meds while traveling with her children to Mexico for a holiday vacation. Unfortunately she develops traveler’s diarrhea, prompting a trip to a Mexican clinic where she is prescribed antibiotics and Lomotil. After three days, the diarrhea is only slightly improved, and Mrs. D becomes confused with inability to speak clearly and with recurrent falls, causing her to fracture her wrist. Evaluation at the local hospital reveals that her blood sodium level is dangerously low and she is also severely dehydrated, requiring two days of IV fluid replacement. The fractured wrist remains painful for several months. The take-home message is that this medical mishap likely could have been avoided if Mrs. D had stopped the water pill when she developed diarrhea, since the combined effect of gastroenteritis and a diuretic can cause severe dehydration. Patients on diuretics should specifically ask their healthcare provider whether to continue these medications during episodes of diarrhea or vomiting, especially since many of these episodes are managed over the phone, and may be handled by staff who are not familiar with the patient’s medications. After the diarrhea or vomiting has resolved the blood-pressure medications can be safely restarted in most cases, but all changes or interruptions of medications should be reviewed with your healthcare professional. More powerful diuretics such as Lasix or furosemide are often used for patients with heart failure or leg swelling. These patients also need to be aware of the risk of dehydration if they develop vomiting, diarrhea, or decreased food intake. A medical office visit may be required to determine whether lab tests and interruption of diuretic therapy is appropriate in these situations. Chapter 4 Medication Interactions and Hazards Section 7 Amiodarone/Cordarone: Controls Heart Rhythm, But Requires Close Monitoring The medication amiodarone, sold under the brand name Cordarone, is used to control serious heart rhythm problems such as ventricular tachycardia or atrial fibrillation. It is used long-term for these heart rate problems, but can also be used short-term after cardiac surgery to suppress atrial rhythm problems. When this drug is used long-term (more than 30 days), it can cause an unusual combination of side effects and complications, as is evident from the following case summary: Mr. A’s cardiologist started him on treatment with amiodarone to prevent recurrent atrial fibrillation, which had been treated previously with electrical cardioversion. Mr. A was advised to have annual chest X-rays and blood tests to check for complications from the drug. This monitoring was performed after 12 months, but Mr. A then moved to another state and did not continue the testing. Two years later, he presented to his new PCP with cough and shortness of breath, and was found to have pulmonary fibrosis in both lungs, which was irreversible and disabling, caused by the amiodarone. He was also found to have a severely under active thyroid gland, also a complication of amiodarone treatment. This patient encountered two of the recognized serious complications of amiodarone therapy, i.e. lung disease and thyroid disease. The drug is also known to cause the formation of unusual deposits on the cornea of the eye, as well as liver inflammation that rarely progresses to liver failure. For these reasons, patients on long-term amiodarone should have the following monitoring procedures at least annually, per the package insert from the drug manufacturer: chest x-ray liver function test thyroid function tests exam with an eye care professional. Cautions are also appropriate for sun sensitivity, with consistent use of sunscreen to prevent skin reactions on this drug. Since amiodarone tends to decrease the heart rate, it should not usually be combined with medications such as beta blockers, verapamil, or diltiazem, which also decrease the heart rate. In addition, it should not be used with quinolone type antibiotics such as Levaquin and Cipro because these medications increased the cardiac effects of amiodarone. When amiodarone is used in combination with the blood thinner Coumadin (warfarin for generic users), it increases the bleeding tendency. Therefore, warfarin doses must be adjusted accordingly. Many other drug interactions have also been reported with amiodarone, so the prescribing cardiologist should be informed when a patient receives a new medication prescription from another clinician (see also the long list of drug interactions cited in the Mayo Clinic link at the end of the reference list below). It is also essential for the patient to clarify whether the cardiologist or the primary care physician will be accepting responsibility for ordering and tracking the annual chest x-rays, blood tests, and eye exams which are required with this medication. Otherwise, these important monitoring steps may ”fall through the system cracks” as was the case for the unfortunate Mr. A described in the case vignette above. More information on this topic is available at: www.nlm.nih.gov/medlineplus/druginfo/meds/a687009.html and www.mayoclinic.org/drugs-supplements/amiodarone-oral-route/precautions/drg20061854 Chapter 4 Medication Interactions and Hazards Section 8 Medication Hazards During Pregnancy The US Center for Disease Control (CDC) reports that medication use in pregnancy has increased more than 60% over the last three decades, with nine out of 10 pregnant women now using at least one prescription or over-the-counter medication. The average maternal age has also increased over this time, and older women are more likely to have chronic medical problems that require medication therapy. This increased medication exposure creates a dilemma for patients and their obstetric clinicians, because fewer than 10% of all medications have been tested adequately for risks during pregnancy. The FDA has however compiled a rating system for medications in pregnancy, so women can at least be informed regarding the short list of medications that are known to be highest risk. These problematic drugs fall into two FDA categories: Category “D” - with documented negative impact on the fetus, but with some cases where the drug’s benefit may exceed the risk, as judged by the physician. Category “X” - with no justifiable use in pregnancy. Some examples of Category D drugs included the following: Seizure medications such as Depakene (generic valproic acid) and Tegretol (carbamazepam). These are known to cause birth defects, but might still be needed if the mother’s seizures can’t be controlled with other drugs. Blood Pressure drugs in the “ACE” and ”ARB” categories, such as Zestril (lisinopril) and Cozaar (losartan). These drugs decrease fetal amniotic fluid after the first trimester with negative impact on the fetus, but might be needed if the mother’s BP cannot be controlled with other meds. Lithobid and Eskalith (lithium) therapy for manic depression. Lithium can cause fetal heart defects and thyroid disease, but might be continued with careful monitoring if the manic illness can’t be controlled with other meds. Tetracycline antibiotics, including Vibramycin (doxycycline). Cause no harm in early pregnancy, but can cause fetal tooth and bone defects when used in the second and third trimester of pregnancy. Examples of Category “X” Drugs, which should always be avoided in pregnancy: Accutane for acne - no longer made in the US but available in generic form as isotretinoin. Causes serious birth defects. Cholesterol drugs in the “Statin” category, such as Lipitor (atorvastatin) and Zocor (simvastatin). These are not known to cause birth defects, but are not appropriate in pregnancy where cholesterol levels need to increase to support fetal development. Blood thinner Coumadin (generic warfarin)…This drug causes birth defects in early pregnancy and bleeding complications in late pregnancy. Newer blood thinners are available for those pregnant women who require anticoagulation to treat or prevent blood clots…a safer alternative to warfarin. The medications mentioned above are not a complete list of all drugs which increase risk to the fetus, and all pregnant women should communicate with their obstetrician before starting any over-the-counter or prescription medication. The online resources listed at the end of this section can also provide additional useful information on medication hazards during pregnancy. The risk of recreational substances during pregnancy is also a serious nationwide problem, with the following well-publicized concerns: Smoking - makes it more difficult to get pregnant and increases the risk for premature delivery and several other serious pregnancy complications. Smoking during and after pregnancy also increases the risk of asthma and respiratory infections for the child. Cocaine and methamphetamines or “Speed” - Increase the risk of miscarriage and premature birth, as well as birth defects and withdrawal symptoms for the newborn baby. Alcohol - There is no safe amount of alcohol intake for a pregnant woman. It is well established that alcohol can cause mental retardation and facial abnormalities in the child, and newborns can also suffer with alcohol withdrawal syndrome. Marijuana - does not appear to cause birth defects, and there is conflicting evidence whether it can cause low birth weight and premature birth with related complications. At this point the American College of obstetricians recommends that marijuana be avoided during pregnancy. Additional information on these topics is available at: www.mothertobaby.org/fact-sheets-parent/ www.cdc.gov/pregnancy/meds/treatingfortwo/facts.htm Chapter 5 Risks with Transitions in Care Section 1 Hospital Discharge Plans: Hand-offs Are Often Mishandled Many research studies have confirmed that patients are at risk for medical adverse events when care transfers from hospital to home or from one facility to another. Primary care physicians in most communities no longer provide hospital care, delegating those duties to hospital-based physicians. This system is more efficient but does create opportunities for communication errors that can impact the patient. Medications are commonly changed during the hospital stay, so it is essential that the discharge planning process include careful attention to the medication list and any related follow-up care. The following case summary highlights the consequences of confusion at the time of hospital discharge: Mrs. J is a 60-year-old patient admitted to the hospital for treatment of a rapid and irregular heartbeat called atrial fibrillation or A-Fib. She begins treatment with propranolol medication to slow her heart rate, and warfarin to prevent blood clots. Attempts to convert her heart rhythm back to normal are unsuccessful however so she is sent home on these two new medications with instructions to have her blood tested by her PCP within three days. When she calls for that appointment the office cannot book her until eight days later. On day seven, unfortunately, she is readmitted to the hospital with a severe nosebleed due to excessive anticoagulant effect from her warfarin, and then develops aspiration pneumonia as an additional complication. Take-home points: 1) Those patients who cannot be seen for follow-up office appointment shortly after hospital discharge are at risk for medical mishaps, a finding recently confirmed by Boston University researchers (see first link below). 2) Since many communities are now underserved by adult PCPs, with long waits for appointments, it may be appropriate to schedule the first hospital followup visit with a specialist who saw the patient in the hospital, for example the cardiologist in the case described above. 3) Many hospitals do not schedule follow-up visits, so patient and/or family members are wise to make phone calls to find an available follow-up appointment before leaving the hospital (may be difficult to identify the specialist seen in the hospital after discharge to home). 4) Warfarin medication requires careful blood monitoring, especially during the first week of treatment when dose changes are frequent. Newer blood thinners such as Pradaxa and Xarelto do not require monitoring of blood tests and can be discussed as a more convenient alternative treatment for patients with AFib or blood clots. The Agency for Healthcare research and Quality has also publicized a guideline for safer care transitions from hospital to home, titled the “IDEAL” Discharge Planning Program. This acronym is explained as follows: Include the patient and family as full partners in the discharge planning process Discuss with them the 5 key areas to prevent problems at home: Describe what life will be like at home Review and reconcile medications with the list of meds used prior to the hospital stay Highlight warning signs and symptoms Explain test results Make followup appointments Educate the patient and family in plain language about the patient’s condition, the discharge process, and the next steps at every opportunity throughout the hospital stay. Assess how well doctors and nurses explain the diagnosis, condition, and next steps in the patient’s care to the patient and family, and use “teach back” techniques to confirm understanding Listen to and honor the patients and family goals, preferences, and concerns. Patients and their family members should expect attention to all of these “IDEAL” standards by hospital staff at the time of discharge, and should speak up if any of these steps seem to be missing from the process, to decrease the chances for any medical mishaps after discharge Additional information about best practices for hospital discharge coordination can be found at: www.bu.edu/fammed/projectred/components.html The review of blood thinners that do not require lab monitoring can be found by clicking here. And more information regarding the “IDEAL” discharge process can be found at www.ahrq.gov/professionals/systems/hospital/engagingfamilies/strategy4/index. html Chapter 5 Risks with Transitions in Care Section 2 Incidental CT Findings: A Frequent Source of Lawsuits Computerized tomography (CT) scans have enabled clinicians to more accurately diagnose many conditions, but they are also capable of detecting unexpected incidental findings that can create the opportunity for medical mishaps. CT scans may be ordered by a physician in the emergency department or in the hospital; most likely, this same physician will not see the patient for follow-up care. In that case, the CT report may not be forwarded to the primary care physician. In this scenario it is challenging to ensure that incidental CT findings such as an unexpected lung nodule are tracked with follow-up CT scans and other tests as appropriate. Consider the following actual case that highlights the importance of careful CT follow-up: Mrs. J presented to her local hospital with appendicitis, confirm by CT scan. The surgeon removed her appendix through a tiny incision with an endoscope, and sent her home the following day. The final CT report, which was not available until after the patient’s discharge to home, revealed an incidental nodule in the kidney. This information was not relayed to the patient or to her primary care physician. Fortunately, the patient returned to her physician four months later for a Pap smear and he noted the CT report and sent Mrs. J to a surgeon who removed a kidney cancer. The cancer had not yet spread beyond the kidney but would likely have become far advanced except for the diligence of Mrs. J’s PCP. Many patients in similar circumstances are not as fortunate. Although this case involved a kidney mass it is also common for CT scans to detect unexpected findings in the lungs, liver, adrenal glands and blood vessels. It’s also important to note that these incidental findings are not confined to CT scans. A study by Dr Sayon Dutta published in the Annals of Emergency Medicine in August 2013 revealed that 4.5% of all radiology reports generated in a large ED included recommendations for followup of incidental findings, but only 49% of discharge instructions for those patients included any mention of the needed followup. Similar findings were reported from SUNY Upstate Medical Center (see linked reference at the end). Healthcare systems are working to improve their tracking mechanisms to prevent cases like the one described for Mrs J above, but with the fragmented nature of current medical care it is safest for patients to become more directly engaged with their healthcare records as follows: Take-Home Points: 1) Take advantage of any opportunity to get direct access to your electronic health record, including copies of x-ray and test reports. Many physician offices now offer this online access. 2) At the time of hospital or emergency department discharge ask whether any test results are still pending, and confirm that you and your physician will be notified regarding those results. 3) When you are advised verbally regarding a result of any CT scan, ask specifically whether there were any incidental findings which need follow-up. Actively engaged patients are less likely to be the victims of a medical mishap due to follow-up failures like the one described above. More information on this topic is available at: www.hindawi.com/journals/emi/2011/624847/ Chapter 5 Risks with Transitions in Care Section 3 Specialist “Hop Scotch”: Can Lead to Mishaps Consider the following true story that involved a close relative: Mr. K underwent surgery for colon cancer, with a prolonged recovery, notable for poor appetite, bloating, and persistent abdominal discomfort. At the same time, he was undergoing treatment for rheumatoid arthritis for which his arthritis specialist had prescribed prednisone tablets at 10 mg daily. There was no communication, however, between the surgical and arthritis specialists, and the primary care physician (PCP) had not yet received a rheumatology note listing the new prednisone medication. When Mr. K returned the third time to the surgical office with his post-op complaints, he was advised that infection was unlikely because he had no fever. But, Mr. K’s prednisone therapy was in fact masking his fever, and masking also the signs of inflammation in his belly. After another week of misery at home, he took himself to the local emergency department where he was noted to have dangerously low blood pressure and a CT scan that showed a very large abdominal abscess, which yielded more than 2 liters of pus through a drain. This near fatal delay in appropriate care was the result of poor communication among the patient's three treating physicians. This is, unfortunately, a common occurrence in our fragmented healthcare system. Although some large multispecialty group practices have electronic medical records (EMRs) that are shared across all clinical offices, most private offices do not share a common EMR platform and do not communicate electronically with all the other clinicians who are treating you. In fact the various specialist usually have no idea who else is treating you unless you tell them. This point brings us back to the first recommendation posted in Chapter 1 from the US Agency for Healthcare Research and Quality (AHRQ): Make sure that all of your doctors know about every medicine you’re taking. This includes prescription and over-the-counter medicines and dietary supplements. Even if the medical office staff does not specifically ask for this information, it is in your best interest to provide this information and ask that it be included in the specialist records. This will reduce the likelihood of an adverse event like the story of Mr. K above. Another way to improve the coordination of your personal care is to take a minute to call your PCPs office staff to inform them of any treatment plans proposed or implemented by other clinicians. In theory the PCP will eventually receive a mailed letter from the specialist with that information, but this is not a guaranteed process, and may not happen for several weeks. This theme is also consistent with another familiar recommendation from AHRQ to “Make sure that someone such as your primary care doctor coordinates your care”. The federal government has recently implemented regulations requiring all medical offices to adopt electronic medical records, subject to financial penalties for noncompliance. Most of these EMR’s do include patient portals, which give patients online access to their primary care site. This is a convenient way to update your PCP’s office (without struggling to get through on the phone) regarding care that you have received elsewhere. Several states, including Massachusetts, have taken the additional step of creating an electronic database to capture all of the narcotic prescriptions authorized for every patient. Clinicians are encouraged to check this database before issuing a new narcotic prescription to a patient, to avoid dangerous duplication of prescriptions, and to avoid Dr. shopping by drug abusers and drug dealers (see also Chapter 3 for more information on narcotic risks). For frail, elderly patients with multiple medical problems, there are now many nurse-based care coordination programs sponsored by physicians who participate in Accountable Care Organizations or “ACOs.” These ACOs are generally set up with financial incentives which reward well coordinated care, keeping patients out of trouble and out of the hospital. The nurses in these programs work closely with patients, families, and clinicians to prevent communication gaps and to insure that patients receive all needed services in timely fashion. Initial results from the Medicare ACO programs demonstrate that well orchestrated care management programs of this type can improve the quality of care as well as its cost. Quality improvement and cost control are truly two sides of the same coin in these promising systems of care. These same goals are also addressed by a national movement to create a formal “Medical Home” for each patient at their PCP office. Many group practices in Massachusetts and other states are already recognized for their achievements with this program, which includes aggressive goals for coordination of patient centered care, quality improvement, and patient access. The National Committee for Quality Assurance sets most standards for this campaign, described in detail on their website linked at the end of this summary. At that website, you can also learn whether your PCP office has achieved recognition as a confirmed “Medical Home.” I could go on for many pages about these efforts by physicians working with Medicare, state authorities, and NCQA to improve the coordination and quality of medical care, but the primary focus of this book is to educate patients regarding those steps they can take as individuals to steer their care in safer directions. So the interested reader is referred to the following additional links to learn more about opportunities to prevent medical mishaps with better care coordination: www.ncqa.org/Consumers/ImprovingPrimaryCarePCMH.aspx www.pcmh.ahrq.gov/page/defining-pcmh www.aafp.org/practice-management/transformation/pcmh.html Chapter 5 Risks with Transitions in Care Section 4 The Transition to Medical Frailty: When Gravity Starts to Win Medical frailty is now recognized as a specific disorder in older adults and those with disabling neurologic conditions, increasing the risk of falls and serious injury. This syndrome is characterized by signs of: physical decline, including decreased grip strength, decreased walking speed, reduced physical activity, low energy, and unintentional weight loss. Aging is the most powerful predictor of this frailty disorder, which ultimately affects approximately 25% of community dwelling adults over age 85. This is the most rapidly growing segment of the US population, so the consequences of the frailty syndrome are already having major impact on our healthcare system and our caregivers. In addition to those patients who developed the frailty syndrome as a result of aging, younger patients with disabling neurologic syndromes such as multiple sclerosis, Parkinson’s disease, and recurrent strokes may also develop disabilities that fit the pattern described above. Patients with dementia may also move into the frail category sooner than adults with intact cognitive functions. The pioneering medical work in this arena was published in 2001 by geriatrician Linda Fried, dean of the Columbia School of Public Health. Her study of more than 5000 older patients revealed that any patient with at least three of the five factors listed above could be expected to have more falls and a higher risk of death regardless of the other medical conditions diagnosed for that patient. Falls become the prelude to disability and death for these patients, so much research has been devoted to those approaches that can reduce the risk of falls in this population. The first step is to find a clinician who has training or interest in the care of geriatric patients. This is not an easy task, since there is a shortage of certified geriatricians in America. However most physicians certified in internal medicine or family medicine do have the skills needed in this regard. Fortunately geriatric researchers have devised a simple test to identify patients with increased risk for falls, called the “Get Up and Go Test.” Family members and caregivers can also use this simple test to determine whether the patient is a candidate for proactive fall prevention strategies. The test is performed as follows: Patients wear their normal shoes and can use a cane as needed. Begin by having the patient sit in a standard armchair, and mark a walking distance exactly 10 feet in front of the chair. Instruct the patient that you are going to measure the time required for him/her to stand up from the chair, walk to the line on the floor, turn around, walk back to the chair, and sit down again. On the word “GO" begin the timing process (smart phones have convenient stopwatch functions which can help here). Any older adult who needs more than 12 seconds to complete this test is at high risk for falling and can benefit from preventive measures. Patients who flunk this home test should also have the test repeated at the doctor’s office for confirmation and for assessment of specific gait/balance problems. After the patient has been confirmed to have frailty with increased risk of falling, the treating physician can order a home PT assessment or a home nursing assessment, focusing on safety measures such as the removal of throw rugs, use of nightlights, bathroom grab bars, removal of household clutter and bedside commodes for night use. Some patients can also benefit from physical therapy or nutrition supplements, depending on their condition. It is also essential to check for correctable visual defects that are very common in this population. Medication lists should also be reviewed for drugs that can increase the risk of falls, such as sleeping pills, tranquilizers, and high doses of diuretics (water pills). Many frail elderly patients would be safer in a supervised living situation, but insist on living at home alone in order to maintain a desired level of independence. If these patients continue to fall despite preventive interventions, it is particularly important that they wear an electronic alert device which enables them to request assistance even if they are unable to rise from the floor. In fact these devices should be standard practice for all patients who qualify as having medical frailty. Hip fractures are morbid events for elderly patients, causing death or nursing home placement in many cases, so these preventive efforts have great value for those individuals who have transitioned into medical frailty (see also Chapter 1 regarding osteoporosis detection and management) More information on these topics is available at: www.cdc.gov/injury/STEADI Medical Orders for Life-Sustaining Treatment (MOLST) For patients who are crossing the threshold to “frail” status, it is also important to document preferences regarding end-of-life medical interventions. Hospitals in Massachusetts and other states regularly inform patients of their right to designate a healthcare proxy, an individual who can make medical decisions for you if you become unable to do so. This is an important document which should be in place for all adults and which requires the certification of a physician for implementation at that time when you are no longer competent to make medical decisions. However, the healthcare proxy form provides no instructions regarding your wishes for end-of-life care, so the Massachusetts Department of Public Health implemented in April of 2012 a separate planning document titled “Medical Orders for LifeSustaining Treatment “ or MOLST, which provides your physician and your family with very specific guidance regarding your wishes. Most other states in the U.S. have already launched similar programs, which in many cases are titled “POLST” or “Physician Orders for Life Sustaining Therapy.” You can check the status of your home state at polst.org. The two-page Mass MOLST form is available online at http://molst-ma.org, along with instructions for patients and their clinicians. The health care proxy form is also posted on the site. Page 1 of the MOLST document provides your responses to questions about CPR, assisted breathing, and hospitalization. It must be signed by you and your clinician (M.D., nurse practitioner, or physician’s assistant), and you have the option of designating an expiration date. Completion of the form does not require witnesses, notarization, or the assistance of a lawyer, and there is no related bracelet to be worn by the patient. Page 2 of the MOLST form is optional, and addresses more specific questions about types of breathing support, dialysis treatment for kidney failure, feeding tubes, and intravenous fluids. These are important questions that can create confusion and angst for your health care proxy and family members if there has been no prior discussion of these topics before you become incapacitated. So it’s wise to begin the discussion as part of your regular check up or physical exam, before you are actually impacted by a serious illness, starting perhaps at age 50 or 55 in the absence of any serious medical problem. State DPH staff recommend that the signed MOLST or POLST document be printed on pink paper to make it easier to find and recognize. Staples and Office Depot stock “Astro Bright Pulsar Pink” paper for this purpose. Your primary care clinician and your health care proxy should each keep a copy, and you should keep one in your home in a readily available location. The form can be updated at any time, with sign off by your medical clinician (MD or NP or PA) The MOLST program replaces the prior “Comfort Care Program”, in Massachusetts which provided less specific guidance and did not include physician orders, so if you have a bracelet or document from that program you can use this info to update and clarify your wishes via the MOLST form. More specific information about the MOLST process in Massachusetts can be found at: http://www.molst-ma.org or for other states: www.POLST.org Chapter 5 Risks with Transitions in Care Section 5 Hospice and Care at The End of Life: “The Ultimate Transition” Consider the tragic story of Mrs. D, who encountered many problems with her care and she approached the end of her life: Mrs. D, an 85-year-old patient with severe emphysema, diabetes, and congestive heart failure had been living with her daughter since she was widowed five years earlier. Progressive shortness of breath caused her to be up many times each night, with hospitalizations three times during a six-month period, causing her doctors to advise that her condition was likely to worsen despite all available medical care. Meanwhile, she was unable to keep up with her medical expenses, accumulating growing credit card debts. Her daughter needed to stop working in order to manage her care, and confessed to a growing sense of exhaustion with her caregiving role. Mrs. D was seeing three different specialists for her care, and encountered frequent problems with lack of coordination between the physicians, including duplicate prescriptions and conflicting treatment plans (for example, with prednisone doses which aggravated her diabetes problem). At the end she was admitted to the intensive care unit with lung failure requiring initiation of full breathing support with a respirator, and could not be weaned off the breathing tube despite three weeks of treatment. She died in the intensive care unit with multiple tubes in place, confused and unable to speak, despite prior statements indicating that she wished to die at home. This story will sound familiar to thousands of caregivers who are struggling to meet the needs of their elderly parents at home. In the scenario described above, there is no mention of hospice as a recommended treatment option, also typical since many physicians and families have the mistaken impression that hospice services are limited to patients with advanced cancer. Mrs. D was probably eligible based on her poor prognosis, with death likely within six months. A brief summary of the hospice program is therefore in order: This is a benefit available to all patients who have Part A Medicare coverage, including those who are members of a Medicare HMO or Medicare Advantage Plan. The benefit provides for all care needed in the home or nursing home, with virtually no out-of-pocket costs except for room and board at a nursing home. The maximum medication co-pay is just five dollars and patients are also eligible for 95% coverage of up to five days of respite care in a hospice facility to give a break to caregivers. This respite benefit can be accessed periodically to prevent caregiver burnout and to optimize the care plan for the patient. Patients who sign on for hospice care must also agree not to pursue "curative treatment", but this should not be a significant barrier since no curative treatments are available for patients like Mrs. D, or for patients who have already failed attempts at curative cancer therapy. Patients must also agree to allow the hospice team to arrange for all needed medical care, but that hospice care can include brief hospitalizations for symptom control. Such hospital coverage could include for example the placement of a chest tube to relieve shortness of breath due to fluid accumulation around the lungs. Hospice does provide full coverage for medical supplies, medical equipment, and professional care, but not for routine replacement of eyeglasses and hearing aids, though these may be covered by any Medigap policy that is in place for the patient. Surveys of those patients and families who have received hospice services reveal very high levels of satisfaction, with glowing comments regarding the personalized and patient-centered care offered by the nurses who perform the home visits. These services can be tremendously helpful for overburdened caregivers. However, hospice services are underutilized, with only a small minority of Medicare patients using these services at the end of life. In addition, most hospice referrals are too late in the course of illness, such that the average length of stay on hospice was only about 3 to 4 weeks in recent years. Studies reveal that about 70% of patients want to die at home, but fewer than 30% actually die in that more comfortable setting. Several misconceptions seem to be barriers for patients and families who might otherwise consider hospice services: First, patients need to recognize that choosing hospice does not translate to giving up hope. These services should be regarded as redefined hope, targeting better control of symptoms, better quality of life, and more time at home with loved ones. Curative treatment for these patients is no longer realistic, and attempts in this direction can lead to poor quality of life and of death as described above for Mrs. D. A second misconception is that hospice services are available just for cancer patients. For example there are many patients with end-stage emphysema (COPD) congestive heart failure (CHF), kidney failure, or dementia who have a life expectancy not exceeding six months. In this regard a couple of test results can be helpful for prognosis, such as a cardiac “ejection fraction” less than 20-25% for patients with CHF, or a lung function test called “FEV1” less than 25% for COPD patients, or a kidney function test called “estimated GFR” less than 15 mL per minute (unless the patient plans kidney dialysis). Patients with severe dementia who are unable to recognize or speak with family members may also qualify. In all cases, the treating physician must certify the estimated lifespan, but the parameters mentioned above can facilitate those estimates for patients with progressive functional decline. Patients and family members may need to initiate these discussions with the physician, since some physicians are apprehensive about raising the question of hospice. Patients and families who choose the hospice option, as supported by their physician, can expect to receive excellent care at the end of life, with all care coordinated by the hospice team, with preservation of dignity for the patient, and with careful attention to the emotional needs of caregivers. Telephone support and urgent visits from the hospice nurses are available 24x7, and the hospice medical directors are experts with respect to the management of pain and shortness of breath. Financial savings are also a major secondary benefit, with greatly decreased out-of-pocket costs as described above. Recent improvements to the hospice benefit have also made it more flexible, allowing coverage for some hospital tune-ups for symptom relief, as well as the respite stays described above. Primary care physicians can continue to be involved with patients after hospice enrollment, to provide medical information and support, but the hospice team is in charge. For patients and families who are having difficulty making the hospice decision, it is possible to get useful guidance also from local visiting nurse services, who are all very familiar with the hospice programs. These nurses are often already involved with these very ill patients and can provide helpful input regarding potential advantages for each patient. More information on Medicare’s hospice benefit is posted on their website at: www.medicare.gov/pubs/pdf/02154.pdf And reviews of hospice advantages are available at: www.hospicenet.org/html/faq.html Chapter 6 Self-Monitoring Opportunities Section 1 Home Blood Pressure Monitoring: Can Help to Prevent Unnecessary Meds and Visits High blood pressure, also known as hypertension, is the most frequent reason for visits to adult primary care offices, affecting more than half of all patients who reach age 65. Studies have shown however that at least 20% of patients diagnosed with high blood pressure have normal readings at home, primarily a result of anxiety at the doctor’s office. This so-called “white coat “ hypertension can have negative consequences for the patient, as seen in the following case summary that is based on an actual patient event: Mrs. D had several visits to her new doctor with elevated blood pressure (BP) readings that did not improve despite salt restriction and weight loss. So, she was prescribed “HCTZ”, a diuretic pill for BP control. She took the medication faithfully for six weeks, but began experiencing faint spells, especially when standing. Return visits to her doctor revealed that her seated blood pressure was now in the normal range so it was recommended that she reduce her medication dose to just every other day. Despite this adjustment the patient had a fainting spell that caused her to fall and fracture her hip, requiring surgery and a long stay and a rehab facility. When the patient restarted her HCTZ, the doctor asked her to check home blood pressures. Upon self-monitoring, the patient found her readings to be below the normal range, with standing pressures that were much too low. She was then able to stop the medication, relying on home blood pressure readings, experiencing no further fainting spells. Her doctor concluded that she had “white coat hypertension”, which needed no further treatment. Take Home Points: This patient likely would have avoided the hip fracture and the BP medication if her BP had been monitored at home from the start. Note that the US Preventive Services Task Force announced in 2015 that no patients should be started on BP medication without first confirming the diagnosis with home readings. Automated digital BP monitors are now available for less than $50 and have acceptable accuracy as compared to medical office equipment. Cuffs that wrap the bicep area are more reliable than those that attach to the finger or wrist. Consumer Reports cites Omron as the most accurate and simple to use brand. A confirmed diagnosis of “white coat hypertension” can generate significant financial savings for the patient, with respect to prescriptions and office visits, in addition to the safety benefit, which is apparent from the case described above. Even patients with bona-fide hypertension can benefit from home monitoring, potentially reducing the number of needed medications. Home monitoring can also provide an opportunity to see whether symptoms such as headache and faint spells are related to BP changes. Home BP readings can also help patients understand that their BP can be high even when they have no symptoms; hence, the reputation of hypertension as the “silent killer”. Home BPs can therefore serve as a reminder of the importance of medication compliance for patients who “feel fine”. Further information on this topic is available online from the American Heart Association at: www.heart.org/HEARTORG/Conditions/HighBloodPressure/SymptomsDiagnosisM oniteringofHighBloodPressure/Home-Blood-PressureMonitering_UCM_301874_Article.jsp Also at: www.mayoclinic.org/diseases-conditions/high-blood-pressure/basics/lifestylehome-remedies/con-20019580 Chapter 6 Self-Monitoring Opportunities Section 2 Home Glucose Monitoring: Best Way to Insure Good Diabetes Control The American diabetes epidemic continues to challenge our healthcare system, with an explosion in the number of diagnosed diabetics, driven by the simultaneous obesity epidemic and our sedentary lifestyles. For those patients diagnosed with this disorder or with “pre-diabetes” it is essential that they be engaged actively in the monitoring and management of their disease, which is the most common cause of amputations, blindness, and end-stage renal disease today. Type I diabetes is characterized by the total absence of insulin production, so all of these patients are insulin-dependent, usually requiring multiple daily insulin doses or an insulin pump to maintain adequate glucose control. Almost all of these patients do also monitor their blood glucose at home on a frequent basis (the “refusers” tend to have a significantly shorter life expectancy). The more common type II diabetics have reduced levels of insulin production, as well as resistance to the effects of insulin in the body, but they can usually be managed initially with oral medications. Over time however nearly all type II diabetics note decreasing effectiveness of oral diabetes medications, due to progressive failure of their pancreas gland. For these patients insulin supplements become essential for achievement of good glucose control, and insulin management requires periodic home glucose monitoring for patient safety, and for guidance of insulin dosing. The 2014 standards for diabetes care from the American Diabetes Association recommend that all patients on multiple daily doses of insulin should monitor their glucose levels at home, at a frequency recommended by their treating clinician (see also first reference at the end). For patients who use oral meds or a single daily dose of insulin, such as bedtime glargine, it may not be necessary to do daily home glucose monitoring (HGM), but there is evidence that periodic glucose monitoring can help to improve overall glucose control and to prevent symptomatic episodes of low glucose blood glucose which can be dangerous. Patients who have been trained to do HGM can also use this tool to evaluate unexplained symptoms such as lethargy or weakness, and they are also better equipped to monitor their status during episodes of respiratory infection or gastroenteritis, when blood glucose levels are often out-of-control. For those patients who are diagnosed with “pre-diabetes”, also known as “glucose intolerance, home glucose testing can help to reveal those dietary habits which are causing elevated glucose levels, especially if the testing is performed about one hour after meals. Patients may be surprised to see that routine foods such as breakfast cereals and PBJ sandwiches are causing high glucose levels after meals (over 150) , despite the observation that these foods are not generally regarded as “sweets”. Pre-diabetes is diagnosed when the fasting blood glucose is between 100 and 124, typically in patients who have no symptoms to suggest the diagnosis. The testing process itself has become much more convenient and comfortable with use of tiny micro-lancets which are spring-loaded, associated with minimal discomfort. The cost of testing strips is covered by Medicare as a Part B benefit and, to some extent, by most commercial insurance policies, including the Obamacare options, subject to variable deductible requirements. Recently, another option has surfaced for home glucose monitoring using continuous readouts as often as every five minutes from a small catheter sensor placed under the skin. This FDA approved system for continuous glucose monitoring does not however eliminate the need for fingerstick measurements, which should still be performed to validate the continuous readings. These continuous monitoring systems are approved and covered for use only for those patients on multiple daily insulin doses who have failed to achieve adequate control with standard home testing. Some insurance companies also have more restrictive coverage policies, which may restrict the use of the device to those patients using insulin pumps for glucose control. Regardless of the method of testing, the take-home message here is that patients who are actively engaged with home monitoring of their diabetes or pre-diabetes are less likely to have poor control, and more likely to avoid the serious complications of this disease, which can be disabling. In one of my medical leadership roles, I was responsible for the oversight of a chronic disease management program for more than 12,000 diabetics. For that program the single most accurate predictor of good control was the patient’s willingness to cooperate with home glucose monitoring. These activated patients were also more likely to complete their important screening tests for kidney disease, eye disease, and foot disease, and more likely also to keep their blood pressure in good control (see also the previous section in this chapter regarding home blood pressure monitoring). In the long run, these activated patients will be rewarded with better health and a better chance of maintaining independence well into their retirement years. More information on this topic is available at: www.mayoclinic.org/diseases-conditions/diabetes/basics/definition/con20033091 and www.icsi.org/guidelines Chapter 6 Self-Monitoring Opportunities Section 3 Home INR Monitoring: Safer and More Convenient Therapy with blood thinners such as Coumadin (generic name warfarin) is essential for the prevention of blood clots and strokes for patients who have a history of blood clots, heart valve problems, and patients with irregular heartbeat known as atrial fibrillation (“AFib”). AFib is a very common indication for blood thinners, with more than 1.2 million US patients diagnosed with this condition, which affects almost 10% of individuals over age 80. Coumadin continues to be the most commonly prescribed blood thinner, because it is much less expensive than newer drugs which do not require frequent blood testing But Coumadin is a dangerous drug, in fact containing the same active ingredient as rat poison, so careful monitoring is mandatory. The INR blood test (international normalized ratio for prothrombin time) must be checked every few days when treatment is started, and should also be checked at least once monthly for patients on long-term therapy with Coumadin. This testing can be difficult to accomplish during bad weather, for homebound patients and for those who travel frequently. There may also be inconvenience for those patients who must wait in line at the doctor’s office for their blood test. Fortunately, there is a home testing option available for patients on Coumadin, using fingerstick test kits similar to the ones used by diabetic patients for glucose testing. Carefully conducted studies have confirmed that Coumadin patients who monitor their INR at home have health care outcomes and INR control similar to patients enrolled in comprehensive Coumadin management clinics. With these test kits in the home, patients can also check their INR immediately if they have minor bleeding or any unexplained pains that might suggest hidden bleeding in the abdomen, chest, or head. This method is also much more convenient for patients who travel often and for patients with limited mobility. Medicare provides part B coverage for INR home testing equipment provided that the following conditions are met: 1) Patient has been using coumadin for at least three months 2) Diagnosis is Afib, heart valve disease, or history of blood clots 3) Patient has been instructed regarding use of the test kit 4) Patient performs home testing not more often than weekly, and cooperates with physician’s treatment program. Most commercial insurance plans, including the Obamacare options, do provide coverage using similar criteria, but patients should check their coverage situation before proceeding with purchase of an INR test kit, which requires a prescription from a physician. Patients who monitor their blood thinner with home INR tests should still make Coumadin adjustments only as directed by their medical clinician, because these adjustments must maintain the INR value within very narrow limits. Patients should also continue to report promptly to their clinician any new medications prescribed by other clinicians, and should also avoid aspirin, Advil, Motrin, ibuprofen, Aleve, and all other “NSAID” type medications, which can cause serious bleeding problems even after just a few doses (see also chapter 3). More information on this topic can be found at: www.cms.gov/medicare-coverage-database/details/ncddetails.aspx?NCDId=269&ver=2 www.ncbi.nlm.nih.gov/pubmed/11343485 Chapter 6 Self-Monitoring Opportunities Section 4 Home Peak Flow Monitoring: An Asthma Early Warning Yardstick The prevalence of asthma is increasing in America for reasons that are not clear; the disease now affects one in 12 persons, with serious impact on quality of life. Persons with low household income are more likely to have asthma, possibly because of the increased prevalence of smoking, dust mites, and mold related allergies in these homes. So, the declining inflation adjusted income for most Americans may account in part for the gradually increasing frequency of this disease over the last 20 years. Several studies have also confirmed that children who grow up on a farm with livestock are much less likely to develop hayfever and asthma, but few American children grow up on farms these days. The true cause for the increase in asthma rates is likely complex, with many genetic and environmental factors. Asthma symptoms can be disabling, with frequent illness-related absences at work and at school, and with much suffering related to the suffocating sensation of a typical asthma attack. Fortunately, there are now several categories of effective controller medications that can reduce the frequency of asthma attacks and related disability. These include inhaled corticosteroids like Flovent, Leukotriene inhibitors like Singulair, and long acting inhaled beta agonists like Serevent. Patients do need careful education regarding the use of these medications for preventive purposes, since they may be accustomed to noting immediate relief from their rescue inhaler. Excessive use of a rescue inhaler or a long acting beta agonist such as Serevent (using it like a rescue inhaler) can cause serious cardiac rhythm problems and death. Consider the following common scenario: Ann is a 30-year-old patient who has been treated in urgent care centers for wheezing on four occasions over the past year, each time using a rescue inhaler. She has problems with hayfever in spring and fall, and the current season has been particularly bad, causing her to use the rescue inhaler five times one evening. After the fifth dose she noted severe weakness and palpitations, requiring ambulance transport to the local ED, where she was found to have dangerously high blood pressure and heart rate. Hospital admission was required, and Ann’s out-of-pocket expenses for that adventure were over $1,000. This case highlights several common pitfalls for patients with asthma, including reliance on non-primary care providers, excessive use of rescue inhalers, and lack of an action plan for bad days. We will address all of those issues in the following summary. Patients who experience frequent flare-ups of their asthma often have difficulty deciding when to seek medical care. There is overuse of emergency departments, for some patients, and underuse of appropriate medical office visits. One of the best tools to guide patients in this regard is the “Asthma Action Plan”, which is a threecolor patient handout available from the American Lung Association and distributed by many primary care offices for children and adults. On this chart, the green, yellow, and red zones correspond respectively to “Doing Well”, “Getting Worse”, and “Medical Alert”. The yellow and red zones include specific advice for the patient, as written by the treating physician, which may include medication adjustment. All red zone patients need to seek medical care immediately, either through their PCP or at the nearest emergency department. Home peak flow measurement, using a small handheld “blow meter”, provides an objective and reliable way for patients to decide whether their day qualifies as yellow or red. These plastic meters are inexpensive and are covered by virtually all insurance companies. It is important for the patient to document his or her best peak flow score on a day when no asthma symptoms are present. This is best accomplished at the time of a medical office visit, where the patient’s techniques and effort can be confirmed as appropriate. After that step, the patient can identify yellow days when the peak flow drops to 50 to 80% of personal best, and red days when peak flows remain below 50% within 15 to 20 minutes after use of a rescue inhaler. The physician can modify these percentages and translate them to simple peak flow numbers for each patient, dependent on the severity of disease for that individual. Studies have confirmed that consistent use of the peak flow meter and the Asthma Action Plan can reduce the need for emergency department visits and hospital admissions. These proactive planning efforts can also decrease absenteeism at school and at work. Patients who gain experience with this approach can also use these tools to monitor their progress through the days of an asthma flare-up, providing telephone or online reports to the physician’s office, resulting in the reduced need for frequent office visits. It should be noted that peak flow meters are generally not useful for patients with emphysema or COPD, because those patients tend to have less day-to-day variation in their breathing capacity, unlike the reversible wheezing and bronchospasm that are typical of asthma. Your primary care provider should be able to tell you whether peak flow tracking and the asthma action plan are appropriate for your individual wheezing problem. Take Home Points: 1) Work with your PCP to create an asthma action plan 2) Get a prescription for a peak flow meter and learn how to use it 3) Have your PCP or pharmacist review your inhaler technique 4) Use your asthma meds as prescribed, not more often or less often than recommended Copies of the Asthma Action Plan are available at: www.lung.org/lung-disease/asthma/taking-control-of-asthma/create-an-asthmamanagement-plan.html More information on pediatric treatment is also available at: www.pediatricasthma.org Chapter 7 Self-Triage Tips Section 1 Headache: Which Ones Need Prompt Evaluation Neurologists list more than 200 potential types of headaches, so it is not realistic to expect laypersons to successfully label their personal headaches with the correct diagnosis. However, it is appropriate and essential for patients to have an understanding of those headache symptoms that require immediate attention, either as emergency department visits or as prompt medical office evaluations. Consider for example the following unfortunate scenario: Mr. J is a 32-year-old homeowner who fell off his ladder while cleaning gutters, hitting his head on the patio behind his house. He felt fine for about two hours, then developed a severe frontal headache associated with nausea. He ignored his wife’s urges to go to the emergency department, but then had a seizure one hour later. Hospital evaluation revealed a skull fracture and epidural hematoma, which left him with leg weakness and a speech defect for the long-term. The story represents a common example of the category of headache that requires immediate medical attention. Other urgent categories are summarized in the following list: Severe headache after head trauma, with or without nausea and vomiting, as described above Sudden onset of “The Worst Headache Ever”, particularly in patients not subject to regular headaches, a possible clue to ruptured brain aneurism Headache associated with new neurologic symptoms, such as speech problems, loss of vision, or numbness/weakness on one side of the body, possible clues to bleeding in or around the brain Headache associated with fever or stiff neck, with or without nausea, possible clues to meningitis It should be noted that classic migraine headaches often have preliminary aura symptoms such as shimmering visual defects, speech problems, or numbness, but the symptoms usually fit a stereotyped pattern for individual patients. So, these classic migraine patients may need urgent evaluation only for the first episode, to exclude more serious conditions. Migraine patients often have nausea as well, so that symptom is not worrisome as it represents a typical pattern for that patient. New headaches in patients over age 50 require prompt care (within a day or two), but do not have the same level of urgency as the four categories listed above. The diagnosis of concern in these older patients is “cranial arteritis”, also known as “temporal arteritis.” This disease is caused by inflammation of arterial blood vessels, including the blood supply to the scalp and the eyes. So, these patients may also have brief episodes of visual loss, scalp sensitivity, and pain with chewing (see also chapter 8). The diagnosis can be suspected on the basis of blood testing (ESR and CRP tests), and confirmed by scalp biopsy. Prompt treatment with prednisone tablets is essential to prevent blindness. Brain tumors can also cause headaches, but it may be difficult to distinguish these patients from the frequent medical office visitors with tension headaches and migraines headaches. So all patients with undiagnosed headaches should be evaluated by a healthcare professional for clues that may suggest the need for high fidelity imaging of the brain using CT or MRI scanning. Certainly any patient who has new headaches plus neurologic symptoms should seek care promptly, as mentioned earlier. The same is true for patients under treatment for cancer who develop new headaches. Temporomandibular joint syndrome (TMJ syndrome) is characterized by right or left sided headache pain, which may also be associated with ear pain. These pains are caused by strain and degenerative changes in the joint where the jawbone attaches to the skull, and are often associated with teeth clenching and spasm in the masseter and temporalis muscles on the side of the head. Dental malocclusion (underbite, overbite, missing teeth, etc.) can be a trigger for TMJ, so dental evaluation is essential for patients identified with TMJ pain. Headache patients can suspect this under-diagnosed disorder if they press on the joint just in front of their ear and find tenderness or crunching while opening and closing their mouth. Many of these patients are also teeth grinders, as identified at routine dental visits or by sleep partners who share the same bedroom. Although this summary is intended primarily to help patients seek headache care with appropriate timing and with the right provider, I do want to make one final comment about the importance of narcotic avoidance for headache patients. Both the American Headache Society and the American Academy of Neurology recommend that opioid pain pills and barbiturate drugs (like Fiorinol) be generally avoided for relief of headache pain, because these drugs are likely to worsen the problem. Use of these drugs often leads to “rebound” headaches and the problems associated with drug dependency, increasing the patient’s stress levels and headache frequency. More information on these topics is available at: www.americanheadachesociety.org and www.mayoclinic.org/symptoms/headache/basics/definition/sym-20050800 Chapter 7 Self-Triage Tips Section 2 Stroke: How to Benefit from Clot-Busting Drugs Strokes are defined as brain injuries due to blockage or rupture of a blood vessel. The Centers for Disease Control reports that 87% of strokes are caused by blockages, so-called ischemic strokes, and only 13% are caused by bleeding. Bleeds, which are less treatable, can be caused for example by a ruptured aneurysm, which is an abnormal bulge in the wall of an artery near the brain. Strokes are both common and disabling, with about 800,000 cases in the US each year, often with long-term disability as related to speech, vision, balance and the ability to walk and eat. For those patients who present quickly to the hospital after the start of symptoms it is possible for doctors to use an intravenous drug called “TPA” to relieve the blockage and restore blood flow to the brain. In some cases doctors may also be able to use small balloons and stents, much like the treatment of a heart attack. But these clot-busting treatments must be accomplished within three hours of symptom onset to yield the best results, and after 4 ½ hours there is very little benefit. So it is essential that patients seek care promptly for stroke symptoms. Unfortunately most patients do not seek care soon enough to benefit from these high-tech stroke treatments. In fact recent studies reveal that fewer than 10% of patients who could benefit from clot-busting drugs actually receive this treatment, primarily because of delays in seeking care. This is tragic, since major strokes can leave the patients profoundly disabled and totally dependent on family or nursing home, an outcome all patients would like to avoid. Part of the delay problem relates to the fact that emergency CT scans must be performed before clot-busting drugs can be given, to be certain that there is no bleeding in the brain. This step can cause delays depending on the caseload and radiology resources that are available in the selected emergency department. But, the longest delays are typically caused by patients who stay home too long with their symptoms before seeking care. The initial symptoms of a stroke may be mild or intermittent. In fact, many strokes proceed in stuttering fashion, with symptoms that wax and wane for hours before the blood clot causes complete blockage of a significant artery. If the symptoms resolve in less than 24 hours, the episode is referred to as a “TIA” or transient ischemic attack. But, the patient has no way to predict whether the symptoms will get better or worse, so any symptoms suggestive of a stroke require immediate medical attention. According to the National Stroke Association any of the following symptoms should prompt the patient to dial 911 (see also first reference link at the end): Sudden numbness or weakness in the face, arm, or leg, especially if noted on just one side of the body; Sudden confusion, trouble speaking, or trouble understanding words; Sudden trouble seeing, affecting both eyes (unless the patient has classic migraines); Sudden trouble walking or loss of balance and coordination; Sudden, severe, unexplained headache, without a pattern of gradual worsening. The National Stroke Association has also created a simple tool for laypersons to use for the early dedication of stroke in a friend or family member, using the acronym “FAST” as follows: F= Face: Ask the patient to smile and look for drooping of one side of the face A=Arms: Ask the patient to raise both arms and look for downward drift of one S= Speech: Ask the person to name simple objects. Is the speech clear and accurate? T=Time: If you observe any of these signs arrange an immediate medical evaluation, using 911 if needed. There are also two common types of false alarm symptoms that are worth mentioning, to help patients make appropriate decisions about 911 calls. The first type of false alarm is seen in anxious patients who hyperventilate as a response to sudden stress, or because of an underlying panic disorder. These patients become faint-headed with tingling and numbness all over, or limited to the hands feet and face. There’s usually also a sense of hunger for air, sometimes with palpitations. These symptoms improve with paper or plastic bag breathing (DO NOT place the bag over your head!), and are usually seen in patients under age 40. All of these patients should be evaluated for panic disorder and other treatable conditions, but 911 calls are not necessary. The second type of false alarm is commonly seen in older patients who note the onset of dizziness that lasts less than a minute or so after changes in position or with rapid head turning. This can be described as a spinning sensation or vertigo, sometimes associated with brief nausea. However none of the warning signs described above from the Stroke Association are present. The diagnosis in these cases of brief dizziness is usually found to be “benign positional vertigo”, an inner ear disorder that does not lead to strokes. These symptoms do require medical assessment, but not a 911 call. Hopefully this information can help patients and family members to distinguish serious stroke symptoms from benign conditions, enabling more timely and appropriate access to effective stroke treatment. Of course the best approach is to prevent the stroke completely, with effective management of risk factors such as high blood pressure and diabetes. Those topics are addressed elsewhere in this book. More information on this topic is also available at: www.stroke.org www.strokeassociation.org www.cdc.gov/stroke/facts.htm Chapter 7 Self-Triage Tips Section 3 Chest Pain: When to Call 911 The following true-to-life vignette sets the stage for the subsequent discussion: Mr. W, A 53-year-old bank executive, had just finished dinner at home when he began to experience a vague heaviness and pressure sensation in his chest, which he ascribed to indigestion. He tried two Tums without relief and then took a Prilosec tablet, thinking this was likely acid indigestion. Fifteen minutes later, he suddenly collapsed. Arriving emergency medical technicians tried CPR and a defibrillator, but Mr. W could not be revived. An autopsy revealed acute coronary thrombosis (heart attack) related to undiagnosed coronary artery disease. Pain is one of the most frequent reasons for a physician visit, but patients rarely receive education regarding the distinction between minor pains and serious pains. This initial assessment is particularly important during evening and weekend hours when access to care is more limited. Patients shouldn’t try to behave as substitute physicians, but they do need to make prioritizing decisions about when and how to seek care. In this era of increasing co-pays and deductibles, there are also many patients debating whether to seek care at all for their symptoms, fearing the related costs. Chest pain should be first in line for assessment of symptoms, since heart attacks are the most frequent cause of sudden death in this country. However most heart attack victims describe their symptoms as a crushing or squeezing sensation in the mid chest, not a sharp or knife -like pain. In fact many patients have trouble describing the heart attack discomfort, and may offer a clenched fist to the observer to try to communicate the sensation. This clenched fist is recognized by physicians as the “Levine sign”, named after the physician who initially reported this unique form of sign language. Many heart attack patients confuse their symptoms with indigestion, delaying emergency care while they try Tums or Maalox to treat their “indigestion”. These delays can be fatal, as in the vignette above, so it is essential for all adults to understand the characteristic symptoms of a heart attack, recognized also as “myocardial infarction” or “MI” by healthcare professionals. For men in particular denial and fear of embarrassment can be serious obstacles to the timely use of 911 services, so it is difficult to overestimate the value of sharing tragic stories like the demise of Mr. W described above. Heart attack patients may also note that the discomfort radiates to the neck, jaw, or arms, associated with sweating, shortness of breath, and/or nausea. In a few cases the discomfort can also be limited to the upper abdomen, just below the rib cage, especially if only the right coronary artery is involved. For these patients the associated breathing difficulty and/or sweating may be the only clues to a more serious problem. The key take-home message is that any of these symptoms warrant immediate medical attention, usually with a 911 call to request ambulance assistance. If aspirin is immediately available it is also appropriate to chew one tablet while waiting for the ambulance, since this helps to prevent further clotting of the coronary arteries. Interventional cardiologists can open the blocked artery with special catheters and stents, but these procedures are not effective if the patient delays seeking care for many hours after the onset of chest symptoms. On the other hand there are several types of chest pain that virtually never represent a heart attack. For example some patients experience brief stabs of chest pain lasting not more than a few seconds, often aggravated by changes in position or by pressing on the rib cage. These pains are usually caused by chestwall problems, often involving the rib joints. Likewise patients may complain of chest pains that occur only with a deep breath or with coughing. Such patients may have chest infection or pleurisy, which can be assessed during routine office hours with a physician but which do not require a 911 call or emergency care. Finally there are also patients with obvious sour heartburn who experienced chest pain during an episode of heartburn, due to spasm of the esophagus or “swallowing tube”. If this chest pain responds promptly to antacids like TUMS or Rolaids, it is unlikely to be heart related. Blood clots traveling from the legs to the lungs are recognized as “pulmonary emboli” which can cause relatively mild chest pain, often aggravated by a deep breath. But the more common presentation of pulmonary emboli is with the sudden onset of shortness of breath, sometimes with an initial fainting spell or coughing of blood. Most pulmonary emboli afflict patients with risk factors, such as prior blood clots, cancer treatment, recent major surgery or fracture, prolonged immobilization, or recent long-distance travel. So, patients with any of these risk factors should be prompt to seek medical care for any of the symptoms described above which could suggest pulmonary emboli. One other type of chest pain that requires immediate attention is the pain associated with aortic dissection, i.e. a sudden tear in the wall of the main artery (Aorta) that carries blood from the heart to the rest of the body. This is a relatively rare event but is life-threatening. The pain is usually described as a severe tearing sensation in the mid chest, radiating straight through to the back. These aortic tears may also cause blockage of the arteries to the brain, so there may be simultaneous stroke symptoms such as weakness or numbness on one side of the body or difficulty with speech. Of course, any of the symptoms warrant immediate medical attention with a 911 call. More information on these topics is available at the following links: www.mayoclinic.org/diseases-conditions/chest-pain/basics/definition/con20030540 www.heart.org/HEARTORG/Conditions/HeartAttack/WarningSignsofaHeartAttack /Warning-Signs-of-a-Heart-Attack_UCM_002039_Article.jsp www.ncbi.nlm.nih.gov/pmc/articles/PMC3099272 Chapter 7 Self-Triage Tips Section 4 Abdominal Pain: Which cases Need Prompt Surgical Evaluation Abdominal pain has dozens of common potential causes, some of which do not require prompt medical attention; however, most patients are not well-informed regarding those symptoms that do need prioritized medical evaluation. There is a tendency for patients and family members to try to self-diagnose the problem based on the location or intensity of the pain. However the location and intensity can vary tremendously even for a single diagnoses such as appendicitis. Many appendicitis patients present without the typical story of mid-abdominal pain which later shifts to the right lower abdomen, for example. So it is not useful for patients to attempt self-diagnosis, but there are some general guidelines which patients and family members can use to decide when and where to seek appropriate care for abdominal pain. The Mayo Clinic patient education tool, referenced as the link at the end of the summary, provides helpful guidance in this regard. The following situations should prompt the patient to seek emergency assistance via the 911 line or other immediate transport to emergency care: Severe abdominal pain following trauma due to accident or injury Severe abdominal pain associated with pressure or pain in the chest, especially in males over age 35 and females over age 45 Abdominal pain associated with vomiting of blood or large amounts of bleeding from the rectum. Prompt medical assessment should also be accomplished for the following abdominal pain scenarios, at an urgent care facility or physician office, but urgent ambulance transport is usually not needed for these cases: Pain severe enough to prevent the patient from sitting still, or causing a patient to curl into the fetal position Pain associated with persistent nausea and vomiting, or with moderate amounts of rectal bleeding, without fainting spells Pain that is worse with movement or coughing, or is associated with yellow skin, swelling of the abdomen, or tenderness when the patient touches his or her abdomen. Cases that do not fit the six categories listed above but do include abdominal pain that has persisted for more than 24 hours should be reported to the patient’s physician for assessment and advice, especially if the abdominal pain is associated with fever or loss of appetite. Essentially all primary care offices do offer telephone coverage on weekends, holidays, and evenings so it is appropriate to call for advice in these cases before proceeding directly to an emergency department. Gastroenteritis due to viral infection or food poisoning is perhaps the most frequent cause of abdominal pain, and can be managed by telephone contact without an office visit in many cases. The pain in these cases is cramping in quality, with severe squeezes of pain lasting just a few minutes at a time, usually associated with diarrhea and/or brief vomiting spells. This is different from the steady ache that is typical of appendicitis or diverticulitis or other serious abdominal conditions. For patients with suspected gastroenteritis, however, it is important to report any signs of serious dehydration, such as dry mouth or faintness on standing, in which case medical care may be needed for intravenous fluids. In general, all patients with abdominal pain should avoid using narcotic pain pills prior to medical assessment, since these drugs can worsen some stomach conditions and can also mask the presence of a serious of abdominal problem, making diagnosis difficult. Over-the-counter “NSAID” drugs like Advil and Aleve should also be avoided since they can cause stomach ulcers and bleeding in sick patients. More information on this topic can be found at: www.mayoclinic.org/symptoms/abdominal-pain/basics/definition/sym-20050728 Chapter 7 Self-Triage Tips Section 5 Back Pain: The Danger Signs which Should Trigger Early Action Back pain is the second most frequent symptom seen in medical offices in the United States, and the great majority of these patients note resolution of symptoms within 6-8 weeks without the need for surgical procedures or x-ray studies. However a small percentage of these patients will prove to have more serious underlying disease, as noted in the following case summary: Mr. B, a 60-year-old insurance broker, presented to his primary physician with a four-week history of mid back pain, between his shoulder blades, with no strain or trauma recalled. The pain seemed worst at rest in bed, and was associated with a 10-pound weight loss over six months. His physician recommended Advil, a heating pad, and physical therapy, but the pain was not better at a six week follow-up visit, so the PCP requested standard x-rays which were normal. Four weeks later, the patient developed numbness and weakness in both legs, prompting an MRI scan which revealed a cancerous tumor of the spine. This patient had several high risk factors suggesting the need for MRI evaluation earlier in his fourteen-week course of illness. Those risk factors include some of the following, which are described in detail in the references linked at the end: 1) Predominant symptoms at rest or in bed 2) Unexplained weight loss, at least 10 pounds in 6 months 3) Unexplained fever 4) History of recent cancer treatment 5) History of osteoporosis or long-term prednisone therapy 6) Recent significant trauma 7) No improvement after 6 or more weeks of treatment 8) New lower extremity weakness or numbness 9) Loss of bowel or bladder control 10) History of intravenous drug abuse Patients with any of those 10 risk factors are more likely to have a serious underlying cause of their back pain, and are justified in advocating for MRI assessment before completion of a 6-8 week treatment course (unless their only risk factor is #7 above). Based on my personal practice experience, I also have a higher level of concern for those older patients who described persistent pain in the upper back, instead of the usual lower back location. Many of these patients prove to have compression fractures of the spine or other significant disorders. Medical experts agree that patients with none of these risk factors don’t benefit from plain x-rays, CT scans, or MRI scans unless they fail to improve after 6-8 weeks of treatment. This is true even for those patients who have findings suggestive of sciatica, with leg pain due to disc rupture, because most of these patients will improve without surgery. It’s also important to note that bulging discs in the lower back, without actual herniation of disc fragments, are very common and are not reliably associated with back pain. In fact studies have determined that more than 50% of adults with no history of back pain do have bulging lumbar discs on MRI, causing no pain or other symptoms (see third reference link at the end). This is another reason premature MRI scanning is unwise, since many false positive bulging discs will be found, causing confusion and frustration for patients who often expect some form of surgical treatment for these minor abnormalities. The great majority of patients with lower back disc disease can achieve satisfactory pain relief with a combination of medications, physical therapy or chiropractic care, and the faithful performance of lumbar flexion exercises at home. Surgical intervention is best reserved for the small percentage of patients who don’t respond to these conservative treatment efforts. For those patients who are found to have osteoporosis (low bone density) and spinal compression fractures as the cause of their pain it’s important to initiate treatment with osteoporosis medications, with periodic bone density tests to assess treatment progress. With the aging of the US population this is emerging as an increasingly frequent cause of back pain in older adults and for patients who have used prednisone long-term or on a periodic basis. More information on these topics is available at: www.aafp.org/afp/1999/1115/p2299.html www.ncbi.nlm.nih.gov/pmc/articles/PMC1495170 www.ncbi.nlm.nih.gov/pubmed/8208267 Chapter 7 Self-Triage Tips Section 6 Obstructive Sleep Apnea: Under Diagnosed and Under Treated The American obesity epidemic, which now includes one third of all adults, has also increased the number of patients affected by obstructive sleep apnea (OSA), a lifethreatening disease that significantly decreases quality of life. OSA is defined as the frequent interruption of breathing during sleep, due to obstruction by the soft tissues of the neck, in association with frequent snoring. Since effective sleep is reduced, the patient experiences daytime sleepiness and fatigue, and they also may complain of shortness of breath and chest pain during awakenings at night. They often also note morning headaches and problems with memory, concentration, and depressed mood. Research reveals that OSA can cause high blood pressure, heart failure, and suddendeath, and OSA patients are also at risk for serious complications after surgery and with the use of pain medications, sedatives, and sleeping pills. This serious disease affects at least 3 to 7% of males under age 65 and 2 to 4% of females, and is more frequent in those over age 65 and in those with obesity or a thick neck (17 inches or more in men). Unfortunately many OSA patients are undiagnosed, and are unaware of the serious risk to their health. Children are also at risk for OSA, especially if they have large tonsils and adenoids, with chronic nasal congestion and snoring. Poor quality sleep often causes these children to have difficulty with behavior problems and school performance, which can improve with treatment of OSA. The diagnosis of obstructive sleep apnea (OSA) must be confirmed with an overnight sleep study, with electrodes pasted to the skin for EEG tracking of sleep , oxygen content of the blood, breathing efforts, and pulse. This is often done at a hospital or freestanding sleep lab, but can also be performed at home, unless the patient has serious medical problems such as heart failure or COPD (emphysema). If the diagnosis is confirmed, treatment can be accomplished using breathing support in bed with a CPAP device, which defeats the airway obstruction using pressurized air through a small facemask. Some patients can also achieve relief using a customized oral splint /mouthpiece which maintains an open airway. There are also several surgical procedures that can be curative for patients who don’t achieve improvement with CPAP or splits. As mentioned earlier children with OSA can also benefit from tonsillectomy with adenoidectomy. Weight-loss can also reduce symptoms for obese patients. Take Home Points: 1) OSA is a serious disease that can cause sudden death and heart failure, as well as poor quality of life. Obese patients are at increased risk for this disorder. 2) Patients with any of the symptoms described here should discuss sleep lab testing with their physician or health care provider. 3) Patients already diagnosed with OSA must adhere to recommended treatment to avoid sudden death, heart complications, and motor vehicle accidents. Those who found prior CPAP devices to be unworkable may have better success with newer devices that are more compact, or with “BIPAP” therapy as an alternate approach. 4) Patients with diagnosed OSA should also be very reluctant to use narcotic medications such as opioid pain-killers and tranquilizers, since these meds can worsen the blood oxygen levels in OSA patients increasing the risk of heart complications. Likewise patients with OSA should inform their surgeon before proceeding with any surgical procedures, since special precautions must be taken right after surgery to prevent injury due to low oxygen levels in the blood. More information on this topic is available at: www.mayoclinic.org/diseases-conditions/obstructive-sleepapnea/basics/definition/con-20027941 and at: www.sleepfoundation.org Chapter 8 Unnecessary Procedures Section 1 Stress Tests for Low Risk Patients: Why These Can Be Misleading Consider the following Case History: Mr. J, a 60-year-old obese smoker, scheduled a stress test with his personal physician for reassurance, since one of his friends had recently died of a heart attack. His cholesterol and blood pressure levels were normal. Mr. J passed the stress test without symptoms, and with no EKG signs of heart trouble. However, just two weeks later he returned home from work and collapsed in the living rom without warning. The EMTs and his family were unable to resuscitate him, and an autopsy revealed myocardial infarction (heart attack) with a coronary artery blood clot. How can this happen, and what can patients and physicians do to try to prevent this type of tragic outcome? Heart attacks are defined by the American Heart Association as damage to the heart muscle caused by inadequate oxygen delivery through one or more of the coronary arteries on the surface of the heart. In most cases, there is sudden blockage of one of the three coronary arteries by a blood clot. These clots tend to form at the site of a soft fatty plaque on the inner wall of the artery. These plaques are very common in older adults and are subject to rupture or fissuring without warning, triggering the formation of a clot at that site. If the patient arrives at a hospital quickly, it is possible to attack the clot with drugs or a stent; but in some cases, the heart responds to the clot by triggering a fatal electrical disturbance which stops all blood flow, as in the story of Mr. J above. In fact about 25% of all sudden cardiac deaths have no warning symptoms. For the purpose of this discussion, it is important to note that the coronary artery which becomes blocked by a clot may have been narrowed less than 50% before the plaque rupture, with no restriction to the flow of blood and with no symptoms, even at the time of a stress test. Other patients may have extensive “hardening of the arteries”, causing more extensive narrowing of the coronary arteries beyond 50%. These patients often experience chest pressure with activity or with stress, so-called “angina. “ Their tight coronary blockages prevent them from walking as long as expected on a treadmill stress test, and their EKG and cardiac imaging tests often reveal abnormal findings. So, angina sufferers have warning signs and symptoms that can alert their physicians to the increased risk of a heart attack. But patients who have only soft coronary plaques, with no blockages above 50%, do not have angina pains and usually have normal results on a stress test, even if that test includes thallium images of the heart. These unsuspecting patients are at risk for a heart attack, but no form of stress test would reveal that risk, and no surgery or stenting would be appropriate for these superficial coronary plaques. Fortunately, medical research has identified specific risk factors which can help to identify those patients who are likely to form coronary plaques leading to heart attacks. Those risk factors have been well publicized, and include the following, which are treatable: High Blood Pressure Smoking Diabetes High LDL Cholesterol Low HDL Cholesterol Obesity Sedentary Lifestyle Earlier this year the American Heart Association and the American College of Cardiology (ACC) released a new guideline recommending that all adults consult with their physicians to calculate their 10-year risk for a heart attack, using the risk factors listed above. Those patients with a 10-year risk of at least 7.5% are advised to begin treatment with a “statin” medication, regardless of their cholesterol level. Likewise, patients with diabetes or an LDL cholesterol above 190 are also advised to use statin medications, at the same time that they address all of their other risk factors. The first reference cited below includes access to the new coronary risk calculator, along with the updated prevention guideline. So, Mr. J’s unfortunate story might have concluded with a better ending if the new guideline had been available and implemented at that time. His risk factors translate to a calculated heart attack risk of greater than 7.5%, and he could have benefitted from statin medication regardless of his cholesterol level. There is no guarantee that his heart attack would have been prevented, but the risk would have been significantly reduced. Readers should remember however that stress tests do have substantial value for appropriately selected patients. There are many situations where a cardiac stress test can provide valuable guidance to patients and physicians, particularly for patients with angina symptoms or very high risk for coronary disease (10 year risk exceeding 20%, per the American College of Cardiology). This section just emphasizes the limitations of stress testing in the large group of patients with early coronary disease and non-obstructive coronary plaques. More information on this topic is available at: www.heart.org/HEARTORG/Conditions/HeartAttack/HeartAttackToolsResources/ Heart-Attack-Risk-Assessment_UCM_303944_Article.jsp and www.nhlbi.nih.gov/health/health-topics/by-category/ Chapter 8 Unnecessary Procedures Section 2 Avoidable Procedures: How I Almost Lost My Mother-In-Law In Chapter 2, it was noted that excessive use of antibiotics can have negative consequences for patients. From a broader perspective there are many medical situations where more care and more testing does not translate to better care. The American College of Physicians and the American Board of Internal Medicine have recently compiled a list of tests and procedures which are best avoided, publicized as the “Choosing Wisely” campaign. But physicians often find that patients press for excessive testing, misled with the perception that more tests lead predictably to better health. Consider the following actual case that almost cost the life of the patient: Mrs. B had been experiencing upper abdominal pain after meals and her ultrasound test revealed gallstones is the cause. One of her liver tests (the GGTP) was also mildly abnormal so her husband who was an ICU nurse pressed the G.I. physician to proceed with more testing to exclude a common duct stone. The physician therefore performed an endoscopy test called an ERCP as a prelude to surgery. That ERCP test revealed no abnormality, but within an hour after the procedure Mrs. B begin noting severe abdominal pain and very low blood pressure. Her daughter alerted the nursing staff and Mrs. B was rushed to the operating room for repair of a ruptured loop of bowel, a known complication of ERCP, which extended her recovery by three months and caused several postoperative infections. Take-home points: 1) The ERCP test was not necessary in this case and caused a serious complication, which was preventable. The mildly elevated GGTP test by itself did not suggest blockage of the bile duct with a stone, and is not recognized by experts as an indication for ERCP testing. 2) Physicians often perform tests of marginal value because of patient demands, to reduce their perceived risk of a malpractice lawsuit. Neither patient nor physician are well-served by such misguided testing. 3) Consumer Reports has worked with the Choosing Wisely campaign to create patient friendly summaries of more than 50 medical tests and procedures that are of low value. That information is available free of charge at www.choosingwisely.org/doctor-patient-lists/ 4) Screening tests for healthy patients represent a special challenge for shared decision-making and are addressed in Chapter 9. This is another area where over-testing can have negative results for patients. The following guidelines have also been issued by the American Gastroenterology Association as part of the “Choosing Wisely” campaign, to reduce the frequency of unnecessary GI procedures similar to the one performed on Mrs B: Don’t repeat screening colonoscopy sooner than 10 years after a negative highquality study with normal findings Don’t repeat screening colonoscopy sooner than five years for patients found to have one or two small polyps less than 10 mm, without dysplasia Don’t repeat endoscopy sooner than three years for patients with Barrett’s esophagus who have had a second endoscopy which revealed no dysplasia For patients with functional abdominal pain syndrome (spastic colon) don’t repeat CT scans if the initial CT was normal, unless the symptom pattern changes More information on this topic applicable to other specialties is summarized in the next section. Chapter 8 Unnecessary Procedures Section 3 The Choosing Wisely Campaign: Physicians Designate Low-Value Care The American College of Physicians recently collaborated with all of the medical specialty societies to develop lists of those procedures that have little or no value for patient care. The creation of these lists represents a quality accomplishment as well as a cost control accomplishment, since unnecessary procedures cause complications and side effects as well as unnecessary costs. The following summary includes highlights in laymen terms from the full report, which can be accessed using the link which is referenced at the end. I have added a few explanatory comments in parentheses and have translated some words into laymen terms: Cardiology Don’t do cardiac stress test for low risk patients without symptoms (low risk translates to a 10 year risk for coronary events less than 20%) Don’t do routine annual follow-up stress tests for patients with coronary artery disease (less frequent stress tests are sufficient, as dictated by patient symptoms) Don’t do pre-op stress tests for low risk surgery Chest Physicians Don’t do CT angiograms to rule out pulmonary emboli for low risk patients with normal blood D dimer test results Don’t renew annual home oxygen orders without first checking the patient’s oxygen levels Emergency Medicine Don’t do head CT’s for patients with minor head trauma Avoid antibiotics and wound cultures for patients with uncomplicated skin abscess Avoid head CT for patients with uncomplicated syncope (fainting spell) Use ultrasound rather than CT scan to diagnose most patients with kidney stones under age 50 Avoid back x-rays in patients with no trauma and no risk factors for underlying spinal disease ENT (Ear, Nose, and Throat) Physicians Don’t prescribe oral antibiotics for routine swimmers ear Don’t order sinus x-rays for patients for meet diagnostic criteria for acute sinus infection General Internal Medicine Don’t ask for daily home glucose monitoring for type two diabetics who are not using insulin Don’t recommend cancer screening for patients with a life expectancy less than 10 years (there are risks and complications related to cancer screening tests which can offset the potential benefits, especially for patients near the end of life) Hematology Don’t give blood transfusions for hemoglobin levels less than 7 to 8 unless patient’s symptoms persist Don’t use inferior vena cava filters routinely in patients with blood clots in legs or lungs (this intervention has not been proven to improve patient outcomes) Don’t test for clotting disorders in patients with acute blood clots who have transient risk factors like surgery or trauma Don’t administer plasma or plasma extracts to reverse excessive anticoagulation unless the patient has bleeding or needs emergency surgery Nephrology Don’t prescribe bone marrow stimulators like Epogen for chronic renal failure patients who have hemoglobin levels greater than 10 (these drugs have potentially serious side effects and are very expensive, and higher levels of hemoglobin have not been shown to improve health) Don’t do routine cancer screening for dialysis patients with a life expectancy less than 10 years (see also comments in General Medicine Section above) Avoid “NSAID” drugs (like Advil, Motrin, and Aleve) in patients with hypertension or heart failure or chronic kidney disease Don’t start dialysis treatments without a shared decision-making discussion with patients and their families Neurology Don’t do carotid ultrasound to evaluate patients with fainting spells Avoid opioids and barbiturates for migraines No carotid surgery for patients who have asymptomatic narrowing of the carotid artery unless the surgeon’s complication rate is less than 3% Ophthalmology No pre-op tests needed before cataract surgery, unless for a specific medical condition like diabetes Don’t insert artificial plugs in the corner of the eye for dry eyes unless other methods have failed (artificial tears usually work well) Pediatrics Don’t prescribe antibiotics for suspected viral upper respiratory infections Don’t do head CT’s for minor head injury Don’t do head CT or MRI for simple febrile seizures Don’t do abdominal CT’s for routine evaluation of belly pain More info is also available at: www.choosingwisely.com Chapter 8 Unnecessary Procedures Section 4 CRP Syndrome: Ill-Advised Surgery Can Make Matters Worse Complex Regional Pain syndrome (CRP) is a poorly understood neurologic disorder (formerly known as “reflex sympathetic dystrophy”) that causes severe disabling pain following injury or surgery involving a leg or arm. Initially the pain does not correspond to any abnormal findings on physical exam, lab tests, or x-rays, so physicians are often unable to offer a diagnosis. Patients are likely to visit many physicians seeking diagnosis and relief and may be subjected to inappropriate procedures that can drastically worsen the picture. The following vignette is an actual patient history, shared with permission of the long-suffering patient: Mr. K., an athletic 50-year-old mechanical engineer, sought medical care for an ingrown great toenail, with surgical correction performed by a podiatrist. Following this minor procedure, the nail was no longer ingrown and there was no redness or any findings to suggest infection, but the pain actually worsened, making walking difficult. The podiatrist was puzzled, and referred the patient to an orthopedic surgeon, who examined the toe and foot, finding no abnormality to explain the increasing pain. However, he recommended exploratory surgery “to see what’s going on in there”. The patient reluctantly agreed to proceed, but after the second surgery, which revealed no abnormal findings, the pain became much worse with the gradual development of redness and swelling of the entire foot and lower leg. Over the next several years, the pain, redness, and episodic swelling progressed to involve both legs and eventually the hands, forcing the patient to stop working and abandon his competitive cycling passion. Specialty consultants diagnosed CRP (formerly called reflex sympathetic dystrophy) and attempted local anesthetic injections and “pepper cream”-both of which worsened the symptoms significantly. Take Home Points: Don't agree to surgery when there is no clear indication especially with unexplained pain after injury or an initial surgical procedure on an extremity. In the early stages of CRP there are no objective findings to make the diagnosis obvious, so patients with disabling extremity pain after surgery or trauma should be unwilling to accept dismissive physician interactions and are best served by consultation with a neurologist familiar with treating CRP. In later stages of the disease, the redness, temperature changes, and swelling make the diagnosis more clear, but cures shouldn't be expected even with expert consultants. The prognosis is improved if the disease is diagnosed early in its course, since careful physical therapy with progressive desensitization can prevent worsening of pain and disability. There is growing consensus that this disease develops through involvement of the central nervous system (brain and spinal cord), which explains how the disorder can spread to the other side of the body. So new therapies are focused accordingly – such as spinal cord stimulators, intra-thecal spinal infusions, and intravenous ketamine infusion. Many CRP patients pursue high-dose opioid medications for pain control, with all of the hazards referenced in Chapter 3, and the new approaches referenced above can help to reduce opioid dependence and related complications. Opioid doses can also be reduced with use of the same group of medications that are used to treat nerve related pain, such as gabapentin, amitriptyline, and duloxetine. In those advanced cases where the patient is no longer using the extremity for normal activities there can be loss of bone density and bone strength, so specific medications for that osteoporosis problem may also be required. More information on this topic is available at: www.mayoclinic.org/diseases-conditions/complex-regional-painsyndrome/basics/treatment/con-20022844 and www.ninds.nih.gov/disorders/reflex_sympathetic_dystrophy/detail_reflex_sympath etic_dystrophy.htm Chapter 9 Visual Symptom Alerts Section 1 Cranial Arteritis: Blindness Can Result From Delays in Diagnosis Mary B. is a 62-year-old teacher who presents to her primary care physician (PCP) with a 10-day history of pain in the right jaw area and right forehead, which is aggravated by chewing. She has not experienced headaches previously and recalls no recent teeth clenching or trauma. Her PCP finds tenderness in the area of her right TMJ (where the jawbone connects to the skull just in front of the ear) and offers the diagnosis of TMJ Syndrome with a recommendation for Ibuprofen tablets and a heating pad. Unfortunately, Mary experiences total loss of vision in her right eye four days later, prompting an urgent visit to her eye doctor, who makes the diagnosis of cranial arteritis with optic nerve damage due to loss of blood flow. In retrospect, Mary recalls brief clouding of vision in that eye 10 days prior, but she had not shared that information with her PCP since it seemed unrelated. The eye doctor begins treatment with Prednisone tablets after obtaining blood tests, preserving vision in the left eye, but the right eye remains permanently blind. Take Home Points: Cranial Arteritis (also known as “temporal arteritis” or “giant cell arteritis”) is an inflammatory disorder of blood vessels of unknown cause, which is seen primarily in patients over 50, and which can cause blindness as well as strokes. Involvement of the jaw muscles can also cause pain with chewing, as was seen in this case, which can be confused with TMJ syndrome. Patients in this age group should take care to report any visual symptoms in association with headache, but for some Cranial Arteritis patients, the first sign of visual involvement is blindness, so it is essential that screening blood test be performed promptly for all older patients who present with new, unexplained headaches. The blood tests of interest are the erythrocyte sedimentation rate (ESR) and Creactive protein (CRP). If either of these tests is abnormal, the diagnosis can be confirmed with biopsy of the temporal artery. Prednisone therapy controls symptoms rapidly and can prevent blindness if treatment is begun promptly. This therapy should begin as soon as the diagnosis is suspected, before biopsy results are available. Given the critical importance of rapid treatment, it is essential for older patients to report new headache or visual symptoms promptly to their PCP. In severe cases, there may also be fever, weight loss, and scalp tenderness – all potential clues to this serious disease. For those older patients who do begin prednisone therapy for this disease, it is important to anticipate the predictable side effects, because prednisone is usually needed for many months. So each of these patients should have a bone density test and consideration of medication to prevent the rapid development of osteoporosis, which can become disabling with compression fractures of the spine. (see also chapter 1) Older patients on prednisone should also be checked periodically by their physician for diabetes, which can be triggered by prednisone. Patients who are unable to tolerate prednisone may be candidates for other powerful anti-inflammatory drugs under the direction of rheumatology specialist, but a trial of prednisone is always the first step. Given the critical importance of rapid treatment, it is essential for older patients to report new headache or visual symptoms promptly to their PCP. In severe cases, there may also be fever, weight loss, and scalp tenderness – all potential clues to this serious disease. More information on this topic is available at: http://www.mayoclinic.org/diseases-conditions/giant-cellarteritis/basics/definition/con-20023109 http://www.nlm.nih.gov/medlineplus/ency/article/000448.htm Chapter 9 Unnecessary Procedures Section 2 Visual Floaters and Flashes: Which Ones Need Prompt Attention Most floaters and spots that appear in the field of vision are harmless and need no medical attention. The majority of floaters are caused by age-related changes in the semi liquid center of the eye. This gel, which has a gel-like consistency in younger patients, is referred to as the vitreous of the eye. As we age, however, that vitreous gel becomes more watery, and pieces of gel may break loose and float freely within the eye. These pieces of gel are commonly called visual floaters and are easiest to see against a bright background like a clear sky or a computer screen. However a sudden burst of floaters can indicate that the vitreous gel has pulled away from the retina at the rear of the eyeball, a so-called posterior vitreous detachment or “PVD.” In about 40% of cases, these floaters are also associated with flashes of light, which reflect traction on the retina itself, causing electrical impulses to be sent from the retina to the brain. Studies have confirmed that patients with the sudden appearance of both floaters and flashes have about one chance in seven (15%) of having a retinal tear or retinal detachment, which can cause a permanent visual defect. The retina itself is a thin membrane covering the backside of the inner eye, with millions of light receptors (rods and cones). It has a delicate blood supply that becomes disrupted with any tear or detachment. Patients who note the sudden appearance of floaters and flashes should seek care promptly with an eye care professional, in order to look for evidence of retinal tear or detachment. If an early retinal tear or retinal detachment is detected the injury can be repaired using a laser or an open surgical procedure, depending on the extent of injury. If care is delayed, the outcome maybe suboptimal, as noted for the following case: Mrs. T, a 62-year-old nearsighted school teacher noted a burst of flashes and floaters in her right eye shortly after a minor fall on ice in her driveway. She did not strike her head and figured that the visual symptoms were just related to the stress of the fall. She also noted that the floaters and flashes decreased over the next 24 hours and so concluded that there was no need for medical intervention. Unfortunately, she did not seek medical care until two weeks later because of a hazy shadow that persisted in her right eye. Upon examination, the ophthalmologist found evidence in her right eye of a retinal detachment and attempted surgical repair; however, the visual defect persisted and could not be improved. This case summary underscores the observation that when the retina is torn or detached, it loses its blood supply at the back of the eye and can become irreversibly damaged if the condition is not addressed promptly, generally within a few days. So, patients who notice a burst of floaters and flashes should seek immediate evaluation by an eye care professional, regardless of whether the symptoms improve over the first day or two, as demonstrated by the case of Mrs. T above. Patients with diabetes, prior retinal injury, or direct trauma to the eye or head are at increased risk and should seek prompt eye care if they note any flurry of floaters or flashes, or a sudden decease in vision. The ultimate impact on vision also hinges on whether the retinal injury is near the macula, which is the site on the central retina with the largest concentration of visual nerve receptors. In general retinal tears if limited in size can be repaired with a laser beam but larger tears and detachments often require surgical placement of a gas bubble to press the retina back in position. In contrast to the implications of floaters and flashes, some patients with migraine headaches experience a visual disturbance just before the headache, with gradual growth of a blind spot with shimmering margins. This visual disturbance can be confirmed to be unrelated to any intrinsic eye disease because it is always binocular, ie, present in both eyes simultaneously. Patients can confirm that this type of aura is not eye related by covering first one eye and then the other to confirm that both sides are affected. The cause of this binocular visual disturbance is a slowly spreading electrical disturbance on the surface of the back of the brain, which is a feature of the migraine disease. For binocular visual defects (both eyes) that persist more than 1 hour, migraine is an unlikely explanation; patients with these symptoms should seek prompt medical evaluation with their primary care physician or a neurologist. More information on these topics is available at: www.mayoclinic.org/diseases-conditions/retinaldetachment/basics/definition/con-20022595 www.nlm.nih.gov/medlineplus/retinaldetachment.html Chapter 9 Visual Symptom Alerts Section 3 Glaucoma: Not All Halos Are Good Glaucoma is an eye disease caused by increased pressure within the eyeball, due to poor drainage of fluid from the eye. The disease presents in two varieties: “open angle” and “closed angle,” corresponding to the gap at the edge of the colored iris where fluid drains through the “trabecular network.” The first link at the end of this summary includes a couple of diagrams and videos that clearly demonstrate the anatomy of these two types of glaucoma. In United States “open angle” glaucoma is the most common type, and most patients with this disorder have no symptoms in early stages. For this reason the American Academy of Ophthalmology recommends that all adults over age 40 have periodic eye exams for checking of the ocular pressure and for examination of the optic disc at the back of the eye. That disc, where the optic nerve enters the eye, can show cupping or deformity if glaucoma is causing excessive pressure in the eye. Some patients with open angle glaucoma have normal eye pressure, so it is essential that the optic disc be examined carefully, since this may be only sign of glaucoma for this group of patients. Simple measurement of the ocular pressure for example at a primary care office is not sufficient in this regard. Later in the course of glaucoma there may be dark spots seen in the field of vision, but at that point the glaucoma is far advanced, so it’s unwise to wait for visual symptoms before seeking routine eye exams. Some patients are at increased risk for glaucoma, including Afro-Americans, diabetics, patients with prior eye trauma, and those with a positive family history of glaucoma. Individuals with prior cataract surgery using a hard acrylic lens implant are also at risk for pigment dispersion glaucoma. For these high-risk patients routine screening becomes particularly important. The second type of glaucoma called “closed angle” glaucoma is more common in Asians and those with farsightedness. These patients are at risk for attacks with very high pressure in the eyeball, causing headaches, eye pain, redness of the eye, nausea, and blurred vision. Some of these patients also note warning symptoms that resemble halos or rainbows around bright lights. Any of these symptoms should prompt immediate examination by an eye care professional to prevent permanent loss of vision. During episodes of acute angle closure glaucoma headache and nausea may predominate, so it’s important for patients to pay close attention to the visual clues to ensure that they seek care with an optometrist or ophthalmologist, not just an urgent care center. For both forms of glaucoma there are effective treatments that can prevent loss of vision. Initial treatment is with prescription eyedrops. Cases that cannot be controlled with this approach may be treated with a laser to create better flow of fluid through the trabecular meshwork, or through a small laser hole in the iris for patients who have angle closure glaucoma. In other cases open surgical repair maybe needed. Regardless of the treatment method chosen results are best if the disease is identified in early stages, so all patients should pursue a baseline routine eye exam at age 40 or so, with guidance for future exams based on that assessment. Patients over age 65 should have routine eye exams every 1-2 years, depending on their chronic medical conditions. All adults should also seek prompt professional care with an optometrist or ophthalmologist for any of the warning symptoms discussed above. More information on this topic is available at: www.geteyesmart.org and www.mayoclinic.org/diseases-conditions/glaucoma/basics/definition/con20024042 Chapter 9 Visual Symptom Alerts Section 4 The Red Eye: Can be Misdiagnosed as Conjunctivitis Primary care physicians are often asked to evaluate patients with a reddened and uncomfortable eye. Although conjunctivitis is the single most common cause of this problem there are many other potential causes which are more serious and which can be misdiagnosed and mismanaged. This section will discuss criteria that can be used by laypersons to confirm that an initial PCP medical evaluation is adequate. Consider the following story: “A 15-year-old boy is brought to his pediatricians office for evaluation of a 2 day history of redness and discomfort in the left eye. There is no history of trauma or respiratory symptoms. The physician examines the eye but does not use an ophthalmoscope or penlight and doesn’t use fluorescein stain or anesthetic drops to complete the exam. Visual acuity is measured by the nurse and recorded as mildly reduced in the left eye. A diagnosis of conjunctivitis is offered and the patient is treated with antibiotics drops. Three days later the boy is brought to the ED because of severe eye pain and decreased vision and an ophthalmologist confirms the diagnosis of herpes virus keratitis with damage to the cornea, with long-term scarring and decreased vision.” Primary care physicians are not expected to have all the tools and expertise of an eye specialist for these cases but there are several key steps that should be expected in any evaluation of a red eye in the primary care setting, as follows: 1) Unassisted inspection with the naked eye is not adequate. The clinician should use either an ophthalmoscope (which is usually attached to the wall in exam rooms) or penlight to illuminate the eye. 2) Staining of the eye surface should be performed with fluorescein dye using drops or a strip of special paper. This helps to detect any abrasions or infections that have injured the cornea. 3) Anesthetic drops make the exam more comfortable and inform the physician regarding whether the eye problem is superficial. 4) Inspection beneath the eyelids is important to exclude any foreign body or ulcerations. This is made easier by the anesthetic drops mentioned above. These steps were not followed in the case described above leading to a serious misdiagnosis with long-term consequences for the patient. A drop of fluorescein staining would have revealed the typical branching pattern of herpes ulcers on the cornea, and earlier treatment would likely have prevented the severe scarring which followed. So, when a physician or nurse practitioner does not follow these steps in evaluating your red eye, you would be wise to complain and to seek care elsewhere if the office is not equipped to perform these diagnostic steps. A too- casual diagnosis of conjunctivitis can also miss other more serious eye diseases in adults. These conditions could include acute iritis, angle closure glaucoma, and deep infection of the eye. In these conditions anesthetic eyedrops won’t eliminate the pain which is an important clue to a more serious diagnosis. There are several other key symptoms that can also alert patients to the possibility of a more serious cause for their red eye, as follows: Sensitivity to light, which is also known as photophobia. The symptoms caused by spasm of the ciliary muscle inside the eye and can be a clue to inflammation and iritis. Halos around lights in association with headache can be a clue the most serious form of glaucoma, which can lead to blindness within 24 hours. A major decrease in vision limited to one eye is also a red flag symptom. This can reflect many serious eye disorders including bacterial infection Severe eye pain and decreased vision in a contact lens user is also a high-risk symptom. This may indicate a corneal ulcer which can lead to permanent visual loss. Eye pain and/or decreased vision after direct trauma to the eye or chemical exposure. Any of these five symptoms coupled with the appearance of a red eye should trigger prompt care with an eye care specialist. This would include an ophthalmologist or optometrist but not an optician. These specialists will use a special magnification and lighting system called a “slit lamp” to carefully evaluate the problem. If the symptoms are first noticed on a weekend or holiday it’s appropriate to seek care in the local emergency department where eye care specialists are on call for emergency cases. Adult patients who have a regular source of eyecare are encouraged to make their first phone call to that office when a red eye is noted. Eye specialists have all the equipment that is needed for complete evaluation of these cases. If that access is not available in timely fashion, a PCP or urgent care appointment may be necessary with all the caveats and precautions summarized above. More information on this topic is available at: www.medicine.virginia.edu/clinical/departments/ophthalmology/education/RedE ye Chapter 10 Cancer Screening Section 1 Breast Lumps: Mammograms Can Be Misleading When a woman or her physician discovers a breast lump it is common practice to begin the evaluation process with a mammogram, often followed by an ultrasound test if the mammogram is inconclusive. It is important to remember, however, that 10 to 15% of breast cancers are invisible on these tests. In some communities, magnetic resonance imaging (MRI) is available to help with these challenging cases, but MRI tests are also not 100% conclusive. In light of this, national guidelines now recommend that any persistent breast lump which cannot be defined as benign by these imaging tests should be removed with a biopsy by a surgeon. The following vignette highlights the risk of not performing a biopsy for all unexplained breast lumps: Mrs. G discovered a right breast lump at age 37 and was examined by her physician who ordered a mammogram, which was normal. The radiologist recommended an ultrasound test that was also normal. Mrs. G returned to her PCP one month later and was advised that the breast lump was probably just fibrocystic disease, which could be rechecked at the time of her next regular check up in four months. At the four-month visit it was apparent that the lump had enlarged, and biopsy confirmed breast cancer, which had already spread to her lymph nodes. The patient unfortunately died 18 months later and her family sued her physician for the delay in diagnosis. Take-home points: Some breast cancers cannot be detected by mammograms or ultrasound tests, so persistent lumps require surgical biopsy for diagnosis (or needle drainage for cysts) Breast-cancer in premenopausal women tends to be more aggressive, so early diagnosis is particularly important in this age group, a difficult challenge since benign fibrocystic changes are also more common in younger women Women with a family history of breast cancer or ovarian cancer are at increased risk and should discuss options with their MD for more careful monitoring. Monthly breast self exam is recommended for all women since new lumps can be found outside of the routine screening intervals. More information on this topic is available at: www.breastcancer.org/symptoms/testing/types/biopsy Chapter 10 Cancer Screening Section 2 Cervical Cancer and HPV: Major Missed Vaccine Opportunities Consider the following tragic case summary: Miss W was a 35-year-old impoverished single mother of two children who never returned for follow-up care after her pregnancies and never had a Pap smear performed. She presented years later to the local emergency department with heavy vaginal bleeding and was found have a large mass on her cervix and uterus, which proved to be cervical cancer. Unfortunately, the cancer had spread to her lungs and liver so she died seven months later despite radiation and chemotherapy, leaving two orphaned children. For the first time in medical history we have a vaccine that effectively prevents cancer, specifically cancer of the female cervix. But the compliance with this HPV vaccine program remains weak, a major missed opportunity. For calendar year 2013 the National Committee for Quality Assurance reports that fewer than 15% of 13-year-old girls have completed the recommended three vaccine series for human papilloma virus (HPV), which is known to cause more than 90% of all cervical cancer cases. Since catch-up vaccine doses can be administered up to age 26 there have been other studies to assess compliance rates in older women, revealing that fewer than 50% of all eligible women complete the vaccine series. Since 2011, boys have also been eligible to receive HPV vaccine, but their compliance rates are even lower. This is unfortunate since males are also at risk for HPV related cancer of the oral and genital areas, with over 7000 cases reported annually for men in the US. For both males and females the HPV virus also causes more than 90% of genital warts, an unpleasant precursor to the more serious cancer cases. It’s also known that more than 80% of US women have evidence of HPV infection by age 50, so this is the most common sexually transmitted disease. Scientific studies have confirmed that the HPV vaccine is 93 to 98% effective in preventing early cancerous growths of the cervix, with no serious side effects or complications. So why are most eligible patients not receiving this cancer preventing vaccine? Surveys of adolescents and their parents reveal ignorance to be the primary barrier. Most are not aware of the effectiveness of the vaccine, and others feel that they need not comply until they become sexually active. In fact, the vaccine works best when administered before sexual activity begins, because 50% of Americans can expect to become infected with HPV shortly after sexual activity begins. Another factor that impacts vaccine compliance is the reluctance of schools to require this vaccine, unlike the picture for polio, measles, mumps, rubella, and hepatitis. So far, only the states of Texas, Virginia, and DC require HPV vaccine for school entrance. Pediatricians have been unable to achieve high rates of HPV vaccination in part because adolescents do not come to the doctor’s office as frequently as younger children, generating more restricted opportunities for vaccination. Reimbursement for the vaccine has been a problem for some physician offices and many also report that parents are not well informed about the benefits and risks of HPV prevention. Some parents express the concern that children receiving HPV vaccine will regard this protection as a license to become sexually active, but there is no evidence for increased pregnancy rates or sexually transmitted disease among vaccinated patients to justify these concerns. Every year in the US approximately 11,000 women are diagnosed with cervical cancer, and 4000 die of the disease. Full compliance with HPV vaccine programs could therefore prevent about 3000 deaths annually, as well as several million cases of unwanted genital warts. Since the HPV vaccine doesn’t cover 100% of viral strains it’s also important for women over age 35 to be tested for HPV at the time of their pap smears. We know however that reliance on Pap and HPV testing is not sufficient, because most cases of cervical cancer are seen in women who’ve never had a Pap smear, similar to the woman in the case summary that opened this section. Primary prevention of HPV infection with the vaccine is therefore the best way to prevent thousands of cervical cancer deaths every year in this country. It would be tragic if the first cancer vaccine continues to be underused because of the barriers referenced above, most of which can be resolved with better public education on this important topic. More information on this topic is available at: www.cecity.com/aoa/healthwatch/june_12/print4.pdf and www.ncbi.nlm.nih.gov/pubmed/22119587 and www.cdc.gov/std/hpv/default.htm Chapter 10 Cancer Screening Section 3 Lung Cancer: Effective Screening Is Finally Available, But…. We finally have an effective screening test to reduce deaths due to lung cancer in heavy smokers using annual low-dose CT scans. This recommendation was officially released in December 2013 by the US Preventive Service Task Force, which noted that patients should be engaged in a shared decision-making process with their medical clinician before any screening is attempted. Eligible patients are those with at least 30 pack years of smoking (for example, one pack per day for 30 years, or two packs a day for 15 years), and age 55-80, excluding those who quit smoking more than 15 years prior. These guidelines emerge from the national lung cancer screening trial, which studied more than 50,000 smokers over seven years. “Pros” and “Cons” are summarized by the task force as follows: Pros: There was a 20% reduction in the death rate due to lung cancer and a 7% reduction of death due to all causes over the seven-year study period. Given the rates of cancer noted in the study, this translates to the prevention of one lung cancer death for every 300 smokers screened with CT scans. So, for any individual smoker who begins annual CT scans, the chance of benefit is about one third of 1% (1/300) over seven years. Cons: False alarms were very common. In fact, 40% of the patients screened in the study were found have at least one “abnormal” spot or nodule in their lungs; but 96% of these nodules proved to be benign, with no signs of cancer. In many cases, these benign nodules required a biopsy for diagnosis, using a needle through the chest wall, or a tube down the windpipe, or open surgery under anesthesia. These biopsy procedures do involve risks and some discomfort for the patient as well as variable expense and anxiety. The follow-up CT scans also generate a copay obligation of $100 to $150 for the patient for most insurance companies. So, each qualifying smoker must carefully consider the balance of benefit and risks. The likelihood of false alarms is relatively high as compared to other types of cancer screening and the detection of lung cancer by screening CT does not guarantee a good prognosis; but one lung cancer death can be prevented for every 300 smokers screened, as described above. Smokers need to remember, however, that the most reliable way to reduce the risk of death and disability is to stop smoking, since this habit also causes emphysema, heart disease, stroke, and many types of non-lung cancer (see also Chapter 11). In fact, the risk of lung cancer death is decreased by more than 50% over the next 15 years for those smokers are able to quit (see second reference below). Household smoking also increases the risk of breathing problems and lung cancer for family members. In light of these significant health risks to oneself and to one’s family, smokers should not view CT screening as a protective justification for continued smoking. More information on screening for lung cancer can be reviewed at: www.uspreventiveservicestaskforce./uspstf/.htm or www.cancer.org/cancer/lungcancer-non-smallcell/moreinformation Chapter 10 Cancer Screening Section 4 Colon Polyps: One-Size-Does-Not-Fit-All for Follow-up The widespread use of screening colonoscopy (inspection of the large intestine through a flexible scope) has helped to reduce the annual colon cancer death rate by more than 30% in the US, but some patients are reluctant to undergo screening, or have follow-up testing when polyps have been detected. Polyps can be defined as pre-cancerous growths on the inner wall of the large intestine or colon. Most polyps do not cause bleeding, so stool blood testing is not a reliable method to screen for polyps. The greatest risk for future colon cancer is associated with polyps which are larger than 1 cm (about 3/8 of an inch), multiple polyps, and those with signs of dysplasia (ie disordered growth) on microscopic exam of the polyp. Patients with these high-risk polyps should have repeat colonoscopy every 3 to 5 years, depending on their level of risk. Follow up failures can have serious consequences for the patient, as described in the following true case summary: Mr. K had a screening colonoscopy performed at age 52, revealing three polyps between 1.5 and 2 cm in size, which were removed, with no signs of cancer. He was advised to return in three years for repeat colonoscopy, since he qualified as high risk for future polyps and cancer. However, he did not respond to his physician’s reminder three years later, in part because his insurance company indicated that his co-pay would be $150, and also because he was reluctant to repeat the messy colonoscopy prep. He did well until four years later when he noted rectal bleeding and abdominal pain, diagnosed as widespread and incurable colon cancer. He died 11 months later, after an illness that generated more than $5000 in out-of-pocket costs, placing the colonoscopy co-pay in perspective. Take-Home Points: 1) Polyps can transform to cancer, and require follow-up testing even after polyp removal, since polyp forming patients tend to grow new polyps in the future. 2) The high-risk polyp features described above require particular careful follow up, especially there if there's also a family history of colon cancer. 3) Colonoscopy is the preferred test for follow-up of colon polyps. X-ray studies such as barium enema and virtual colonoscopy, with no scope inserted in the Colon, are less accurate, and always require colonoscopy as a next step if any abnormality is seen on the x-rays. 4) Patients who experience discomfort during initial colonoscopy can request adjustment of their colonoscopy “cocktail” for the next exam. The medications now available for this purpose can mercifully erase all memory of the event for the great majority of patients. More information of this topic can be found at: www.mayoclinic.org/diseases-conditions/colon-polyps/basics/definition/con200319578 and at: www.patients.gi.org/topics/colon-polyps Chapter 10 Cancer Screening Section 5 PSA Testing: Not for All Men…Shared Decision-Making is Key Until recently, PSA blood tests were routinely performed annually for men over 50 to detect prostate cancer in early stages. It was assumed that this screening test would extend lifespans by reducing death rates due to prostate cancer. Several published studies now demonstrate, however, that overall lifespan is not extended by the screening test. There is a modest reduction in deaths due to prostate cancer, preventing one prostate cancer death for every 300 man screened during 14 years of follow-up in the most favorable study. In other words, more than 99% of men who undergo PSA screening do not benefit from that testing. A few men also die of complications related to diagnosis and treatment of early prostate cancer. Given these outcomes, proponents of PSA screening cannot claim yet that it extends lifespan. Additionally, there is growing concern that many of the early cancers detected with PSA screening would never have caused symptoms for the patient. In fact, autopsy studies reveal that more than half of all men by age 70 have microscopic evidence of prostate cancer, with no related symptoms. So the diagnosis and treatment of cancer in those men may cause needless anxiety and many preventable complications. Consider the following true story, which helps to reinforce this point: Mr. B at age 66 was found have an abnormal PSA blood test and biopsy of his prostate gland revealed early stage prostate cancer, for which he received radiation therapy. Unfortunately the radiation cause severe bleeding in his urine, due to "radiation cystitis” of the urinary bladder. Attempts to correct this with infusions of bladder medication as well as electro-cauterization were not successful, and the patient required surgery to remove his urine bladder with urine diverted permanently to a bag on his abdomen. He also developed a serious infection following the surgery with two additional operations were required to drain and resolve that infection, causing also a large hernia which may require further surgery. These complications were all related to the radiation therapy for a cancer that may have never caused symptoms for Mr. B. Those men who choose surgery rather than radiation for treatment also risk significant complications, including impotence and loss of urine control. Take-home points: 1) The American College of Physicians and the American Urology Specialty Society now recommend that men be counseled regarding the pros and cons of PSA screening before any testing is performed. Related patient education information is available at http://www.mayoclinic.org/diseasesconditions/prostate-cancer/basics/definition/con-20029597 and http://familydoctor.org/familydoctor/en/diseases-conditions/prostatecancer.html 2) For those men who choose to be tested and who receive the diagnosis of early prostate cancer, it is reasonable to consider follow-up monitoring instead of aggressive cancer treatment. This watchful waiting approach has been shown to be safe, provided that the urology specialist confirms the cancer to be of the nonaggressive type. Further information about this option is available at http://www.cancer.org/cancer/prostatecancer/detailedguide/prostatecancer-treating-watchful-waiting 3) For those men who choose not to have regular PSA blood testing, it is still appropriate to have digital rectal exams at the time of regular check ups. Prostate cancer and prostate enlargement can be detected by means of these rectal exams. Chapter 11 Injuries That Are Often Misdiagnosed Section 1 Navicular Fractures of the Wrist Some wrist fractures prove to be difficult to diagnose, with serious consequence for the patient. The following story is unfortunately typical: “Mrs. P, a 60-year-old teacher, tripped over her dog’s leash and fell on her outstretched hand causing pain in the wrist. She presented to the local urgent care center where wrist X-Rays were reported negative and she was given a Velcro wrist splint to wear for two weeks. Unfortunately, three months later she was still having pain in the wrist, so she was seen by an orthopedic specialty office where x-rays revealed a previously undetected fracture of the navicular bone with non-union. This required surgical repair and an extended period of physical therapy with out of pocket cost exceeding $3,000 for Mrs. P” Most fractures of the wrist involve the radius bone, the long one that extends from the elbow to the wrist joint. These are generally not difficult to diagnose with standard x-rays but a subset of wrist fractures involve a small bone at the base of the thumb named the navicular bone (sometimes also called the scaphoid bone). This bone is about the size of a large grape and has a precarious blood supply that can be interrupted by a hairline fracture. Injuries to this bone are often not visible on standard x-rays for 4 to 6 weeks, causing up to 40% of these fractures to be missed by the clinicians who initially evaluate the patient. This creates a serious problem because navicular fractures that are not properly treated can lead to loss of blood supply to the bone with deterioration of the wrist joint. These cases usually require surgery with hardware placement and often lead to chronic pain and disability. Ideally all physicians in primary care offices, urgent care, and the ED would be fully aware of this possible medical mishap, but unfortunately this is not always true in these very busy settings. So, it’s helpful for patients to understand how to avoid a missed diagnosis of navicular fracture. The most reliable clue to this diagnosis is tenderness directly over the navicular bone, which is located at the base of the thumb in a pocket formed by the two extensor tendons of the thumb. This indentation, sometimes called the “anatomic snuffbox”, can be easily seen with the palm facing down and the thumb fully extended. Any injured patient who has pain and tenderness localized to this “snuffbox” area should have the thumb and wrist immobilized with a thumb ‘spica’ cast or splint, with follow-up referral to an orthopedic specialist. Standard wrist splints do not immobilize the thumb and are of no benefit with these navicular fractures. The orthopedic specialist may choose to repeat the x-ray in several weeks looking for fracture confirmation, but the most efficient and most accurate way to confirm the diagnosis is with a CT or MRI scan of the wrist. These more advanced imaging techniques can reveal a fracture long before any changes are visible on standard Xrays. So these tests should be utilized in the assessment of wrist injuries with persistent pain beyond the usual 2 to 3 weeks expected with a simple sprain. As with many of the scenarios described in this book a well-informed patient is much less likely to be victimized by a delay in diagnosis of these subtle navicular fractures, thereby avoiding considerable disability and the pain and expense of corrective surgery. More information on this topic is available at : www.hss.edu/conditions_scaphoid-fractures-non-union-wrist-fractures.asp Chapter 11 Injuries That Are Often Misdiagnosed Section 2 Hip Fractures in Patients Who Can Still Walk Most hip fractures immediately disable the patient with extreme pain and the inability to bear weight, because the injured joint is unstable, with moving bone fragments. But a special subset of hip fractures can fool the patient and the doctor, by preserving the patient’s ability to walk with only mild to moderate pain. This can happen when there is a crack in the neck of the hip bone, but with the two ends of the fractured bone jammed together. In medical terms, this is described as an “impacted sub capital fracture of the femur”, which can masquerade as a simple bruised hip. The following story is not unusual: “Mrs. J, an 80 year old grandmother, fell on her front steps at home and was able to get up and walk but with persistent pain in her right hip. She presented the following day to her primary care physician, who performed an X ray of the hip that was reported as normal. So, Mrs. J was sent home with a diagnosis of bruised hip, with instructions to use Advil and a heating pad. Two days later, she collapsed in her bathroom with increased hip pain, and evaluation in the ED confirmed a displaced hip fracture. Review of the recent hip Xray revealed that an impacted subcapital fracture was present then, but was not diagnosed” You can demonstrate this mechanism of fracture with a dry piece of kindling wood, or a dead pine branch about an inch thick. Break that piece of wood over your knee and then jam together the jagged broken ants. You will find that the piece of wood regains most of its original strength if you press the ends together without bending at the fracture site. This helps to explain how a person can still walk with one of these impacted hip fractures. Unfortunately, the sub capital hip fractures can also be very difficult to identify on standard x-rays, especially for the emergency department and urgent care physicians who often provide the initial care for these patients. Several published studies have confirmed that the misdiagnosis rate is substantial for patients who seek care for one of these impacted hip fractures. Doctors are often misled by the patient’s ability to walk and may not even order a hip x-ray. In other cases, they perform the subtle changes on x-ray images. It should also be noted that some patients with these a typical hip fractures describe pain in the thigh, not in the hip. This so called “referred pain” can mislead physicians into ordering x-rays of the wrong area, and can contribute to delays in the correct diagnosis. So it is important for the treating clinician to check range of motion at the hip joint after any injury that causes pain in the thigh area. Patients with a prior diagnosis of osteoporosis are at particularly high risk for hip fractures of all varieties. A missed diagnosis sets the stage for poor healing and more serious trouble for the patient, whose hip may become unstable at any time, causing another fall with more serious injuries. The patient with an undiagnosed hip fracture is also at risk for blood clots at home, especially if he/she becomes immobile because of the hip pain. So, senior patients with persistent hip pain after a fall should press for CT scanning of the hip when standard x-rays are described as normal. Radiologists may also recommend CT or MRI imaging when they see a hip x-ray with finding suspicious for an impacted fracture, because they recognize that standard techniques are at times inadequate for the diagnosis of these injuries. When the diagnosis of impacted sub capital fracture is confirmed, the most reliable treatment is surgical repair. In most cases, the hip can be repaired with long metal screws, a simpler procedure than total hip replacement, which is often required for more complex fractures. Recovery times are reasonably short provided that the patient has not been immobile at home for extended time periods because of misdiagnosis. This is another example of a situation where informed patients and their families can advocate for timely and appropriate medical evaluation to prevent a serious medical mishap. More information on this topic is available at: www.ncbi.nlm.nih.gov/pmc/articles/PMC1285822/?page=3 Chapter 11 Injuries That Are Often Misdiagnosed Section 3 Missed Achilles Tendon Rupture Achilles, the Greek hero of the Trojan War, was slain by an unlucky arrow that pierced his heel, rupturing the tendon, which now bears his name. Modern-day heroes of the weekend warrior variety are also at risk for injury to this tendon, and seniors who use prednisone tablets or quinolone antibiotics are also at increased risk for rupture of this critical tendon. Unfortunately up to 25% of patients who rupture this tendon are misdiagnosed, with long delays in appropriate care. This is surprising at first glance, because the Achilles tendon is essential for walking, and is the thickest tendon in the human body. So how is it possible to miss the diagnosis of these ruptures? Several factors seem to contribute to the failure to recognize tears of the Achilles tendon, as follows: 1) Patients can actually walk after complete rupture of the Achilles tendon, because several other tendons in the ankle can enable flexion at the ankle joint. 2) Though there may be pain initially, many patients have minimal pain after the first few days, because tendons have relatively few nerve fibers. 3) Clotted blood can quickly fill the visible gap which was initially created when the tendon ruptured. Given the challenging nature of the diagnosis it’s important for patients to be able to advocate for consideration of Achilles rupture when they have persistent difficulty walking or running after injury to the heel area. Typically the patient notes sudden pain in the back of the ankle with exertion or with stair climbing, as if someone has struck them with a baseball bat. There may also be a popping sound or sensation, followed by discomfort, and there is often bruising of the heel area over the next few days. When there is suspicion of Achilles rupture it’s essential for your healthcare provider to examine the tendon for a palpable gap and to check the simple Thompson test, which requires that the patient kneel with feet dangling over the edge of the exam table or chair. A squeeze of the calf muscle should cause the foot to flex downward. If that flexion is not observed the patient should be evaluated further with MRI scanning of the Achilles tendon and with prompt orthopedic consultation. Partial tears of the Achilles tendon can be treated with at least 6 weeks of splinting, but complete tears usually require surgical repair. The surgical results may not be favorable if there is a long delay between injury and treatment, so a timely diagnosis is essential to achieve the best possible long term outcome. More information on this topic can be found at www.orthoontheweb.com/achilles.asp Chapter 11 Injuries That Are Often Misdiagnosed Section 4 Spinal Fluid Leaks: When a Runny Nose Can Be Life-Threatening Head injuries are common and many patients with apparently minor head trauma, without loss of consciousness, do not seek medical care. A few of these head injuries, perhaps 1 to 2%, involve fracture of the base of the skull, which can be difficult to detect with standard x-rays, and which can cause leakage of clear spinal fluid (CSF) through the nose or ear. This leakage may not appear until days to weeks after the accident, so patients may assume that allergies or viral infection are the cause. This is a dangerous situation, with significant risk for meningitis as long as the leak continues. The following case summary demonstrates the consequences for failure to diagnose and treat this problem: Mr. Z is a 25-year-old helmet-wearing cyclist who fell off his road bike striking the back of his head without loss of consciousness. He did not seek medical care and noted clear drainage from his left nostril starting two weeks later. He treated this with over-the-counter decongestants without success. Three weeks later he developed severe headache and fever, and was diagnosed with meningitis, which required a two-week hospital stay for IV antibiotics. The meningitis left him with the complications of chronic dizziness and hearing loss. Further testing confirmed the presence of a fracture of the base of the skull that was causing leakage of spinal fluid through his sinuses, requiring surgical correction. Take Home Points: 1) Leakage of clear fluid from just one nostril up to several weeks after head injury should be regarded as evidence of spinal fluid leakage until proven otherwise 2) Failure to recognize and treat this problem can place the patient at risk for life threatening meningitis. 3) For suspected CSF leak the diagnosis can be confirmed by measuring for “Beta 2 transferrin “ protein on a sample of nasal drainage, followed by highresolution CT scan. Treatment begins with a conservative trial of bed rest with elevation of the head. If that does not work within 3 to 7 days, surgical repair can be curative, often performed with an endoscope through the nose without scalp incision. More information on this topic is available at: www.ncbi.nlm.nih.gov/pubmed/11571972 and www.columbianeurosurgery.org/conditions/cerebrospinal-fluid-leaks/ Chapter 12 Our Deadliest Habits: New Perspectives Section 1 Tobacco Smoke: The Only Legal Substance With No Safe Dose If you love someone who smokes, please encourage them to read this brief chapter. I debated whether to include in this book a chapter addressing unhealthy habits, because the rest of the book deals with specific medical situations and the approaches that patients can use to minimize risk in those circumstances. However the three habits addressed in this chapter account for more death and disability than all of the prior chapters combined, so it seemed appropriate to consider them as fair game for any effort to prevent medical mishaps. On reflection, I realized also that most patient education efforts on the topic of smoking are focused on cancer and the risk of death. This mortality-based approach may have an impact for a small subset of smokers, but it is my impression (based on discussions with many smoking patients) that lifespan is not a motivating topic. Smokers understand that they forfeit years of life, but rationalize that all of us will die, so we might as well maximize pleasure, not years. So the following comments are directed to smokers from a different angle, reviewing the prospects for disability and loss of functional independence, topics which seem closer to their motivational sweet spot. Smoking accounts for more premature death and disability than all other risk actors, and is ranked by the CDC and the World Health Organization as the number one cause of preventable disability in the world. No smoker wants to become disabled, unable to continue without restriction those activities he or she enjoys. Unfortunately, the odds do not favor the smoker who gambles at this particular game of chance. We all know of the rare smoker who lives to a ripe, old age without any apparent consequences, except for lizard skin and a husky voice. But the great majority of smokers will not be that fortunate. Consider first the tragic disorder we call emphysema or COPD. The typical smoker who starts the habit by age 21 has a lifetime risk of COPD approaching 40% (including chronic bronchitis as well as emphysema). This figure would be higher if one could add the smokers who die prematurely from lung cancer or heart disease, but they don’t live long enough to develop COPD. COPD sneaks up on the smoker, because more than half of normal lung function must be lost before shortness of breath becomes bothersome. After that point, the symptoms worsen steadily, even if the patient stops smoking. Eventually COPD progresses to the point where everyday activities are no longer possible. And the expensive prescription inhalers which are used for treatment don’t eliminate the symptoms, they just knock them down a notch. For patients with advanced COPD, a minor upper respiratory infection can become a life-threatening event, with severe shortness of breath prompting frequent hospital admissions. Prednisone becomes the drug of last resort, and creates its own set of disabilities related to weight gain, osteoporosis, depression, cataracts, and worsening of diabetes. In the final stages of COPD, the blood oxygen levels can decrease to the point where oxygen supplements are needed, causing the patient to be tethered by nose 24x7 to an oxygen bottle or oxygen generator. Without exception, these patients look back on their smoking habit and say, “It wasn’t worth this”. And COPD is only one of the disabilities that smokers can expect. Next in line is coronary heart disease, which is much more common in smokers. Patients often continue to smoke after their first heart attack, a testimony to the addictive power of nicotine. These persistent smokers can anticipate recurrent heart attacks with repeated damage to the heart muscle, resulting eventually in congestive heart failure and progressive shortness of breath, much like the COPD patients described above. Life becomes a struggle for these unfortunate patients, with restricted mobility and with buckets of expensive medications, which can’t restore the independence and vitality that all of us want. The disability story goes further for the smoker as we consider the impact on other blood vessels in the body, starting with the legs. Virtually all patients who become disabled due to poor circulation in their legs are either diabetics or smokers or both. The disease begins with painful walking, a cramping sensation in the calf muscles which is relieved by rest. As the disease progresses it can cause pain even in bed, with non-healing ulcerations of the feet and legs. Surgeons can offer balloon procedures and bypass operations, but the recurrence rate is high for these blockages, with many patients needing multiple procedures, which may ultimately culminate in amputations, especially if the patient continues to smoke. The major arteries to the brain are also subject to premature atherosclerosis and blockages in smokers. Recurrent strokes are therefore very common in smokers, and can lead to disabilities related to speech, vision, arm use, and walking ability. The small recurrent strokes commonly seen in smokers are also responsible for a form of dementia called “multi infarct” dementia, which compounds the physical disabilities and may also cause loss of control for bladder and bowels. Again, no smoker wants these outcomes. The risk picture does not end with the large arteries of smokers, since smoking also causes damage to small arteries throughout the body. This “micro vascular” disease is thought to be the cause for the increased frequency of impotence seen in male smokers, which is often not helped by the Viagra type medications that are pitched constantly on TV. Tobacco related micro vascular disease is also responsible for the higher rate of “macular degeneration” of the eyes in smokers, increasing the risk of legal blindness. Likewise for a significantly increased risk for osteoporosis in smokers, with painful spinal compression fractures and hip fractures. Dental disease is in the same category, with smoking causing gum disease and loss of teeth, which are embarrassing and expensive problems. It’s also possible to become disabled as a result of one of the non-lung cancers that are more frequent in smokers. The list of such cancers is impressive, and involves potentially the tongue, mouth, larynx, esophagus (swallowing tube), stomach, pancreas, kidney, and urine bladder. Smokers increase their risk for all of these cancers, which may require treatments that are themselves disabling. If I were a more talented desktop publisher, I would add pictures of patients who have endured radical and disfiguring surgery for some of these cancers. But you have probably already seen some of those images on TV as a result of the new series of ads from the American Cancer Society. At this point, it should be clear that smoking is an excellent way to insure that you will become disabled in some fashion; in fact, most smokers can expect to encounter one or more of these problems in a lifetime of smoking, in addition to the premature skin wrinkling which is a significant concern for many female smokers. The good news is that more effective medication support is now available for those smokers who have made the commitment to quit. Recent studies confirm that both Zyban and Chantix tablets when combined with nicotine replacement therapy, can improve the quit rate for smokers. Most smokers do need multiple tries however, and the odds of success actually increase with successive attempts. One final caution regarding a strategy which doesn’t work...ie the misconception that it’s safe to just cut back to a few cigarettes daily. In fact there is no safe dose of inhaled tobacco smoke, and even household members in a smoking home have been shown to have a significantly increased risk of lung cancer and heart disease, the socalled “second hand” smoke factor. This scientific finding has led to the banning of smoking in virtually all public places in the US. From another perspective, it’s also difficult for most smokers to stick with just a few butts daily. If all else fails, there is also the option of electronic cigarettes, which deliver nebulized nicotine but virtually none of the carcinogen levels of cigarette smoke. The FDA is trying to make it more difficult to purchase E cigarettes, but they are clearly far safer than smoked tobacco, without the disabling complications described in this sobering summary. More information about the risk of COPD can be found at: www.ncbi.nlm.nih.gov/pmc/articles/PMC2672326/ and on quitting supports at: www.cdc.gov/tobacco/quit_smoking/index.htm and on overall smoking risks at; www.cdc.gov/tobacco/basic_information/health_effects/index.htm Chapter 12 Our Deadliest Habits: New Perspectives Section 2 Caloric Excess: The Obesity Epidemic and Its Consequences When I graduated from college in 1972, about 12% of adult Americans were obese, defined as a body mass index (BMI) greater than 30. That rate is now hovering around 35%, a dramatic increase that has also spawned a related epidemic of diabetes (BMI is calculated as the weight in Kg divided by the square of height in meters; a calculator is linked at the end of this chapter). Obese patients also have increased risk for heart attacks, high blood pressure, stroke, breast and colon cancer, degenerative arthritis and the need for joint replacement, so the overall impact on US medical costs has been catastrophic, estimated at over $200 billion annually by the US Office of Management and Budget. To put this weight gain in perspective, it is useful to review a hypothetical example. Consider the status of a male 5 feet 10 inches in height with a healthy weight of 160 pounds, translating to a BMI of 23, which is in the range of normal. That individual would need to gain 50 pounds, to a weight of 210, in order to reach the obesity threshold with a BMI of 30. That would be equivalent to carrying a 50 lb bag of cement all day long. Try doing that for 10 minutes during your next trip to a home improvement store, and you are likely to give up after 5 minutes. It’s a bit like the old saw about frogs and boiling water: if you raise the temperature gradually, the frog will stay in the pot until he’s cooked. Likewise, the gradual increase in weight is tolerated even though it creates enormous stress on the body, wearing out our pancreas and our joints, which were not designed to handle the excess load. Pediatric obesity is also a major concern, since it is recognized that children who are overweight, above the 85th weight percentile, are much more likely to become obese adults. The increase in childhood obesity has also caused a dramatic increase in the number of children diagnosed with type II diabetes, a disease which was previously rare in children. The emotional toll of childhood obesity is also well recognized, with lasting negative impact for many of these children following the relentless teasing which they often encounter. Research reveals that childhood weight problems are much more common if one or both parents are also obese, creating an important incentive for obese adults to deal with this family problem. The causes for this epidemic are not surprising: As a country we eat daily about 500 more calories than we did 40 years ago, according to the FDA. During recent years, Americans are also eating less frequently at home with a big increase in fast food, which is primarily fried and loaded with processed carbs and sugar. The USDA estimates that restaurant and fast food calories constituted about 6.4% of our diet in 1977, rising to about 20% by 2008. Another perspective on the issue is apparent from household food expenditures, with about 26% spent outside the home in 1970, rising to 43% in 2012 (including take out food), according to data from the US Department of Agriculture. These unfortunate dietary trends have been accompanied by decreased activity levels for many Americans, who continue to increase time devoted to sedentary activities including online diversions and television. So the solution to the obesity epidemic is simple in principle: We need to consume fewer calories and increase our activity levels. Any sustained method of calorie reduction will work, particularly if it pares down the major culprits of fast food, fried food, and snacks. Regular exercise makes the task easier, decreasing the magnitude of the required calorie reduction. In practice, however, these prove to be challenging hurdles, particularly for those two-income families and single parent households who often have difficulty finding time to exercise or to shop for and prepare healthy foods for meals at home. Increased individual effort is required in order to make progress with either of the weight loss strategies, and many Americans, still struggling to recover from the recent national recession, don’t have energy or time to spare. Financial incentives could become a significant driver for change in this obesity equation, since Obamacare includes regulations that permit employers to adjust their contributions to employee health insurance premiums based on controllable risk factors such as obesity and smoking. In fact, 2014 federal regulations allow employers to decrease the portion of the employee premium contribution they make to as much as 30% for patients who continued to be obese, or for patients who do not take advantage of healthy behavior programs. These financial incentives are controversial and reflect the desperation of policymakers who understand that expenses for the Medicare and Medicaid programs are on track to bankrupt the country, in large measure from the linked obesity and diabetes epidemics. In fact, the Congressional Budget Office estimates that 20% of the increase in healthcare spending between 1987 and 2007 was caused by obesity, and the CDC reports that obese patients each generate more than $1,400 in excess medical costs annually. Federal policy makers are also aware that some private employers have already achieved substantial medical cost savings by means of similar financial incentives. In theory, a person who needs to lose 40 pounds to improve his/her health would want to be working for an employer willing to reward them financially for that difficult endeavor, which requires discipline, time, and energy. It’s clear, however, that many patients don’t want to or feel they cannot expend the required effort and, therefore, regard weight-based incentives as unethical and prejudicial. It would not surprise me to see this question referred ultimately back to the courts for adjudication, even though the life insurance industry crossed this threshold decades ago, charging higher rates for patients who are obese. Regardless of any insurance-based financial incentives, it will continue to be the responsibility of healthcare professionals to educate their patients regarding the risks associated with obesity. This proves to be a difficult task, since many patients take offense at being labeled with this mathematically defined diagnosis. My final comment on this topic is to suggest that patients collaborate honestly with their medical clinicians to begin specific obesity management strategies that are workable for them, in order to decrease their risk for future disability and to decrease also their own future financial burdens for medical care. Even modest weight loss in the range of 10 to 20 pounds can have favorable impact on blood pressure and blood glucose levels, so no weight reduction accomplishment goes unrewarded. A user-friendly BMI calculator is available at: www.mayoclinic.org/bmi-calculator/itt-20084938 And more information on this topic is available at: www.obesitycampaign.org/obesity_facts.asp www.cdc.gov/obesity/data/adult.html www.mayoclinic.org/diseases-conditions/obesity/basics/definition/con-20014834 Chapter 12 Our Deadliest Habits: New Perspectives Section 3 Alcoholism: Off the Front Page, But Still a Potent Killer Alcoholism has been out of the media spotlight recently, overshadowed by the epidemic of opioid abuse and related overdose deaths. However excessive alcohol use accounts for approximately 88,000 deaths annually in the United States, which is more than four times the number of deaths related to all illegal substances combined (see the CDC fact sheet linked at the end of the chapter). This tragic death rate is, however, just the tip of the iceberg of alcohol-related family destruction and misery. In truth, there are very few extended families that have not been impacted by alcohol abuse, still the most common drug addiction of our time. It is important to recognize the distinction between the addicted alcoholic and the more common problem drinker, who is not physically dependent on alcohol but is it risk for that progression. Problem drinking can be defined per the CDC as any of the following: Binge drinking, with men ingesting five or more drinks in one day and women ingesting four or more. Heavy chronic drinking, with men consuming 15 or more drinks per week, and women with more than eight. Problem drinkers are at risk for motor vehicle accidents and other trauma, and they may also have evidence of liver injury, stomach inflammation, high blood pressure, or pancreas injury as a result of their heavy drinking. The problem drinker who progresses to a physical dependence on alcohol increases his or her risk for all of these complications, and adds also life-threatening risks for seizures, delirium tremens (DTs), liver failure with cirrhosis, and severe bleeding from the stomach and intestines. Updated diagnostic criteria from the American Psychiatric Association (DSM-5) combine problem drinking and alcohol dependence into a single disorder called “alcohol use disorder”, but medical clinicians still find it useful to distinguish these two disorders since patients with physical dependency are at risk for lifethreatening DTs if they are deprived of alcohol for any reason, including a hospitalization for unrelated illness. For this text, I’ll refer to alcohol use disorder as alcoholism, a term more familiar to laypersons. Alcoholics Anonymous (AA) posts on its website the following 12 questions to identify individuals who qualify for the diagnosis of alcoholism, based on a “yes” response to four or more questions: Have you ever decided to stop drinking, but only lasted a few days? Do you wish that people would stop telling you to drink less alcohol? Have you ever switched to a different type of alcoholic drink to avoid getting drunk? Have you had an eye-opener on awakening in the past year? Do you envy people who can drink without getting into trouble? Have you had problems connected with alcohol in the past year? Has your drinking caused trouble at home? Do you ever tried to get extra drinks at a party because you didn’t get enough? Do you tell yourself that you can stop at any time you want, even though you keep getting drunk when you don’t mean to? Have you missed days of work or school due to alcohol? Do you have blackouts? Have you ever felt that your life would be better if you didn’t drink? This survey has been validated by thousands of alcoholics who have benefited from the services offered by AA, and can also be used by family members to assess their concerns about a loved one. A shorter version of this diagnostic tool called the “CAGE” questions is used by medical professionals to evaluate patients for alcoholism. The CAGE questions are: Have you ever felt you should Cut down your drinking? Have people Annoyed you by criticizing your drinking? Have you ever felt bad or Guilty regarding your drinking? Have you ever had a drink first thing in the morning (Eye-opener) to steady your nerves or to get rid of a hangover? Patients who responded yes to two or more of the CAGE questions have a high probability of alcoholism, identifying more than 90% of patients with that diagnosis. It is not unusual for physicians to be contacted by family members concerned about alcoholism in a spouse, child, or relative. In many of these cases, the family member wishes to remain anonymous for fear of retaliation. Most healthcare professionals can accommodate these requests and may elect to perform screening blood tests at the time of a future appointment with the patient. Those tests could include a random blood alcohol level, as well as tests for liver function and red blood cell size. These tests will often be abnormal in patients with alcoholism, and can provide leverage for the physician to address the problem with the patient, without revealing any family member contacts. Family members looking for assistance with a suspected alcoholic in the household can also contact Al-Anon or Alateen, where group support can help individuals find solutions that can alleviate the pain associated with these difficult family situations. Pathologic behaviors including inappropriate tolerance and enabling of abuse by an alcoholic can also be addressed effectively in the group setting, or with personalized professional psychology counseling. Once the alcoholic agrees to pursue treatment, it is necessary for a healthcare professional to assess the risk for DTs, with inpatient detox programs required those individuals at risk for this dangerous withdrawal syndrome. Patients who are medically cleared in this regard may then benefit from newer medications designed to help alcoholics stay sober. Antabuse medication has been used for this purpose in the past, but causes severe nausea and vomiting with any alcohol intake. The newer medication naltrexone simply blocks the high which alcohol causes, without the nasty side effects. The new med acamprosate tends to decrease alcoholic cravings, to assist the treatment process. The seizure medications topiramate and gabapentin can also be used to maintain abstinence, though these are not FDA approved uses for those meds. Many alcoholics are not aware that these new medications are available to help them through the treatment process, so mention of these options may help to persuade some alcoholics to seek treatment. Denial is a major feature of the disease, so many avenues of approach may be needed help the patient reach the final conclusion that he/she needs treatment. No progress can be made without that critical patient decision. More information on this topic is available at: www.cdc.gov/alcohol/fact-sheets/alcohol-use.htm www.aa.org and www.mayoclinic.org/diseases-conditions/alcoholism/basics/definition/con20020866 About the Author Dr. Michael Kelleher has 34 years of experience as a physician and medical executive responsible for patient safety and quality of care in large group practices in Texas and in Massachusetts. He graduated from the Amherst College magna cum laude with a BA in chemistry and completed his MD training at the University of Rochester New York, followed by internal medicine internship and residency at the University of Minnesota Hospitals. He achieved board certification in internal medicine and emergency medicine and practiced in both settings during his medical career. Dr. Kelleher served as a member of the Massachusetts Medical Society Committee on the Quality of Medical Practice, and was also a member of the American College of Medical Quality. He co-authored in 2003 a seminal paper in the Journal of the American Medical Association on “Adverse Drug Events Among Older Persons in the Ambulatory Setting, “and has presented at regional and national medical forums on the patient safety and clinical performance improvement programs which he developed as Medical Director For Quality and Patient Safety at Reliant Medical Group. He continues to work as a medical consultant with particular focus on the transparent management of medical adverse events, including disclosure and apology interventions.