RESTRICTIVE PULMONARY DISORDERS: AN OVERVIEW

Transcription

RESTRICTIVE PULMONARY DISORDERS:
AN OVERVIEW
by
Aimee Staggenborg
RRT, MA, BA, RCP
RC Educational Consulting Services, Inc.
16781 Van Buren Blvd, Suite B, Riverside, CA 92504-5798
(800) 441-LUNG / (877) 367-NURS
www.RCECS.com
AN OVERVIEW
BEHAVIORAL OBJECTIVES
UPON COMPLETION OF THE READING MATERIAL, THE PRACTITIONER WILL BE
ABLE TO:
1. Gain new knowledge about restrictive pulmonary diseases and disorders.
2. Identify the physiologic differences of restrictive lung diseases from those with an
obstruction component.
3. Learn to identify the unique signs and symptoms of restrictive diseases.
4. Assess early risk factors that could contribute to development of an occupational lung
disease.
5. Make recommendations for testing for restrictive disorders with presentation of acute or
chronic symptoms.
6. Learn how restrictive disorders are diagnosed.
7. Know about the course of treatment and medical care for lung diseases, and how they differ
from treatment of obstructive lung diseases.
8. Know how to select which treatment and medical care is appropriate for each disease
process.
9. Understand about the long term prognosis that comes with restrictive lung diseases.
10. How special considerations can affect managing care of patients that suffer from restrictive
disorders.
COPYRIGHT © 2009 BY RC EDUCATIONAL CONSULTING SERVICES, INC.
AUTHORED (2009) BY AIMEE STAGGENBORG, RRT, MA, BA, RCP
ALL RIGHTS RESERVED
This material is copyrighted by RC Educational Consulting Services, Inc. Unauthorized duplication is prohibited by law.
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AN OVERVIEW
This course is for reference and education only. Every effort is made to ensure that the
Clinical principles, procedures and practices are based on current knowledge and state of
the art information from acknowledged authorities, text and journals. This information is
not intended as a substitution for diagnosis or treatment given in consultation with a
qualified health care professional.
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AN OVERVIEW
TABLE OF CONTENTS
INTRODUCTION .............................................................................................................. 8
FREQUENCY..................................................................................................................... 8
UNITED STATES ......................................................................................................... 8
INTERNATIONAL ....................................................................................................... 9
MORTALITY / MORBIDITY ...................................................................................... 9
PREVALENCE BY POPULATION............................................................................. 9
ALVEOLAR CAPILLARY MEMBRANE ....................................................................... 9
BACKGROUND ......................................................................................................... 10
CHARACTERISTICS OF RESTRICTIVE DISORDERS .............................................. 10
PATHOPHYSIOLOGY OF RESTRICTIVE DISEASES .......................................... 11
PATIENT HISTORY................................................................................................... 12
CLINICAL CAUSES........................................................................................................ 14
INTRINSIC LUNG DISORDERS (LUNG PARENCHYMA DISEASE) ................. 14
FIBROTIC DISORDERS (INTERSTITIAL LLUNG DISEASES) ........................... 14
PULMONARY FIBROSIS.......................................................................................... 14
SACRCOIDOSIS......................................................................................................... 14
ACUTE LUNG INJURY............................................................................................. 15
PLEURISY .................................................................................................................. 15
LUNG CANCER ......................................................................................................... 15
ASBESTOSIS .............................................................................................................. 16
PNEUMONIA.............................................................................................................. 16
RESPIRATORY DISTRESS SYNDROMES .................................................................. 17
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AN OVERVIEW
ARDS........................................................................................................................... 17
SARS............................................................................................................................ 17
IRDS ............................................................................................................................ 17
EXTRINSIC LUNG DISORDERS (EXTRAPARENCHYMAL DISEASES) ............... 18
NEUROLOGICAL DISORDERS ............................................................................... 18
SPINAL CORD INJURY ....................................................................................... 18
CEREBRAL PALSY (CP) ..................................................................................... 18
CENTRAL SLEEP APNEA (CSA)........................................................................ 19
MECHANICAL DISORDERS............................................................................... 19
ASCENDING LATERAL SCLEROSIS (ALS)..................................................... 19
MYASTHENIA GRAVIS (MG) ............................................................................ 19
GUILLIAN BARRE ............................................................................................... 20
MUSCULAR DYSTROPHY (MD) ....................................................................... 20
SCOLIOSIS / KYPHOSIS / KYPHOSCOLIOSIS................................................. 20
PHYSICAL MANIFESTATIONS ................................................................................... 21
INTRINSIC DISORDERS........................................................................................... 21
EXTRINSIC DISORDERS ......................................................................................... 21
CLINICAL WORK UP..................................................................................................... 21
LAB STUDIES ............................................................................................................ 21
INTRINSIC LUNG DISEASES .................................................................................. 21
IMAGING STUDIES .................................................................................................. 22
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(X-RAY) CHEST RADIOGRAPHY FOR INTRINSIC LUNG DISORDERS .... 22
(CT SCAN) COMPUTED TOMOGRAPHY OF THE CHEST ............................ 22
TESTS FOR EXTRINISIC DISORDERS.............................................................. 23
(PFT) PULMONARY FUNCTION TESTS........................................................... 23
PROCEDURES ........................................................................................................... 24
BRONCHOSCOPY ................................................................................................ 24
BRONCHOALVEOLAR LAVAGE (BAL) .......................................................... 24
LUNG BIOPSY ...................................................................................................... 24
SURGICAL LUNG BIOPSY ................................................................................. 24
THORACENTESIS PROCEDURE ....................................................................... 24
HISTOLOGICAL FINDINGS.......................................................................................... 24
MEDICAL CARE AND TREATMENT.......................................................................... 25
INTRINSIC DISORDERS........................................................................................... 25
SURGICAL PROCEDURES............................................................................................ 27
GOALS OF TREATMENT......................................................................................... 27
CONSULTATION CONSIDERATIONS ................................................................... 28
DETERRENCE / PREVENTION ............................................................................... 28
COMPLICATIONS ..................................................................................................... 28
PROGNOSIS ............................................................................................................... 29
MEDICAL / LEGAL PITFALLS................................................................................ 29
SPECIAL CONCERNS ............................................................................................... 29
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AN OVERVIEW
CLINICAL SCENARIO’S ............................................................................................... 30
CLINICAL SCENARIO DISCUSSIONS ........................................................................ 32
SUMMARY...................................................................................................................... 34
SUGGESTED READING AND REFERENCES ............................................................ 35
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AN OVERVIEW
INTRODUCTION
R
estrictive pulmonary diseases represent only half of the spectrum in the lung disorder
equation. They may range in severity and complexity and in the health care and medical
treatments needed. The ultimate goals for management of the patient with any
pulmonary disease are control of illness related symptoms, improved quality of life, and
extension of life expectancy. All lung diseases have a common ground, the patient who lives
with the disease. The only thing these patients value is the medical care that can improve his/her
life. So extensive knowledge on restrictive diseases for RCP’s is necessary to provide the patient
with the valuable healthcare treatments they seek. This educational course will provide and
overview of many common restrictive lung diseases. Provided in the following sections is
information on the signs, symptoms and diagnostic testing related to treating lung disorders that
impair the ability to expand the lungs. These types of disorders may be chronic conditions or
may exacerbate acutely with caused by a lung infection. Ongoing medical care is great to treat
the immediate causes of respiratory diseases, but the most important information RCP’s should
provide to their patients is education about the patient’s disease, how to self treat, how to manage
the illness, and how to prevent recurrent exacerbations (if preventable by disease process), and
the importance of compliance with MD ordered therapy.
FREQUENCY
United States
F
or intrinsic lung diseases, studies cite an overall prevalence of 3-6 cases per 100,000
persons, with a prevalence of idiopathic pulmonary fibrosis (IPF) of 27 - 29 cases per
100,000 persons. The prevalence for adults aged 35 - 44 years is 2.7 cases per 100,000
persons. Prevalence exceeded 175 cases per 100,000 persons among patients older than 75
years. Exposure to dust, metals, organic solvents, and agricultural employment is associated
with increased risk.
•
In North America, the prevalence of sarcoidosis is 10 - 40 cases per 100,000 persons.
•
The incidence of chronic interstitial lung diseases in persons with collagen vascular
diseases is variable, but it is increasing for most diseases.
•
Kyphoscoliosis is a common extrinsic disorder. It is associated with an incidence of mild
deformities amounting to 1 case per 1000 persons, with severe deformity occurring in 1
case per 10,000 persons.
•
Other nonmuscular and neuromuscular disorders are rare, but their incidence and
prevalence are not well known. (Sharma, 2006)
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AN OVERVIEW
International
In Sweden, the prevalence rate for sarcoidosis is 64 cases per 100,000 persons. In Japan, the
prevalence rate of sarcoidosis is 10-40 cases per 100,000 persons. The prevalence of sarcoidosis
is difficult to determine, and tuberculosis is common.
•
The worldwide prevalence of fibrotic lung diseases is difficult to determine because
studies have not been performed. (Sharma, 2006)
Mortality / Morbidity
The median survival time for patients with Idiopathic Pulmonary Fibrosis (IPF) is less than 3
years. Factors that predict poor outcome include older age, male gender, severe dyspnea, history
of cigarette smoking, severe loss of lung function, appearance and severity of fibrosis on
radiologic studies, lack of response to therapy, and prominent fibroblastic foci on histopathology
evaluation. Those patients with neuromuscular disorders often succumb to respiratory distress,
then respiratory failure, coupled with lung infections from impaired cough. The rate of mortality
is proportional to availability of care for treatment, family support, compliance to treatment
routine and ongoing exposure to environmental irritants. Restrictive disorders are not reversible,
but somewhat manageable with medical care.
Prevalence by Population
•
Age prevalence although a familial variant of IPF exists, a genetic predisposition is not
documented. US prevalence of sarcoidosis is estimated to be 10 - 17 times higher among
African Americans compared to white Americans.
•
Race Lymphangioleiomyomatosis (LAM) and lung involvement in tuberous sclerosis
occur exclusively in premenopausal women. Men are more likely to have
pneumoconiosis because of occupational exposure, IPF, and collagen vascular diseases
(rheumatoid lung). Worldwide, sarcoidosis is slightly more common in women.
•
Sex IPF is rare in children. Some intrinsic lung diseases present in patients aged 20 - 40
years. These include sarcoidosis, collagen vascular-associated diseases, and histiocytosis
X. Most patients with IPF are older than 50 years.
ALVEOLAR CAPILLARY MEMBRANE
T
he alveolar capillary membrane is the thin layer of tissue between the alveolar sacs and
the blood in the pulmonary capillaries by which oxygen and carbon dioxide diffuses.
Many restrictive disorders are affected by low levels of pulmonary surfactant in the lungs.
Surfactant is a protein based substance in the lungs that coats the surface area of the lung sacs
and helps to reduce surface tension. Surfactant functions to increase pulmonary lung compliance
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AN OVERVIEW
and prevent lung collapse and the end of expiration. Low levels of lung surfactant decreases the
diffusing ability for gas exchange across the alveolar capillary membrane. Acute Infant
respiratory syndrome occurs with inadequate surfactant levels, affecting the lungs’ ability to
expand and ventilate.
Background
Restrictive pulmonary disorders are classified by decreased lung capacity, either because of an
alteration in lung wall lining or a disease of the internal wall of the lung, or neuromuscular
mechanism. In physiology terms, restrictive lung diseases are identified by reduction in total
lung capacity (TLC), vital capacity, or resting lung volume. Associated characteristics are
conversed airflow and regular airway resistance, which are calculated as the functional residual
capacity (FRC). If caused by parenchymal lung disease, restrictive lung disorders are
accompanied by decreased gas exchange, which may be clear clinically by desaturation
following exertion.
The many disorders that cause reduction or restriction of lung volumes may be divided into 2
groups based on anatomical structures.
•
The first is intrinsic lung disorders or disorders of the lung lining. The diseases cause
swelling or scarring of the lung tissue (interstitial lung disease) or cause influx of the air
spaces with secretions and waste. (These diseases can be characterized according to
etiological factors. They include: idiopathic fibrotic diseases, connective tissue diseases,
drug-induced lung disease, and primary diseases of the lungs, including sarcoidosis).
(Sharma, 2006)
•
The second is extrinsic disorders or extraparenchymal diseases. The chest wall, pleura,
and respiratory muscles are the components of the respiratory pump, and they need to
function normally for effective ventilation. Diseases of these structures result in lung
restriction, impaired ventilatory function, and respiratory failure (eg, nonmuscular
diseases of the chest wall, neuromuscular disorders). (Sharma, 2006)
CHARACTERISTICS OF RESTRICTIVE DISORDERS
•
Loss of lung tissue comes from exposure to environmental irritants, connective tissue
diseases; interstitial lung diseases that destroy fragile pulmonary tissue. Fibrotic scar
tissues form in the space. Fibrotic tissue does not have the ability to exchange gas. This
type of damage is not reversible.
•
Decrease of lungs ability to expand is affected by the actual available space in the lungs
to expand. In the case of extrinsic disorders the area for expansion is restricted by
physical limitations in the lung. If the expansion mechanism in the lung is impaired the
capacity of the lungs is reduced. This leads in hypoventilation for inadequate ventilation
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AN OVERVIEW
with V/Q mismatch and hypoxia. It becomes difficult to properly oxygenate the tissues
of the body if the ability to ventilate the lungs is adversely affected.
•
Decrease of lungs ability to transfer oxygen or carbon dioxide in and out of the blood
when lung damage exists. Reduction of the available surface area for gas diffusion is
uneven when fibrotic scar tissue present. Dyspnea is indicative of restrictive disorders as
the body tries to compensate for shallow breaths by increasing the respiratory rate
causing tachypnea or hyperventilation.
Pathophysiology of Restrictive Diseases
Restrictive Lung Disorders (RDL) are diseases and disorders that impair both lung expansion
and lung compliance (RLD) diseases and disorders cause the lungs to become “stiff” and
increase inability to ventilate the lungs. Without proper ventilation of the lungs become less able
to get rid carbon dioxide. The by products of metabolism are retained in the lung’s tiny air sacs.
This change manifests itself in a reduced Total Lung Capacity, Inspiratory Capacity and Vital
Capacity. As the elasticity of the lungs become weaker the patient may begin to suffer from
hypoventilation, hypoxia, and inability to clear lung secretions with ineffective coughing.
Restrictive lung disease is a chronic disorder that causes a decrease in the ability to expand the
lung (breathe in) and sometimes makes it harder to get enough oxygen to meet the body's needs.
The most common restrictive pulmonary diseases are: Interstitial pulmonary fibrosis/interstitial
lung disease (including Sarcoidosis-granulomatous disorder) extra pulmonary restrictive lung
disease (including scoliosis). Interstitial lung disease is a disease of the lung parenchyma and
connective tissue. The lung parenchyma is the covering of the lungs. The connective tissue is
the tissue that holds the air sacs together. Interstitial lung disease happens when the lung
parenchyma is damaged in some way and inflammation occurs. Changes in the parenchyma and
connective tissue happen when the inflammation is chronic. Once significant amount of damage
to the air sacs develop, the disease is not reversible. In advanced disease, air sac dilation occurs,
resulting in impaired blood flow in the lungs. This reduced blood flow can reduce the amount of
oxygen available for your body to use and increase shortness of breath. There are more than 100
different interstitial lung diseases. In about 70 percent of patients the cause of the disease
remains unknown.
Known causes are occupational and environmental inhaled irritants (dust, gases, fumes, fiber
glass, asbestosis, or aerosols), radiation, poisons and drugs. Sarcoidosis is one type of interstitial
lung disease. Sarcoidosis is a multi-system disorder which commonly affects the lungs, skin and
eyes. During chronic inflammation, normally elastic tissue stiffens, decreasing the flexibility of
the lung tissue. Affected tissue in the lung looks like a honeycomb. The cause of sarcoidosis is
unknown. In contrast to OPD, RLD values for Tidal Volume, Expiratory Reserve Volume,
Functional Residual Capacity and Respiratory Volume are unchanged. The FEV1 and FCV for a
patient with RLD will be decreased however FEV1/FVC ratio will be normal or increased for a
RLD patient.
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AN OVERVIEW
Patient History
Initial or ongoing diagnosis is affected by information revealed in the patient past medical
history. Each patient should receive an initial assessment consisting of a complete history,
including onset of symptoms, past environmental exposure, habits, recent travels, occupation
history, hobbies, and HIV risk factors is key for determination of etiologic cause.
•
Duration of symptoms for diagnosis of restrictive disorders factor in the treatment and
plan of care. Acute disorders with symptoms that may last from a few days to a few
weeks, pneumonia, pneumonitis or alveolar hemorrhage have a sudden onset and require
immediate medical intervention. Chronic disorders occur frequently with numerous
exacerbations over several months to several years. Treatment of chronic disorders focus
on reduction of symptoms, hospitalizations, improvements in ADL’s (activities of daily
living), and improved overall quality of life.
•
Smoking history is important when assessing signs and symptoms of pulmonary disease.
History of smoking has been shown it contribute to restrictive pulmonary diseases.
Impairment of the respiratory cilliary function affects the lungs ability to deep cough and
expels irritants and foreign particulate for the lungs, resulting in concurrent lung
infections. Histiocytosis X, desquamative interstitial pneumonitis, IPF, and respiratory
bronchiolitis occur with increased frequency among persons who smoke or those who
previously smoked. Assessment of past exposure to smoking is necessary to determine
contributing factors for current clinical presentation.
•
Prior medication use. An increasing number of drugs are recognized to induce
distinctive patterns of infiltrative lung disease (ILD), ranging from benign infiltrates to
life-threatening adult respiratory distress syndromes. Drug-induced respiratory disease
from prior drug use warrants a detailed past history of medication use as a tool to rule out
or confirm origination of a pulmonary disease. Syndromes of drug induced may cause
mild effects or major life threatening respiratory distress and impending respiratory
failure, pulmonary diseases may include: asthma, bronchiolitis obliterans organizing
pneumonia (BOOP) hypersensitivity infiltrate, interstitial pneumonitis or fibrosis,
noncardiogenic pulmonary edema, pleural effusions, pulmonary infiltrates with
eosinophilia or pulmonary vascular disease. Considerations to explore when assessing
past usages of drugs and medications can give a blue print for where to start questions for
the patient to help arrive at preliminary conclusions. Some common drugs that can cause
pulmonary disease may include: anticonvulsant, antipsychotic, antidepressants (i.e.:
fluoxetine, phenothiazines phenytoin), anti-inflammatory, (i.e.: aspirin, gold,
methotrexate), antimetabolic, (i.e.: cytarabin fludarabine), biologic response modifiers,
(i.e.: Interferon, Interleukin-2), Cardiovascular, (i.e.: beta-blockers, anticoagulants),
chemotherapeutic and immunosuppressive agents, intravenous substances, (i.e.: blood
products), illicit drugs, (i.e.: cocaine, heroin, methadone, methylphenidate narcotic and
sedative drugs). Misc. agents, (i.e.: appetite suppressants, mineral oil, silicone, and
radiation therapy).
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AN OVERVIEW
•
Family history has not been shown to hide restrictive pulmonary diseases in primary or
recessive DNA genes, however a familiar link has been found to exist in (IPF) interstitial
pulmonary fibrosis, sarcoidosis, and LAM.
•
Occupational exposure. A detail patient history of employment and know exposures to
toxins, is necessary for assessment of pulmonary diseases. Discussion of exposure to
inhaled irritants could involve education about risk factors and history. Occupational
lung disease is the number one work-related illness in the United States based on the
frequency, severity, and preventability of diseases. These illnesses are usually caused by
extended exposure to irritating or toxic substances that may cause acute or chronic
respiratory ailments, although severe single exposures can cause chronic lung disease as
well. Known occupational related disorders: Occupational lung cancer, asbestosis,
mesothelioma, byssinosis, black lung disease, Silicosis, Hypersensitivity pneumonitis,
WTC lung disease. (Further examination of diseases is discussed later in this course).
•
Environmental exposure is similar to those occurring during occupational exposure
however, environmental exposure may occur in a less controlled situation, such as in the
home or in the outdoors. The patient assessment for this type of exposure is the same as
occupational. Some environmental considerations: Stachybotrys chartarum, and
Aspergillus.
•
Symptoms of intrinsic diseases. Progressive proportional dyspnea is the primary
symptom. Assessing the severity of dyspnea is useful as a method to determine the
advancement of the disease and to follow its course. A dry cough is common and may be
a tell-tell sign. A productive cough is a common sign in most patients with diffuse
parenchyma lung disorders. Hemoptysis or grossly bloody sputum occurs in patients
with diffuse alveolar hemorrhage syndromes and vasculitis. Wheezing is an uncommon
manifestation but can occur in patients with lymphangitic carcinomatosis, chronic
eosinophilic pneumonia, and respiratory bronchiolitis. Chest pain is uncommon in most
instances of the disease, but pleuritic chest pain can occur in patients with rheumatoid
arthritis, systemic lupus erythematosus, and some drug-induced disorders.
•
Symptoms of extrinsic diseases. Nonmuscular diseases of the chest wall affect patients
with scoliosis or kyphosis or combination of both. Patients under 35 years of age may be
asymptomatic, with higher risk middle-aged patients developing shortness of breath,
decreased activity tolerance, and recurrent respiratory infections. The cause of
respiratory failure is often variable between several function and is secondary to spinal
deformity, muscle weakness, disordered ventilatory control, sleep disordered breathing,
and airway disease. Neuromuscular disorders occur as the respiratory muscle weakness
progresses. Patients develop dyspnea upon exertion, followed by dyspnea at rest, and
their condition ultimately advances to respiratory failure. Patients with neuromuscular
diseases develop significant respiratory muscle weakness and may demonstrate fatigue,
dyspnea, impaired control of secretions, and recurrent lower respiratory tract infections.
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AN OVERVIEW
Acute and chronic respiratory failure, pulmonary hypertension, and cor pulmonale
eventually ensue. Disorders include: ALS, MD, and MS.
CLINICAL CAUSES
Intrinsic Lung Disorders (Lung Parenchyma Disease)
Fibrotic Disorders (Interstitial Lung Diseases)
I
nterstitial Lung Disease (ILD) is a general term that includes a variety of chronic lung
disorders. When a person has ILD, the lung is affected in three ways. First, the lung tissue is
damaged in some known or unknown way. Second, the walls of the air sacs in the lung
become inflamed. Finally, scarring (or fibrosis) begins in the interstitium (or tissue between the
air sacs), and the lung becomes stiff. The tissue between the air sacs of the lungs is called the
interstitium. Interstitial lung disease is named after this tissue because this is the tissue affected
by fibrosis (scarring). Interstitial lung disease is sometimes also known as “interstitial
pulmonary fibrosis”. The terms interstitial lung disease, pulmonary fibrosis and interstitial
pulmonary fibrosis are often used to describe the same condition. The course of these diseases is
unpredictable. If they progress, the lung tissue thickens and becomes stiff. The work of
breathing then becomes more difficult and demanding. Some of the diseases improve with
medication if treated when inflammation occurs. Some people may need oxygen therapy as part
of their treatment.
Pulmonary Fibrosis
Pulmonary fibrosis is generally idiopathic when diagnosed. Pulmonary Fibrosis involves
scarring of the lung. Gradually, the air sacs of the lungs become replaced by fibrotic tissue.
When the scar forms, the tissue becomes thicker causing an irreversible loss of the tissue’s
ability to transfer oxygen into the bloodstream. Traditional theories have postulated that it might
be an autoimmune disorder, or the after effects of an infection, viral in nature. There is a
growing body of evidence which points to a genetic predisposition. A mutation in the SP-C
protein has been found to exist in families with a history of Pulmonary Fibrosis. The most
current thinking is that the fibrotic process is a reaction to microscopic injury to the lung. While
the exact cause remains unknown, associations have been made with the following: Inhaled
environmental and occupational pollutants; cigarette smoking; diseases such as Scleroderma;
Rheumatoid Arthritis; Lupus and Sarcoidosis; Certain medications; therapeutic radiation.
Clinical presentation: Shortness of breath, particularly with exertion; chronic dry, hacking
cough; fatigue and weakness; discomfort in the chest; loss of appetite; rapid weight loss.
Sacrcoidosis
An immune system disorder characterized by non-caseating granuloma (small inflammatory
nodules) that most commonly arises in young adults. The cause of the disease is still unknown.
Virtually any organ can be affected, however granulomas most often appear in the lungs or the
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AN OVERVIEW
lymph nodes. Symptoms can occasionally appear suddenly but usually appear gradually. The
clinical course varies and ranges from asymptomatic disease to a debilitating chronic condition
that may lead to death. Sarcoidosis most often manifests as a restrictive disease of the lungs,
causing a decrease in lung volume and decreased compliance (the ability to stretch). The disease
typically limits the amount of air drawn into the lungs, but produces higher than normal
expiratory flow ratios. The vital capacity (full breath in, to full breath out) is decreased, and
most of this air can be blown out in the first second. Sarcoidosis is a systemic disease that can
affect any organ. Common symptoms are vague, such as fatigue unchanged by sleep, lack of
energy, weight loss, aches and pains, arthralgia, dry eyes, blurry vision, shortness of breath, a dry
hacking cough or skin lesions. The cutaneous symptoms vary, and range from rashes and noduli
(small bumps) to erythema nodosum or lupus pernio. It is often asymptomatic.
Acute Lung Injury
Acute lung injury is generally caused by a direct injury to the lungs. These lung injuries cause
inflammation to the fragile lung tissue. Inflammatory disorders of the lung are most commonly
caused by sepsis, pneumonia, trauma, and/or aspiration following an acute lung injury. Damage
to the endothelium and the alveolar epithelium results in the creation of an open interface
between the lung and the blood, facilitating the spread of micro-organisms from the lung
systemically, stoking up a systemic inflammatory response. Moreover, the injury to epithelial
cells handicaps the lung’s ability to pump fluid out of airspaces. Fluid filled airspaces, loss of
surfactant, microvascular thrombosis and disorganized repair (which leads to fibrosis) reduces
resting lung volumes (decreased compliance), increasing ventilation-perfusion mismatch, right to
left shunt and the work of breathing.
Pleurisy
Pleurisy also known as plueritis, inflammation, swelling and irritation of the pleural chest wall
lining surrounding the lungs, which can cause painful respiration (also called pleuritic chest pain)
and other symptoms. Pleurisy can be generated by a variety of infectious and non-infectious
causes. The effects of pleurisy can often be felt long after the condition has gone away. Pleurisy
results following other illnesses such as viral or bacterial infections of the lungs, injury or trauma
to the lungs may also cause pleurisy. Often deemed idiopathic by doctors, pleurisy may be acute
in nature with a sudden onset. Sharp stabbing is felt with inspiration, and may subside between
breaths. Deep breathing, coughing, or movement may make the pain feel worse and more
intense. Often the pain is localized in a specific location which may be pin-pointed, quick
shallow breathing may result from the deep pain.
Lung Cancer
Lung cancer is a disease of uncontrolled cell growth in tissues of the lung. This growth may lead
to metastasis, invasion of adjacent tissue and infiltration beyond the lungs. The vast majority of
primary lung cancers are carcinomas of the lung, derived from epithelial cells. Lung cancer is
the most common cause of cancer-related death in men and the second most common in woman.
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AN OVERVIEW
The main causes of lung cancer (and cancer in general) include carcinogens (such as those in
tobacco smoke), ionizing radiation, and viral infection. This exposure causes cumulative
changes to the DNA in the tissue lining the bronchi of the lungs (the bronchial epithelium). As
more tissue becomes damaged, eventually a cancer develops.
Asbestosis
Asbestosis is a restrictive lung disorder caused by inhaling asbestos fibers. Prolonged exposure
over time can cause accumulation of particulate in the lung tissue. Extensive scaring to the lung
tissue can diminish the lungs capacity to diffuse gas across the alveolar capillary membrane into
the blood stream. Asbestosis is considered an occupational hazard that leads to this disorder.
Asbestosis is a chronic inflammatory medical condition affecting the parenchymal tissue of the
lungs. It occurs after long-term, heavy exposure to asbestos, e.g. in mining, and is therefore
regarded as an occupational lung disease. Sufferers have severe dyspnea (shortness of breath)
and are at an increased risk regarding several different types of lung cancer. Asbestosis is a
natural mineral product that is resistant to heat and corrosion. It has been used in the past in the
building and manufacturing industry. Some of its more common uses were in pipe and duct
insulation, fire-retardant materials, brake and clutch linings, cement, and some vinyl floor tiles.
The primary symptom of asbestosis is generally the slow onset of shortness of breath on
exertion. In severe, advanced cases, this may lead to respiratory failure. Coughing is not usually
a typical symptom, unless the patient has other, concomitant respiratory tract diseases. People
with extensive occupational exposure to the mining, manufacturing, handling or removal of
asbestos are at risk for developing asbestosis sometime in the future. Long tern use of tobacco
has shown to increase the risk factors of developing asbestosis symptoms.
Pneumonia
Pneumonia is an infection of the lung wall lining. There are several types of pneumonia
including viral, bacterial, fungi. Bacterial pneumonias tend to be the most serious and, in adults,
the most common cause of pneumonia. The most common pneumonia-causing bacterium in
adults is Streptococcus pneumoniae (pneumococcus). The route for transmission is inhalation of
small droplets containing pneumonia causing organisms. Pneumonia is often a complication of a
pre-existing condition/infection and triggered when a patient's defense system is weakened, most
often by a simple viral upper respiratory tract infection or a case of influenza, especially in the
elderly. Pneumonia affects the lungs in different ways. Lobar pneumonia affects a lobe of the
lungs, and bronchial pneumonia can affect patches throughout both lungs. Pneumonia can also
be caused by the inhalation of food, liquid, gases or dust. One type of pneumonia caused by
fungi is pneumocystis carinii pneumonia (PCP) which primarily affects AIDS patients. Certain
diseases, such as tuberculosis, can also predispose someone to pneumonia. People considered at
high risk for pneumonia include the elderly, the very young, and those with underlying health
problems, such as chronic obstructive pulmonary disease (COPD), diabetes mellitus, congestive
heart failure and sickle cell anemia. Patients with diseases that impair the immune system, such
as AIDS, or those undergoing cancer therapy or organ transplantation, or patients with other
chronic illnesses are particularly vulnerable. Initially symptoms mimic a cold which are then
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AN OVERVIEW
followed by a high fever (sometimes as high as 104 degrees Fahrenheit), shaking chills, and a
cough with sputum production. The sputum is usually discolored and sometimes bloody. People
with pneumonia may become short of breath. The only pain fibers in the lung are on the surface
of the lung, in the area known as the pleura. Chest pain may develop if the outer pleural aspects
of the lung are involved. This pain is usually sharp and worsens when taking a deep breath,
known as pleuritic pain.
RESPIRATORY DISTRESS SYNDROMES
•
(ARDS) - Adult respiratory distress syndrome is an acute, quick onset lung injury
effecting one or both lungs. Generally the injury covering most of the surface area of the
lungs. Patient’s who suffer from ARDS suffer from severe dyspena, which may require
mechanical ventilation to counter respiratory failure. ARDS is not a specific disease;
instead it is a type of severe, acute lung dysfunction that is associated with a variety of
diseases, such as pneumonia, shock, sepsis (a severe infection in the body) and trauma.
ARDS us characterized by inflammation of the lung parenchyma leading to impaired gas
exchange with concomitant systemic release of inflammatory mediators causing
inflammation, hypoxemia and frequently resulting in multiple organ failure. This
condition is life threatening and often lethal.
•
(SARS) - Sudden acute respiratory syndrome is an infectious respiratory disease in
humans which is caused by the SARS coronavirus (SARS-CoV). Initial symptoms are
flu like and may include: ever, myalgia, lethargy, gastrointestinal symptoms, cough, sore
throat and other non-specific symptoms. The only symptom that is common to all
patients appears to be a fever above 38°C (100.4°F). Shortness of breath may occur later.
Symptoms usually appear 2 - 10 days following exposure, but up to 13 days have been
reported. In most cases symptoms appear within 2 - 3 days. About 10 - 20% of cases
require mechanical ventilation.
•
(IRDS) - Infant respiratory distress syndrome is a syndrome caused in premature infants
by developmental insufficiency of surfactant production and structural immaturity in the
lungs. It can also result from a genetic problem with the production of surfactant
associated proteins. RDS affects about 1% of newborn infants and is the leading cause of
death in preterm infants. The incidence decreases with advancing gestational age, from
about 50% in babies born at 26 - 28 weeks, to about 25% at 30 - 31 weeks. Surfactant
deficient preemie lungs have higher surface area tension causing decreased ability to
ventilate and oxygenate the lungs. Respiratory distress syndrome begins shortly after
birth and is characterized by tachypnea, tachycardia, chest wall retractions, expiratory
grunting, nasal flaring and cyanosis during breathing efforts. As the disease progresses,
the baby may develop ventilatory failure (rising carbon dioxide concentrations in the
blood), and prolonged apneic episodes during the breathing cycle. Early interventions are
essential for better recovery outcomes. Initiation of surfactant replacement therapy early
on can help minimized potential lung damage. Nasal CPAP has also been shown to
deliver a continuous positive airway pressure, which helps to hyper-inflate the lungs with
17
AN OVERVIEW
inspiration, and prevent alveolar collapse upon expiration. Untreated IRDS can be fatal,
so early intervention is needed for the best possibility for treatment response and
recovery. (Further treatment information is covered later in the course).
EXTRINISIC LUNG DISORDERS (EXTRAPARENCHYMAL DISEASES)
Neurological Disorders
•
Spinal cord injury presents a unique condition where respiratory complications are
common, depending on location of the traumatic injury to spinal column. Injuries to
cervical vertebrae affect the nerves impulses that control brain signals to the respiratory
muscles, the higher the level of injury, the greater the loss to the respiratory muscle
control. The lungs and trachea are no usually affected with an injury, but complications
may occur post injury. Respiratory problems may occur when the signals sent from the
brain can no longer flow through the spinal cord to control the respiratory muscles. Loss
of control of the respiratory muscles can lead to a restrictive component affecting lung
expansion. Complete injuries in the thoracic or cervical regions usually result in the
permanent loss of respiratory muscle function below the level of injury. However,
Injuries in the thoracic area (T1 - T12) of the spinal cord affect the control of the intercostal and abdominal muscles. A lower level of injury, such as a T10, results in the
individual losing a small amount of muscle control. With a higher level of injury, such as
a T2, individuals will lose most of their inter-costal and abdominal muscle control. Loss
of respiratory muscle control often will involve respiratory failure and the inability for
the patient to breathe on his/her own. Mechanical ventilation is common for treating
muscle failure. This poor ability for lung expansion may result in pneumonia, atelectasis
and pulmonary embolism. Complications require adequate health maintenance and
protection from these complications are appropriate and necessary as part of the longterm care of he spinal cord-injured individual.
•
Cerebral Palsy (CP) characterized by faulty development or damage to the motor part of
the brain that controls body movement and posture. The disorder normally occurs as
result before or during birth (anoxic brain injury from asphyxia) or from head trauma or
infection in brain within the first months after birth. Spastic CP is the most common type
of cerebral palsy. It causes the muscles to be stiff and permanently contracted.
Respiratory complications may occur. Children with cerebral palsy do not frequently
have problems with their lungs as a direct result of their impairment. However,
depending upon the type of cerebral palsy they have, they may have physical (muscle
constriction) limitations that significantly add to respiratory problems. Difficulties in
swallowing, a weak cough, reflux, and seizure can all cause a child to accidentally
breathe in saliva, food, liquid, or stomach contents, these conditions are risks factors for
development of lung infections such as pneumonia from impaired ability to clear the
upper airway. Cerebral palsy related to head trauma (head injury, shaken baby
syndrome) or brain infection (bacterial meningitis, encephalitis) effect the ability to
regulate respiration results from damage to the brain stem (respiratory center).
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AN OVERVIEW
•
Central Sleep Apnea (CSA) results in a lack of effort to breath. Characterized by
cheyne-stokes breathing, central apnea can cause a restrictive disorder in the lungs. The
cause of central apnea occurs in the respiratory center of the brain involving problems in
how the brain controls respiration. CSA may be caused by cervical spine injury. CSA
may cause difficulty swallowing, numbness and weakness throughout the body. Poor
control of respiratory muscles may translate to a restrictive lung component. Muscle
weakness affects the ability to expand the lungs. Consideration for central sleep apnea
should be examined when doing a patient history and assessment.
Mechanical Disorders
•
Ascending Lateral Sclerosis (ALS) also known as “Lou Gehrig's” disease is a
progressive, usually fatal, neurodegenerative disease caused by the degeneration of motor
neurons, the nerve cells in the central nervous system that control voluntary muscle
movement. The disorder causes muscle weakness and atrophy throughout the body as
both the upper and lower motor neurons degenerate, ceasing to send messages to
muscles. Unable to function, the muscles gradually weaken. Progression of atrophy and
weakness reaches the diaphragm, which will impair the diaphragm and the ability to
expand the lungs. The onset of symptoms usually begins with weakness in the limbs,
(arms, legs, hands, and feet) with tingling, twitching, stiffness, and cramping soon
following. The progression of the muscle atrophy varies depending on which, and
location of damaged motor neurons. The restrictive respiratory component becomes
more apparent as degeneration occurs. As the diaphragm and intercostal muscles (rib
cage) weaken, forced vital capacity and inspiratory pressure diminish. In bulbar onset
ALS, this may occur before significant limb weakness is apparent. Non-invasive
ventilation like Bi-level positive pressure ventilation is frequently used to support
breathing, first at night, and later during the daytime as well. It is recommended that long
before BiPAP becomes insufficient, patients must decide whether to have tracheotomy
and long term mechanical ventilation. Most patients do not elect this route, and instead
choose palliative hospice care at this point. Most people with ALS die of respiratory
failure or pneumonia, not the disease itself.
•
Myasthenia Gravis (MG) is a muscular disorder that causes serious muscle weakness is
a neuromuscular disease leading to fluctuating muscle weakness and fatiguability. It is
an autoimmune disorder, in which weakness is caused by circulating antibodies that
block acetylcholine receptors at the post-synaptic neuromuscular junction, inhibiting the
stimulative effect of the neurotransmitter acetylcholine. Myasthenia is treated medically
with cholinesterase inhibitors or immunosuppressant’s, and in selected cases,
thymectomy. At 200-400 cases per million it is one of the less common autoimmune
disorders. The disease begins at the top of the body around the head area and progresses
downward to the feet. Medical considerations must be addressed with pending muscle
weakness to the diaphragm. Impairment of the major muscles of ventilation can lead
aspiration, dysphasia, higher risk for pneumonia and respiratory failure. MG is a rare
autoimmune disease, in the past, untreated MG carried a mortality rate of 30 - 70%. In
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AN OVERVIEW
the modern era, patients with MG have a near-normal life expectancy. Prognosis is good
with early diagnosis, intervention, and supportive treatment.
•
Guillian Barre is an acute, autoimmune, polyradiculoneuropathy affecting the peripheral
nervous system, usually triggered by an acute infectious process. Acute inflammatory
demyelinating polyneuropathy (AIDP), which is frequently severe and usually exhibits as
an ascending paralysis (ascends from feet to head) noted by weakness in the feet that
spreads to the upper body and arms and the face along with complete loss of deep tendon
reflexes. Many patients may require hospitalization for medical care, and management of
ventilatory complications that accompany generalized diaphragmatic weakness. With
prompt treatment of plasmapheresis followed by immunoglobulin and supportive care,
the majority of patients will regain full functional capacity. However, death may occur if
severe pulmonary complications and dysautonomia are present.
•
Muscular Dystrophy (MD) is progressive muscular degenerative disease that causes
may symptoms from muscle weakness and wasting. The most common is Duchene’s
(DMD). The onset is generally in child hood. Other dystrophies can be diagnosed
anytime with symptom onset. These conditions are inherited, and the different muscular
dystrophies follow various inheritance patterns. Onset of symptoms can affect the
patient’s ability to: walk; balance; limited ranges of motion; notably respiratory
difficulties and in some dystrophies affects on the heart from cardiomyopathy or
arrhythmias. The diagnosis of muscular dystrophy is based on the results of a muscle
biopsy. In some cases, a DNA blood test may be all that is needed. A physical
examination and the patient's medical history will help the doctor determine the type of
muscular dystrophy. Specific muscle groups are affected by different types of muscular
dystrophy. The MD’s that effect the main muscles of respiration are of special concern.
The diaphragm, inter-costal, and other muscles of ventilation with atrophy may cause
inability for the patient to breathe adequately and ventilate the lungs. The severity of the
atrophy will warrant treatment’s ranging from bi-level non-invasive ventilation, noninvasive mask ventilation via ventilator or invasive ventilation via tracheotomy.
•
Scoliosis / Kyphosis / Kyphoscoliosis. Scoliosis is a medical condition in which a
person's spine is curved from side to side, and may also be rotated. It is an abnormal
lateral curvature of the spine. On an x-ray, the spine of an individual with a typical
scoliosis may look more like an "S" or a "C" than a straight line. Kyphosis is a “hump”
of the upper spine. In general terms, it is a curvature of the upper spine. It can be either
the result of bad posture or a structural anomaly in the spine. In the sense of a deformity,
it is the pathological curving of the spine, where parts of the spinal column lose some or
all of their lordotic profile. This causes a bowing of the back, seen as a slouching
posture. Symptoms of Kyphosis, that may be present or not, depending on the type and
extent of the deformity, include mild back pain, fatigue, appearance of round back and
breathing difficulties Severe cases can cause great discomfort and even lead to death.
Kyphoscoilosis is term used to describe a condition that combines both types of spine
20
AN OVERVIEW
curvature; spine curvatures present distortion to the thoracic and constriction of surface
area for lung expansion.
PHYSICAL MANIFESTATIONS
Intrinsic Disorders
T
he physical examination in patients with intrinsic lung disorders may yield distinguishing
physical findings. Those with chest wall disorders show obvious massive obesity and an
abnormal configuration of the thoracic cage (kyphoscoliosis, ankylosing spondylitis).
Velcro crackles are common in most patients with interstitial lung disorders. Inspiratory squeaks
or scattered, late, inspiratory high-pitched rhonchi are frequently heard in patients with
bronchiolitis. Cyanosis at rest is uncommon in persons with interstitial lung diseases, and this is
usually a late manifestation of advanced disease. Digital clubbing is common in those with IPF
and is rare in others (eg, those with sarcoidosis or hypersensitivity pneumonitis). Extra
pulmonary findings, including erythema nodosum, suggest sarcoidosis. A maculopapular rash
can occur in those with connective tissue diseases, or it may be drug-induced. Cor pulmonale
occurs in the late stages of pulmonary fibrosis or advanced kyphoscoliosis. Pulmonary
hypertension and cor pulmonale become evident when signs include a loud P2, right-sided
precordial lift, and right-sided gallop.
Extrinsic Disorders
Nonmuscular diseases of the chest wall, in which kyphosis/scoliosis can be idiopathic or
secondary, may cause restrictive lung disease. The most common cause of secondary
kyphoscoliosis is neuromuscular disease (eg, polio, muscular dystrophy). Fibrothorax, massive
pleural effusion, morbid obesity, ankylosing spondylitis, and thoracoplasty are other causes.
Neuromuscular diseases manifest as respiratory muscle weakness and are due to myopathy or
myositis, quadriplegia, or phrenic neuropathy from infectious or metabolic causes.
CLINICAL WORK UP
Lab Studies
Intrinsic Lung Diseases
R
outine laboratory evaluations often fail to reveal positive findings. However, anemia can
indicate vasculitis, polycythemia can indicate hypoxemia in advanced disease, and
leukocytosis can suggest acute hypersensitivity pneumonitis.
The decision to perform additional tests should be directed by the findings of the clinical
assessment. Antinuclear antibodies and rheumatoid factor should be measured to screen for
collagen vascular disorders, creatine kinase for polymyositis, antineutrophilic cytoplasmic
21
AN OVERVIEW
antibodies for vasculitis, and antiglomerular basement membrane antibody for Good Pasture
Syndrome.
The presence of precipitating antibodies to an antigen may help in diagnosing hypersensitivity
pneumonitis. Serum angiotensin-converting enzyme levels are often elevated in patients with
sarcoidosis, but this finding has poor specificity.
Extrinsic Disorders
An elevated creatine kinase level may indicate myositis, which may cause muscle weakness and
restrictive lung disease.
Imaging Studies
(X-ray) Chest Radiography For Intrinsic Lung Disorders
•
The diagnosis of an interstitial lung disorder is often initially based on abnormal chest
radiograph findings, which can be normal in as many as 10% of patients. All previous
chest films should be reviewed.
•
The most common radiographic abnormality is a reticular pattern. Nodular,
reticulonodular, or mixed patterns, such as alveolar filling (i.e., ground-glass appearance),
and increased interstitial markings are not unusual, however these are not predictive of a
specific pathological picture.
•
Air-space opacities suggest pulmonary hemorrhage, eosinophilic pneumonia, and BOOP.
•
Upper-zone predominance on chest radiographs is observed in patients with sarcoidosis,
histiocytosis X, chronic hypersensitivity pneumonitis, pneumoconiosis, or ankylosing
spondylitis. Lower-zone predominance is seen in patients with IPF, asbestosis, or
collagen vascular diseases.
•
The finding of honeycombing correlates with advanced fibrosis and indicates a poor
prognosis. Bilateral hilar lymphadenopathy, with or without mediastinal adenopathy,
suggests sarcoidosis.
(CT Scan) Computed Tomography of the Chest
•
High-resolution computed tomography of the chest can be helpful, but the accuracy of the
findings for helping determine a specific etiology is inconsistent. Bibasilar peripheral
lung zone involvement is seen in patients with IPF, asbestosis, connective tissue disease,
or eosinophilic pneumonia.
22
AN OVERVIEW
•
Central disease along bronchovascular bundles is indicative of sarcoidosis or
lymphangitic carcinoma.
•
Upper-zone predominance is observed in patients with sarcoidosis, eosinophilic
granuloma, or chronic hypersensitivity pneumonitis. Lower-zone predominance is seen
in patients with IPF, asbestosis, or rheumatoid arthritis.
•
Lower-zone and peripheral infiltration is ordinarily seen in patients with IPF or
asbestosis.
•
The presence of bilateral cysts and nodules, with preservation of lung volumes, may
suggest a diagnosis of LAM or histiocytosis X.
•
Bibasilar reticular fibrosis with coexisting retraction bronchiectasis indicates end-stage
irreversible disease, and ground-glass. Attenuation may indicate the presence of an
active inflammatory process with potential to respond to medical therapy.
Tests for Extrinsic Disorders
•
Evidence of nonmuscular diseases of the chest wall and associated deformities of the
spinal column and ribs are readily appreciated on chest radiographs. The severity of
kyphoscoliosis is determined by the Cobb angle, which, when greater than 100°, indicates
severe deformity. Neuromuscular diseases are also diagnosed based on chest radiograph
findings showing low volumes and basal atelectasis.
•
Fluoroscopy is used to assess for diaphragm paralysis.
•
A positive result from a sniff test may demonstrate paradoxical upward movement of the
affected diaphragm.
(PFT) Pulmonary Function Tests
•
Used to diagnose restrictive diseases and degrees of severity. Restrictive disorders tend
to have decreased lung volumes. When tested the TLC-total lung capacity, FEV1-forced
expiratory volume at once second, FVC- forced vital capacity, and FRC-functional
residual capacity have lower volume from direct chest wall constriction from restrictive
disorders and diseases. (Please note that full information on pulmonary function testing
is not covered in this course, however RC Educational Consulting Services, Inc. does
have an entire course on the subject).
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AN OVERVIEW
Procedures
•
Bronchoscopy is a procedure that allows your doctor to look at your airway through a
thin viewing instrument called a bronchoscope. During a bronchoscopy, your doctor will
examine your throat, larynx, trachea, and lower airways. Bronchoscopy may be done to
diagnose problems with the airway or to treat problems such as an object or growth in the
airway. This procedure can be used to Identify the cause of airway problems, such as
bleeding, difficulty breathing, or a long-term (chronic) cough; take tissue samples when
other tests, such as a chest X-ray or CT scan, show problems with the lung; diagnose lung
diseases by collecting tissue or mucus (sputum) samples for examination; diagnose and
determine the extent of lung cancer; remove objects blocking the airway; evaluate and
treat growths in the airway; control bleeding; treat cancer in the airway using radioactive
materials (brachytherapy).
•
Bronchoalveolar lavage (BAL) is a medical procedure in which a bronchoscope is
passed through the mouth or nose into the lungs and fluid is squirted into a small part of
the lung and then recollected for examination. BAL is typically performed to diagnose
lung disease. In particular, BAL is commonly used to diagnose infections in people with
immune system problems, pneumonia in people on ventilators, some types of lung
cancer, and scarring of the lung (interstitial lung disease).
•
Lung biopsy may be used in the diagnosis of a lung disease. Cells or tissues from the
lungs are collected and examined under a microscope. This procedure is helpful to
identify strains of infections that affect the lungs, examine damaged lung tissue, and
diagnosis lung cancer.
•
Surgical lung biopsy is similar to lung biopsy, rather a portion of the lung is surgically
removed to examine and diagnosis lung disease, and provide further insight to the origin
and pathology of the disorder.
•
Thoracentesis procedure, a needle is pushed through the chest wall in order to drain fluid
from the chest. The patient will be given medicine to numb the area. Removing this
fluid will allow the patient to breathe more easily.
HISTOGICAL FINDINGS
T
he histological findings of various interstitial pneumonias include an interstitial cellular
infiltrate and interstitial fibrosis, eventually leading to an end-stage honeycomb lung.
24
AN OVERVIEW
MEDICAL CARE AND TREATMENT
Intrinsic Disorders
•
Idiopathic pulmonary fibrosis advancement of disease is variable. Response to
treatments differ from patient to patient with good response to treatment at best is in the
early stages of the disease process. Ending treatment will need consideration when no
response has been noted or if adverse reactions occur.
•
Supplemental oxygen is used to support the patient with pulmonary disease. Oxygen is
used as a drug to treat low oxygen levels below 90%. O2 helps to reduce the work of
breathing, and decrease cardiac work load. Oxygen can be administered at flow rates
between 0.1 lpm to 15 lpm, depending on patient need and stage of pulmonary disease.
Interfaces for delivery of supplemental oxygen may include nasal cannula, simple mask,
aerosol mask, venti-mask, non-rebreather mask. (Please note further information on
supplemental oxygen is not covered in this course).
•
Conventional therapies provide only marginal benefit for patients with interstitial lung
disease.
•
Corticosteroids are among the first round of treatments for pulmonary disease. Steroids
provide ongoing management of bronchial response to airway inflammation and
constriction by providing relaxation of the smooth muscle in the airway. Alternate
therapies may be instituted if steroids side effects occur.
•
Lung transplant is a surgical procedure that replaces a diseased lung with a healthy lung
from a donor. Donor lungs come from the National Donor Registry. Lung transplants
are necessary for improved quality of life and sustaining health. This procedure is used
to treat those with advance stage pulmonary fibrosis, chronic pulmonary hypertension.
•
Cytotoxic therapy immunosuppressive cytotoxic agents may be considered for patients
who do not respond to steroids, experience adverse effects, or have contraindications to
high-dose corticosteroid; therapy will reduce inflammation by inhibiting key steps of the
immune system. Azathioprine and Cyclophosphamide are specific cytoxic agents used in
treatment therapy.
•
Antifibrotic therapy helps to slow progression of fibrotic tissue damage from advancing
interstitial lung disease. These therapies, including colchicine, are suggested for a variety
of fibrotic disorders, including IPF.
25
AN OVERVIEW
Extrinsic Disorders
•
Treatment of disorder to minimize impairment may consist of treatment of the
underlying cause of the chest wall disorder. Many chest wall dysfunctions are
degenerating and progressive in nature so many treatments for minimizing speed of
advancement. Deformation of the chest wall and spinal cord that cause physical
restriction such as scoliosis are treated with surgical interventions, and braces. The goal
of treating the underlying cause of pulmonary restriction may deter complications that
may come with chest wall restrictions: pneumonia and other lung infections.
•
Non-invasive positive pressure ventilation (NPPV). NPPV decreases the work of
breathing and thereby improves alveolar ventilation while simultaneously resting the
respiratory musculature. The improvement in gas exchange with BI-level therapy occurs
because of an increase in alveolar ventilation. Delivery of NPPV is through a Bi-Level
therapy is available to treat restrictive lung disorders from chest wall lung disorders, and
neuromuscular diseases. Bi-level therapy is able to mimic the patient’s own natural
breathing. This therapy can be used to treat disorders of the chest wall and restrictive
neuromuscular diseases. The patient can benefit from this therapy as the bi-level pressure
adds EPAP and IPAP to hyper-inflate the lungs and splint the upper airway to prevent
airway collapse. This type of therapy may be started early in diagnosis. NIPPV may de
delivered via many types of mask interfaces. The selection of the interface is dependant
on the patient preference and breathing style and pattern, however the most common
being either nasal (pillows, prong, mask) or nasal (full face, pillows with covered mouth).
Other types available include delivery to whole face (moon face) or directly to mouth.
•
Mechanical ventilation via artificial airway is used to treat advanced restrictive lung
disease. A ventilator is used to treat restrictive lung disorders, as most are not curable or
reversible, or progressive degeneration to lung tissue. The purpose of mechanical
ventilation is to reduce the work of breathing, reduced cardiac work load, provide patient
comfort, and ventilate the lungs adequately. There two three types of ventilation;
positive pressure and negative pressure (via Iron lung). Positive pressure ventilation is
the choice most common. The iron lung is used to treat polio patient’s, but in not
common in use today. Positive pressure ventilation may be delivered with a set (VT)
tidal volume or (PC) controlled pressure, selection is made by medical doctor. Artificial
ventilation is designed to replace the body’s natural respiratory cycle, or may simply
assist the patient’s own breathing ability. (Note: further information about mechanical
ventilation is not covered in this course). Using mechanical ventilation to treat restrictive
lung diseases aids in patient ease of breathing, maintain extended life expectation in those
with degenerating lung diseases, and slow advancement to respiratory muscle
impairment.
•
Oral /Nasal suctioning is a therapy selection that is started to counter the advancement of
respiratory muscle weakness. In disorders such as ALS or MD the progression of muscle
26
AN OVERVIEW
degeneration will begin to affect the body’s ability to cough effectively and expel irritants
to the upper airway from lower airway to be cough out. Suction machines generate
negative pressure to form a vacuum and allow secretions and other substances to be
removed from the airway. Oral/nasal suctioning is done via a yaunker or other external
interfaces. Endotracheal suction is done via artificial airway. Removal of the secretions
is necessary to keep the airway clear, thus avoiding further complications such as
pneumonia.
•
Mechanical Insufflator-Exsufflator (cough-assist). Cough Assist is a noninvasive
therapy that safely and consistently removes secretions in patients with an ineffective
ability to cough (peak cough flow <270 l/m). Typical Cough Assist patients include
those with the following conditions:
Amyotrophic lateral sclerosis; Spinal muscular atrophy; Muscular dystrophy, Myasthenia
gravis, spinal cord injuries.
Cough Assist clears secretions by gradually applying a positive pressure to the airway,
then rapidly shifting to negative pressure. The rapid shift in pressure produces a high
expiratory flow, simulating a natural cough. This therapy alternates between negative
and positive pressure, the combination allows for hyper-inflation and a vacuum. Benefits
of Cough Assist include: removes secretions from the lungs; reduces the occurrence of
respiratory infections; safe, noninvasive alternative to suctioning; easy for patients and
caregivers to operate and flexibility for use in home, hospital, or on the go.
SURGICAL PROCEDURES
S
urgical treatment is often an option to treat whole diseased lungs or a portion of a diseased
lung. A selection criterion is reviewed with Medical Physician for best procedure selection
based on best projected outcome and necessity.
•
Pneumonectomy: one entire lung is removed
•
Lobectomy: a section (lobe) of the lung is removed
•
Segmentectomy/wedge resection: part of a lobe is removed
•
Laser surgery: a high-energy beam of light destroys the cancer cells in a tumor
Goals of Treatment
Ultimate goals of treatment of restrictive lung diseases, is an overall improved quality of life,
maintenance of respiratory base line, management of pulmonary disease, and, in some cases,
sustainment of longer life expectancy. To improve quality of life compliance with treatment
27
AN OVERVIEW
schedule is necessary, along with ongoing medical support to address new symptoms or concerns
that may arise during course of treatment. Keeping the patient healthy and out of the hospital
with recurrent lung infections is critical with therapy goals. Taking steps to insure that the
pulmonary baseline remains intact will help keep the patient at home longer and give them fewer
days in the hospital. Management of pulmonary disease includes, if possible, early diagnosis,
intervention, treatment, and ongoing management. Sustaining life expectancy in those patients
with progressive lung diseases that choose advanced respiratory interventions are looking to
extend life as long as possible at home with family and friends. Generally the choice to continue
therapy is dependant on notable response to treatment. If response to treatment is marginal or
non-existent, changes in the treatment plan would be necessary.
Consultation Considerations
Consultation with medical physicians is needed for diagnosis, education, support, therapy, and
management. Consultation could include:
•
Pulmonologist is a lung specialist who will help to education, support and manage
ongoing lung disorders.
•
Primary care physician may be the first care provider to suspect a lung disorder. Good
communication about symptoms will help to make an initial diagnosis, then a referral to
specialist.
•
Hospice Care could come at the end stage of a progressive lung disease. Hospice care is
designed to provide supportive, palliative, and comfort measures in a dignified setting as
the patient succumbs to his/her disease.
Deterrence / Prevention
Although many restrictive lung diseases are not predictable or preventable, avoiding exposure to
environmental and occupational irritants will help reduce risk factors for development of
pulmonary disease. Careful management of one’s personal health and staying away from illegal
drugs, along with getting vaccines for diseases such as influenza and pneumonia may lower risk
factors for disease development. Avoidance of risky behavior that could yield a lower,
weakened immune system could go a long way to prevent lung disease. In the case of
unavoidable disease like MD or ALS the best way to deter advancement of the disease is patient
compliance with prescribed treatment regimen.
Complications
Treating restrictive diseases can present many complications. Complications rise from the
advancing nature of some disorders such as ALS, MD, and pulmonary fibrosis. Once an illness
is diagnosed degeneration continues to advance the respiratory impairment. Treatment for these
types of diseases then becomes an ongoing battle. The needs of the patient tend to change
28
AN OVERVIEW
rapidly. The ability to ventilate, oxygenate, clear the airway and use of respiratory muscles may
become progressively difficult. Treatment and medical care must be adaptable to meet the
patient ever changing respiratory needs. Keeping this in mind when treating those patients with
advancing lung restriction will help the practitioner be better prepared to adjust medical care as
needed.
Prognosis
•
The natural history of interstitial lung diseases is variable. It depends on the specific
diagnosis and the extent and severity of lung involvement. Idiopathic Pulmonary
Fibrosis (IPF) is typically a relentless progressive disorder, and patients have a mean
survival of 4 - 6 years after diagnosis.
•
Pulmonary sarcoidosis has a relatively benign self-limiting course, with spontaneous
recovery or stabilization in most cases. Approximately 15% of patients develop
pulmonary fibrosis and disability.
•
Prognosis for collagen vascular diseases, eosinophilic pneumonia, Bronchiolitis
Obliterans with Organizing Pneumonia (BOOP), and drug-induced lung disease is
generally favorable with treatment.
•
Patients with chest wall diseases and neuromuscular disorders develop progressive
respiratory failure and succumb during an intercurrent pulmonary infection.
Medical / Legal Pitfalls
Since many restrictive pulmonary diseases advance and progress to the point of death education
for patient advance care planning and advance directive is helpful to provide the patient with the
care they wish to have or to remove care at the point all possibilities have be exacerbated.
Special Concerns
Some special consideration to address is there is no treatment model that will work for every
patient that has pulmonary disease the same. Exacerbation of IPF begins with increased WOB
and dyspnea, the progression will vary. Making changes to treatment schedule will be ongoing.
Meeting the present needs for the patient for the present is the first priority. Providing comfort
measures for advancing disease will come as the to ensure that airway care is provided according
to best practice.
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AN OVERVIEW
CLINICAL SCENARIO’S
Clinical Scenario #1
You are assigned to the E.R. unit of a large 700 bed University teaching hospital. You have just
received your current patient shift report. You have orders to perform a blood gas on a 16 year
old asthma patient, and give a breathing treatment to an older patient with shortness of breath.
After you perform the blood gas on the 16 year old you enter the room with the older gentleman.
Paulo is a 62 year old Russian immigrant who speaks very limited English. He is with his 15
year old granddaughter. You make your initial patient assessment and observe, the patient
leaning forward with shallow labored breathing, he is using his accessory muscles, is short of
breathe, but has no notable wheezing. What would you check during this initial assessment?
You begin your assessment by checking the patient’s respiratory rate, oxygen saturation, breath
sounds, blood pressure, heart rate and temperature. You had been ordered to administer 0.5 ml
of nebulizer albuterol breathing treatment. Observed manifestations: respiratory rate 32, blood
pressure 144/96, heart rate 99, breath sounds-inspiratory crackles, O2 Sats 88% and a temp of
102 degrees. The patient also has a productive cough which reveals there is blood in the sputum.
Note: There is no available patient history available for this patient. Is 0.5 ml albuterol via small
volume nebulizer the best course of treatment for this patient? What other initial treatment
would you recommend?
You have determined that supplemental oxygen is necessary to treat the patient low saturation
level. You then proceed to administer the breathing treatment for this patient. You place the
patient on 2 lpm O2 via nasal cannula after he finishes the breathing treatment. You notice the
patient has distal clubbing of the fingers. What diagnosis would you suspect to this point? What
other clinical assessment tools would you employ?
You have assessed this case warrants further investigation. Since the patient does not speak
English you inquire to get medical history from the granddaughter. What type of medical history
questions would you ask the granddaughter? What other clinical testing would you recommend
to further asses this patient? You reveal to the doctor on duty the clinical findings you observed.
Along with shortness of breath, you noticed that patient grimaces with pain when breathing in,
distal clubbing in the fingers. You recommend the patient should have a chest x-ray. What
justification would warrant a chest film? The chest x-ray reveals: A diffuse fibrotic pattern in
the lower lobes.
You proceed to ask the granddaughter about the patient’s medical history. What questions
would you ask? Does the patient have a history of smoking? She says “no”. Does he have a
history of other breathing problems? She says “only when he goes up and down the stairs or
walking fast, he starts to breathe fast and gets really tired.” How long has he had trouble
breathing fast and hard? She says “about 2 years.” How long had he had chest pain when
breathing? She says “about the same time.” What other medical problems has he had? She says
“he did have pneumonia a couple of months ago.” Has he traveled to another county? She says,
30
AN OVERVIEW
“He was born and raised in Russia, he came to the US only three years ago.” Does he have a
history of any exposure to dust particles, hazardous materials, or chemicals? She says “Maybe,
he used to work in construction back in Russia, and worked in the coal mines back in Russia.”
How long has it been since he worked in construction and the mines? She says, “He worked in
the mines until three years ago and worked in construction about 10 years ago. At this point do
you have enough patient history of symptoms to make a determination of this patient’s diagnosis
and make further clinical recommendations for testing and treatment? Diagnosis and treatment
is reveled in the discussion section.
Clinical Scenario #2
You are on call for the ER on a cold winter night. You have finished your last rounds and just
sat down to eat your dinner when you are paged to the ER. Oliver is a 27 year old homeless man
that has presented to the ER department. He has waited for than three hours to be seen. You
have been called to the ER to assess him and give your recommendations to the Physician on
duty. His initial complaint is increased shortness of breathe with activity, difficulty breathing
when he is trying to sleep, coughing, a low grade temperature, fatigue, increased muscle
weakness, headaches, and nausea. This man is a frequent visitor to the ER. He seems to come in
more frequently in recent weeks. What should you assess with this patient? Can you make any
treatment recommendations for this man?
You are very familiar with this patient. He has a very recent history presenting with the same
symptoms. However you look through his chart and see a strange pattern of the same
reoccurring symptoms. He has been in the admitted three times in the last three months for
recurrent lung infections, fatigue, increased muscle weakness, difficulty swallowing, and muscle
spasms in his feet and ankles. Today he complains of an inability to walk, inability to lift or pick
up things, decreased mobility, and feels like he is being choked by the secretions in his throat
and chest. What is wrong with this patient? Can you make an assessment and diagnosis
recommendations at this point?
Further investigation of the patient chart reveals Oliver has a history of hypertension and cor
pulmonale. After you assess the patient your assessment reveals: respiratory rate 30, blood
pressure 151/104, temp 101.1, breath sounds-diminished bilaterally, heart rate 112, SPO2 87%.
Chest film reveals: low volume basal atelectasis. What blood tests would you recommend to
gather additional clinical information?
Note: this patient does have a history of excess alcohol consumption, cigarette smoking, and
tobacco use. How would you treat this patient? What testing would you perform to further
assess this patient? What clinical interventions would you recommend?
31
AN OVERVIEW
Clinical Scenario Discussions
Case #1
In this case scenario the history of occupational exposure should key off the diagnosis. Since the
latency period of ASBESTOSIS can be years later. It is important to do a full patient history to
include or rule out possible risk factors for occupational lung disease. The most important
treatment for this type of lung disease is to eliminate any possible exposure. However since
many immigrant workers had exposure in a country with little to no occupational safety
standards. Exposure to asbestos causes irreversible lung damage, from breathing in the small
particles of asbestos fibers. These fibers damage the fragile lung tissue forming scar tissue, with
has no ability to diffuse gas. Damaged lung tissue decreases the available surface area to take in
oxygen and expel carbon dioxide. The course of treatment is to address presenting symptoms,
and improve the overall quality of life for the patient, since there is no cure for this type of lung
damage.
Diagnosis may involve any, or all of the following:
1. Pulmonary function testing
2. Chest x-ray
3. Arterial blood gases
4. Pulse oximetery
5. CT- Scan
6. Bronchoscopy
7. Assessing breath sounds
8. Assessing vital signs
After initial diagnosis is made as a respiratory therapist you may have opportunity to make
recommendation on the best course of treatments. You could recommend any or all of the
following:
1. Removal from exposure hazards
2. Supplemental oxygen to treat hypoxia
3. Radiation or chemotherapy for Mesothelioma
32
AN OVERVIEW
4. Surgical removal of any cancerous tissue
5. Pulmonary rehab
6. Aerosol medications to thin secretions
7. Lung transplant in severe cases
Case # 2
The patient in this case scenario presents to the ER with recurrent and worsening symptoms.
This diagnosis of the disease would come from rule out of other neuromuscular diseases. This
disorder can mimic other neuromuscular diseases. However the onset and advancement of this
disease is indicative Amyotrophic Lateral Sclerosis (ALS). The progression of this disease
begins to affect the respiratory system with impairment of the diaphragm and other muscle used
in respiration. ALS is difficult to diagnosis, especially with your homeless patient. There is no
cure for ALS, and advancement may require intubation or tracheal airway with mechanical
ventilation to keep the patient alive. These patients’ often have recurrent lung infections from
the inability to cough and clear secretions. Keep the symptoms in mind when making
recommendations for treatment of the patient. After initial diagnosis the goals of treatment shifts
to patient comfort and prevention of recurrent lung infections. There is no cure for ALS, and
ultimately a decision would need to be made whether to use mechanical ventilation or not. Once
the diaphragm is affected by muscle atrophy, the patient would soon have no ability to ventilate
and oxygen. Respiratory distress and respiratory failure are inevitable. ALS is a terminal
disease with a poor prognosis.
Diagnosis of ALS may require recommendation of any or all of the following:
1. Neurological exam
2. Chest X-Ray
3. Nerve conduction velocity (NCV)
4. Electromyography (EMG)
5. MRI-Scan to rule out spinal cord disease
6. Laboratory tests to check protein and hormone levels
7. Cerebral and Blood tests to rule out genetic, autoimmune, neurotoxin, viral disorders
33
AN OVERVIEW
Treatment focuses on relieving symptoms and maintaining an optimal quality of life.
1.
Medications to prevent progression and prolong life such as Riluzole (Rilutek®)
2.
Medications that treat muscle spasms
3.
Nonsteroidal anti-inflammatory drugs to treat pain
4.
Physical, occupational, and speech therapy to treat on-going change to muscle ability
5.
Bi-Level pressure therapy to treat increase SOB, and diaphragmatic impairment
6.
Long-term mechanical ventilation, if the patient chooses
7.
8.
Cough-assisting therapy devices to assist with in-effective cough
Oral /airway suctioning to remove excess secretions
9.
Feeding tube (GI) or (PEG)
SUMMARY
I
n conclusion to the course, it becomes important to expand the areas of thinking when
assessing and treating patient’s with restrictive lung diseases. Understanding restrictive
means thinking outside the respiratory therapy box as there are over 100 known restrictive
lung diseases. Initial training teaching identification, treatment, and medical care of those with
COPD diseases, however many restrictive lung diseases may show only a few or none of the
typical pulmonary disease signs and symptoms. Do research and ask questions to find the
clinical clues that could yield a quicker diagnosis of a restrictive respiratory disease. The key to
advancing the therapist knowledge base is exposure to a wide variety of clinical diseases
presentations. Get additional information from specific radiologic findings, histological testing,
and other measures that are indicative of restrictive diseases. Additional reading is suggested in
specific areas to better understand these specific testing measures.
34
AN OVERVIEW
SUGGESTED READING AND REFERENCES
Sharma, Sat MD, FRCPC; Restrictive Lung Disease, Professor and Head, Division of Pulmonary
Medicine, Department of Internal Medicine, University of Manitoba; Site Director, Respiratory
Medicine, St. Boniface General Hospital, June 2006.
British Lung Foundation, Facts about Respiratory Disease, www.lunguk.org April, 2008.
Canada Lung Association, Fact Sheet for Lung Cancer, http://lung.ca/diseases-maladies/cancercancer/treatment, April, 2008.
Med Central Health System, Medical Services, Restrictive Lung Disorders.
http://www.medcentral.org, April, 2008.
Bone Marrow Transplantation, Post transplantation complications, respiratory cillary function.
June 2001, www.nature.com April, 2009.
ERS Journals Limited, Drug-induced infiltrative lung disease, Ph. Camus, P. Foucher, Ph.
Bonniaud and K. Ask. March 2001. http://erj.ersjournals.com, April, 2008
Drug Induced Lung Disease, CCJM Journals, www.ccmj.org, April, 2008
American Lung Association , Lung Disease fact sheet, Occupational Lung diseases,
www.lungusa.com, April, 2008
Office of Environmental Health and Safety, Division of Environmental Health, Washington State
department of health, http://www.doh.wa.gov/ehp/TS/IAQ/MoldStachybotrys, April, 2008
Journal of Occupational and Environmental Medicine, Building associated Pulmonary Disease,
www.joem.org, May, 2008
British Lung Foundation, conditions and disease, Symptoms of extrinsic diseases,
http://www.lunguk.org/you-and-your-lungs/conditions-and-diseases, May, 2008
Docstoc, Presentation of Restrictive Disorders,
http://www.docstoc.com/docs/521384/Presentation-on-RESTRICTIVE-LUNG-DISEASE, May,
2008
American lung Association, Lung fact sheet, interstitial Lung Disease, www.lungusa.com, May,
2008
Pulmonary Fibrosis Foundation, Pulmonary fibrosis fact sheet,
http://www.pulmonaryfibrosis.org, May, 2008.
35
AN OVERVIEW
Merck, Merck Online manuals, Acute Lung Injury and Acute Respiratory Distress Syndrome,
http://www.merck.com, May, 2008
Breathing Better Living Well, Community Index, Pleurisy, www.breathingbetterlivingwell.com,
April, 2008
Lung Diseases, Pleura and Pleurisy, About.com, www.lungdiseases.about.com, May, 2008
Lung Association of Canada, Pleurisy Fact sheet, http://lung.ca/diseases-maladies/a-z/pleurisypleuresie, May, 2008
National Heart Lung and Blood institute, Diseases and conditions index, what is Sarcoidosis?
http://www.nhlbi.nih.gov/health/dci/Diseases/sarc, May, 2008
National Cancer Partnership, Lung Cancer facts, http://www.nationallungcancerpartnership.org,
May, 2008
Merck Manual Professional Edition, Lung Carcinoma: Tumors of the Lungs, online edition,
http://www.merck.com/mmpe, May, 2008
Mayo Clinic Online, Diseases and conditions, Asbestosis
http://www.mayoclinic.com/health/asbestosis, May, 2008
Asbestosis, Occupational Lung Diseases, emedicine by WebMD, Basil Varkey, MD, FCCP,
Professor, Department of Internal Medicine, Division of Pulmonary and Critical Care, Froedtert
Memorial Lutheran Hospital, Medical College of Wisconsin
http://www.emedicine.com/med/topic171.htm, November 2007..
American Lung Association, Pneumonia fact sheet, Diseases A-Z, www.lungusa.com, May,
2008
Mayo Clinic online, infectious Diseases, Pneumonia,
http://www.mayoclinic.com/health/pneumonia, May, 2008
Medicine net, Pneumonia, Signs and symptoms,
http://www.medicinenet.com/pneumonia/article.htm, May, 2008
ARDS Support Center, Understanding ARDS,
http://www.ards.org/learnaboutards/whatisards/brochure, May, 2008
American Family Physician, Peer reviewed journal, Acute Respiratory Distress Syndrome,
http://www.aafp.org/afp, May, 2008.
36
AN OVERVIEW
World Health Organization, Coronavirus never before seen in humans is the cause of SARS,
http://www.who.int/mediacentre, May, 2008
Wikipedia, Infant Respiratory Distress Syndrome, online encyclopedia,
http://en.wikipedia.org/wiki/Infant_respiratory_distress_syndrome, May, 2008
Spinal Cord 101, possible complications,
http://www.spinalinjury.net/html/_complications_of_a_spinal, May, 2008
Spinal Cord Injury Information Network, Understanding and Managing Respiratory
Complications after SCI, Info sheet #19, October 2007, http://www.spinalcord.uab.edu, May,
2008.
Origins of Cerebral Palsy, Associated Conditions of Cerebral Palsy: Breathing Difficulties,
http://www.originsofcerebralpalsy.com/03-conditions, May, 2008.
Associated Conditions of Cerebral Palsy, Online Resource, Respiratory Problems,
http://www.associatedconditionsofcerebralpalsy.com/respiratory.html, May, 2008.
Cerebral Palsy." (National Center on Birth Defects and Developmental Disabilities,
www.cdc.gov, May, 2008.
Medline Plus Medical Encyclopedia: Central Sleep Apnea,
http://www.nlm.nih.gov/medlineplus/ency/article, May, 2008.
The Muscular Dystrophy Association-ALS division, http://www.als-mda.org, May, 2008
The ALS Association, ALS fact sheet, www.alsa.org, May, 2008
eMedicince by WebMD, Myasthenia Gravis by Newton, Edwin MD, March 2007,
http://www.emedicine.com/emerg/TOPIC325.HTM, May, 2008.
GBS/CIDP Foundation International, About Guillian Barre Syndrome, http://www.gbs-cidp.org,
May, 2008
Muscular Dystrophy, Online information library, http://dystrophy.com retrieved May 31, 2008.
Spinal Deformities: Benefits of Early Screening and Treatment, by John Albert Odom, Jr., M.D.
http://medicalreporter.health.org, May, 2008
WebMD, Bronchoscopy, A to Z guides
http://www.webmd.com/a-to-z-guides/bronchoscopy, May, 2008
37
AN OVERVIEW
Cough Assist® Mechanical Insufflator-Exsufflator, by Respironics
http://coughassist.respironics.com, May, 2008
Emedicine by WebMD, Pulmonology, Interstitial Lung Disease, Imaging Studies,
http://www.emedicine.com/MED/topic2012.htm, May, 2008
Medicine by WebMD, Pulmonology, Interstitial Lung Disease, Radiographigic Studies,
http://www.emedicine.com/MED/topic2012.htm, May, 2008
Emedicine by WebMD, Pulmonology, Interstitial Lung Disease, Prognosis for pulmonary
disease, http://www.emedicine.com/MED/topic2012.htm, May, 2008
38
AN OVERVIEW
POST TEST
DIRECTIONS: IF COURSE WAS MAILED TO YOU, CIRCLE THE MOST CORRECT
ANSWERS ON THE ANSWER SHEET PROVIDED AND RETURN TO: RCECS, 16781
VAN BUREN BLVD, SUITE B, RIVERSIDE, CA 92504-5798 OR FAX TO: (951) 789-8861.
IF YOU ELECTED ONLINE DELIVERY, COMPLETE THE TEST ONLINE – PLEASE
DO NOT MAIL OR FAX BACK.
1. Which is NOT considered a restrictive pulmonary disease?
a. Lung cancer
b. Pulmonary fibrosis
c. Emphysema
d. Pneumonia
2. Restrictive Lung disorders affect the ability for the lungs to expand.
a. True
b. False
3. Which is NOT a classification of a restrictive lung disease?
a. Disease of the internal lung wall
b. Disease of the lining of the lung
c. Impaired Neuromuscular mechanism
d. Distal alveolar distention
39
AN OVERVIEW
4. Which environmental irritant is a risk factor to develop lung disease?
a. Fiber Glass particle exposure
b. Asbestosis exposure
c. exposure to gases, dust, and fumes
d. All of the above
5. Which is classified as an occupational lung disease?
a. Pneumonia
b. Sarcoidosis
c. Pulmonary Fibrosis
d. Kyphoscoliosis
6. Those patients with neuromuscular disorders often succumb to_____________?
a. Respiratory distress
b. Respiratory failure
c. Lung infections
d. All of the above
7. Initial patient assessment should include all, except?
a. Duration of symptoms
b. History of smoking
c. Sexual orientation
d. Occupation
40
AN OVERVIEW
8. Which is considered a mechanical pulmonary disease?
a. Emphysema
b. ALS
c. Pulmonary fibrosis
d. Pleurisy
9. Cyanosis at rest is common in persons with interstitial lung diseases.
a. True
b. False
10. Neuromuscular diseases manifest as respiratory muscle weakness and are due to_______?
a. Myopathy or Myositis
b. Quadriplegia
c. Phrenic neuropathy from infectious or metabolic causes
d. All of the above
11. PFT's testing can be used to diagnosis restrictive lung diseases.
a. True
b. False
41
AN OVERVIEW
12. The radiographic findings of various interstitial pneumonias include?
a. Interstitial cellular infiltrate
b. Interstitial fibrosis
c. Honeycomb lung
d. All of the above
13. Supplemental Oxygen is used to support the pulmonary patient by all, except?
a. Maintaining adequate ventilation
b. Decreases work of breathing
c. Decreases cardiac workload
d. Treat oxygen levels below 90%
14. Corticosteroids are among the first round of therapies used to treat pulmonary disease.
a. True
b. False
15. Which Patient may be a candidate for a lung transplant?
a. Pneumonia
b. Cerebral Palsy
c. Pulmonary fibrosis
d. Asthma exacerbation
42
AN OVERVIEW
16. Antifibrotic therapy helps to slow progression of fibrotic tissue damage from advancing
interstitial lung disease.
a. True
b. False
17. Non-invasive positive pressure ventilation treatment benefit shows?
a. Decreases the work of breathing
b. Improves alveolar ventilation
c. Resting the respiratory musculature.
d. All of the above
18. What are the goals to treatment?
a. Improved quality of life
b. Maintain respiratory baseline
c. Sustain longer life expectancy
d. All of the above
19. The prognosis and progression of Interstitial Lung disease is predictable.
a. True
b. False
43
AN OVERVIEW
20. Changing patient ventilatory needs from advancing restrictive disorders may vary and
present a complication to consider when treating the patient.
a. True
b. False
AS: Test Version A
44
AN OVERVIEW
ANSWER SHEET
NAME____________________________________ STATE LIC #_______________________
ADDRESS_________________________________ AARC# (if applic.)___________________
DIRECTIONS: (REFER TO THE TEXT IF NECESSARY – PASSING SCORE FOR CE
CREDIT IS 70%). IF COURSE WAS MAILED TO YOU, CIRCLE THE MOST CORRECT
ANSWERS AND RETURN TO:
RCECS, 16781 VAN BUREN BLVD, SUITE B,
RIVERSIDE, CA 92504-5798 OR FAX TO: (951) 789-8861. IF YOU ELECTED ONLINE
DELIVERY, COMPLETE THE TEST ONLINE – PLEASE DO NOT MAIL OR FAX BACK.
1.
a b c d
16. a b
2.
a b
17. a b c d
3.
a b c d
18. a b c d
4.
a b c d
19. a b
5.
a b c d
20. a b
6.
a b c d
7.
a b c d
8.
a b c d
9.
a b
10. a b c d
11. a b
12. a b c d
13. a b c d
14. a b
15. a b c d
AS: Test Version A
45
AN OVERVIEW
EVALUATION FORM
NAME:____________________________________________ DATE:______________
AARC # (if applic.)________________________ STATE LICENSE #:______________
RC Educational Consulting Services, Inc. wishes to provide our clients with the highest quality
CE materials possible. Your honest feedback helps us to continually improve our courses and
meet CE regulations in many states. Please complete this form and return/submit it with your
answer sheet. Thank you.
YES
NO
Were the objectives of the course met?
Was the material clear and understandable?
Was the material well-organized?
Was the material relevant to your job?
Did you learn something new?
Was the material interesting?
Were the illustrations, if any, helpful?
Would you recommend this course to a friend?
What was the most valuable portion of the material?
________________________________________________________________________
What was the least valuable portion of the material?
________________________________________________________________________
Suggestions for future courses: ______________________________________________
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46

RESTRICTIVE PULMONARY DISORDERS: AN OVERVIEW

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