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Transcription

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Please note:
• Opinions and materials presented by the nurses are theirs alone and do not necessarily reflect those of the sponsor.
• The information provided is not intended to be a substitute for professional medical advice. A licensed healthcare professional should be consulted for diagnosis and treatment of any and all medical conditions.
Made possible through an educational grant from GRIFOLS
EDL46-1-US-11
Acknowledgment
The information contained in this flash drive was prepared by:
Becki Berkowitz RN; Hemophilia Treatment Center of Las Vegas
Sue Geraghty RN, MBA; UCD Hemophilia and Thrombosis Center
Judy Kauffman RN, MS, CPNP; Kansas City Regional Hemophilia Center
Jennifer La Franco RN; Mary M. Gooley Hemophilia Center
Susan M. Peterson MSN, ARNP; University of Kentucky HTC
Susan Zappa RN, CPN; Fort Worth Bleeding Disorders Program
Common issues for different age groups
von Willebrand disease (VWD) and your child’s development
Early childhood – 1 to 4 years old
Relationships
Your preschooler learns by doing things. The preschooler’s favorite phrase is “I’ll do it myself!” It may
be difficult, but you have to let your little one try new things. Children continue to be very physical at this
age. Your preschooler will want to run, jump, and climb and will most certainly fall sometimes. He or she
is becoming more independent but still relies on having parents there to provide support, guidance, and
comfort.
Continue to use the daily bath as a time to check out your child - look for
bruising, tender spots, swelling, or anything unusual. Call your hematology
team with concerns.
The world
Your preschooler learns about the world by mastering new activities. At this
age a child will want to try things that may seem risky or dangerous to you.
Help your child try new activities, while making them safer for him or her.
Make your child’s environment as safe as possible! Children should wear appropriate protective gear for
whatever activities they enjoy. A bike helmet while riding a tricycle or big wheel will protect the head
during falls and help the child become accustomed to wearing the helmet. Knee and elbow pads may help
protect the joints during activities.
However, accidents and bleeds may happen. If your child has severe Type 1 or Type 3 VWD you may want
to have an emergency bag ready to go in case you need to seek care for bleeding. Fill the bag with contact
information for the hematology team, emergency care information, your flash drive, snacks, and a few things
that will keep your child entertained while awaiting care. Having a bag ready will allow you to add your
treatment product to the bag and take off quickly if your child is injured.
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DDAVP®/Stimate®
After age two, your child may be tested to discover whether DDAVP is effective in raising circulating factor
levels. If your child is expected to be responsive to it, Stimate may be prescribed for your child. You can help
your child learn to use the nasal spray by practicing with over-the-counter saline nasal spray. Having Stimate
nasal spray for treatment of bleeding episodes can be useful for mild to moderate bleeding episodes. Your
care provider will educate you as to when to use Stimate. Refer to the medication sheet on DDAVP/ Stimate.
Common injuries during this age include:
Bruising:
A child with a bleeding disorder will bruise more easily than other children. He
or she may develop bruises with falls, or from injuries that you know nothing
about. Bruises are unsightly, but should not cause you concern unless they
grow larger and cause your child pain. It may be helpful to draw a line around
a worrisome bruise so that you can tell if it is growing. Ice or cold packs can
be applied to help treat a bruise. If a bruise causes your child to stop using
the affected arm or leg, call your hematology team for advice.
Head bumps:
If your child experiences a “head bump” (injury to the head from falling or hitting a hard surface) he or she may
need treatment and a head CT. Call your hematology team for instructions as soon as possible after the injury.
Muscle and joint bleeds:
Muscle bleeds and joint bleeds are extremely rare in children with VWD. Symptoms of this type of bleed include
severe pain, extreme swelling and inability to use the body part. If you suspect your child has this type of bleed,
call your treatment center.
Mouth care:
Your child should brush his or her teeth at least twice daily. Brushing should be supervised by an adult at this
age to ensure that it is done correctly. By age 2, your child should have a dental checkup. Brushing and regular
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dental visits will help keep your child’s gums healthy and minimize gum bleeding. Sometimes bleeding will
occur when your child loses a baby tooth or with injury. This type of bleeding can often be treated with Amicar®.
Nosebleeds:
Nosebleeds may occur when the air is dry, or during allergy season. Preschoolers may develop a nosepicking
habit that makes bleeds worse. Efforts to prevent nose bleeding include: using a humidifier to moisten the air,
using saline nose spray to keep the inside of the nose from cracking, and encouraging your child not to pick
his or her nose. Persistent nosebleed may require treatment to control. Refer to the section on Nosebleeds.
Home treatment for those with severe VWD:
If your child has severe VWD. If your child has severe VWD, give him or her a “job” to do during bleeding
treatment. Have your child be ready to apply gauze and a bandage at the end of the infusion. Give choices
when possible. Have the child help fill out the infusion record. All of these things will help give your child
mastery over this part of life.
At this age, your child will respond to suggestions that he can be a “big” boy or girl and may not require being
held so tightly for infusions. You may be able to give the child choices about body position during the infusion
(can he or she sit up, and not have to be held down?). Give the child permission to cry, with instructions to
hold as still as possible. Afterwards, praise his or her handling of the infusion so that your child will learn how
to handle infusions. Your love and support continue to help your child through the experience.
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School age - 5 to 10 years old
Relationships
Your school aged child will begin to have relationships outside of the family. A child’s friends become very
important to him or her at this age. School - aged children really enjoy and benefit from being a member
of a club or team. Scouting, youth sports and other “belonging” opportunities will give the child a chance
to be part of a group and will benefit his or her development.
School teachers, school nurses, coaches, and others who spend time with your child will need to learn
about von Willebrand disease (VWD). The hematology staff may be able to supply educational and inservice materials to those involved with your child to ensure that he or she will be well cared for when
away from you.
Teach your child to tell you when he or she is hurt and to be able to describe where the pain is, what
happened, etc. A matter - of fact attitude toward injuries/bleeds will allow the child to feel more comfortable
telling you what has happened.
These years are an especially important time to start teaching your child to become involved in care for
the bleeding disorder. Parents need to start taking time to talk about the bleeding disorder in a language
the child understands and make sure the child can begin to talk about their bleeding disorder. The child
should be able to say the name and important facts about their disorder and even to be able to simply
describe it to the others. Your hematology team will assist in this teaching, but they only see your child
occasionally, so parents/caregivers are the ones who must keep up with repetitive education.
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The world
Your school-aged child learns about the world by trying new things and through relationships with family
and friends. Children with VWD should go to school regularly. School is very important and absences
should be minimal, but if they occur the work should be made up at home to keep the child current. There
are many helpful pamphlets about VWD for your child’s school, which your hematology team can provide
to you.
Ask your VWD care provider about camps for kids with bleeding disorders in your area.
Teaching proper nutrition will assist your child throughout life. Eating a well-balanced diet and getting
enough exercise is key to a healthy life.
Your VWD care providers can help you determine which activities are appropriate for your child. See the
Sports section.
Teach your child to tell you when he or she is hurt and to be able to describe where the pain is, what
happened, etc. A matter of fact attitude toward injuries/bleeds will allow the child to feel more comfortable
telling you what has happened.
Bruising:
You have seen by this age that a child with a bleeding disorder will bruise more easily than other children.
Your child may develop bruises with falls, or from injuries that you know nothing about. Bruises are unsightly,
but should not cause you concern unless they grow larger and cause pain to your child. Ice or cold packs can
be applied to help treat a bruise. If a bruise causes your child to stop using an affected arm or leg, call the
hematology team for advice.
Head bumps:
If your child experiences a “head bump” (from a fall or hitting a hard object), he or she may need treatment
for bleeding and a head CT. Call your hematology team for instructions as soon as possible after the injury.
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Muscle and joint bleeds:
Muscle bleeds and joint bleeds are extremely rare in children with VWD. Symptoms of this type of bleed
include severe pain, extreme swelling and inability to use the body part. If you suspect your child has this type
of bleed, call your treatment center.
Mouth care:
Your child should brush his or her teeth at least twice daily. Brushing should be supervised by an adult at this
age to insure that it is done correctly. Brushing teeth and regular dental visits will help to keep your child’s
gums healthy and minimize gum bleeding. Sometimes bleeding will occur when your child loses a baby tooth
or with injury. This type of bleeding can often be treated with Amicar® or simply by holding pressure to the site
with clean gauze or a wash cloth.
Nosebleeds:
Nosebleeds may occur when the air is dry, or during allergy season. Children may develop a nosepicking habit
that makes them worse. Efforts to prevent nose bleeding include: using a humidifier to moisten the air, using
saline nose spray to keep the inside of the nose from cracking, and encouraging your child not to pick his or
her nose. Persistent nosebleeds may require treatment to control. Refer to section on Nosebleeds.
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Infants – 6 months to 1 year old
Relationships
Your baby continues to learn and grow through strong, supportive relationships with his or her family. Parents
provide the foundation through which your baby can explore and learn about the world. Baby is becoming more
independent but still relies on having parents there for support guidance, and comfort.
Continue to use the daily bath as a time to check out your baby - look for bruising, tender spots, swelling, or
anything unusual. Call your hematology team with concerns. When your baby
needs a factor infusion or blood draw, be prepared to comfort him or her. With
infusion your baby may cry loudly! Much of the crying will be because of being
held tightly for the infusion. Babies don’t like to be restrained! Your love and
support will help get your baby through the experience.
The world
Babies learn about the world by exploring it with their senses. This means they
need to touch, taste, look at, and smell everything they can find! You have to allow this, and at the same time
protect your baby from being harmed. The bleeding disorder can make this more challenging.
Baby will begin to roll, crawl, creep, and pull up to stand during this time. As your baby masters new skills, he
or she may fall or become injured. Baby may begin to need factor infusions with these injuries.
Baby-proofing the house is a must at this stage of your baby’s life! It can help to have carpeting to pad the floor
and to put “bumpers” on the crib, coffee table, and hearth. Protective gates are a must near stairways and other
places where injuries might occur.
However, accidents and bleeds may happen. If your child has severe Type 1 or Type 3 VWD you may want to
have an emergency bag ready to go in case you need to go to seek care for bleeding. Fill the bag with contact
information for the hematology team, your flash drive, emergency care information, snacks, and a few things
that will keep baby entertained while awaiting care. Having a bag ready will allow you to add your treatment
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product to the bag and take off quickly if your child is injured.
Bruising
A baby with a bleeding disorder will bruise more easily than other children. He or she may develop bruises
when being picked up, or where the car seat strap crosses his or her shoulder, or on the knees from crawling.
Bruises are unsightly, but should not cause you concern unless they grow larger and cause your baby pain. It
may be helpful to draw a line around a worrisome bruise so that you can tell if it is growing. Ice or cold packs
can be applied to help treat a bruise. If a bruise causes your baby to stop using his or her arm, or to stop
crawling, call your hematology team for advice.
Head bumps
If your baby experiences a “head bump” (if he or she falls while pulling up to stand, hitting his or her head, for
instance) he or she may need a factor infusion and head CT. Call your hematology team for instructions as soon
as possible after the injury.
Mouth bleeds
Mouth bleeds are common and sometimes difficult to control. They may occur when your baby falls with
something in his or her mouth, or bites his or her lip with his or her new teeth. It is difficult, if not impossible,
to apply pressure or ice to an injury in the mouth. It may help to give the baby cold liquids to drink. Amicar®
can often be alone used to control bleeding in the mouth. If your child has severe type 1 or type 3 VWD, factor
infusion may be necessary with Amicar® added to help keep the clot in place. Call your hematology team for
instructions on treating mouth bleeds.
Immunizations
Immunizations are good for your baby’s health! Ask your pediatrician to use the smallest gauge needle possible
when giving shots. After the shot is given, apply pressure to the site for a few minutes.
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School age - 11 to 15 years old
Each developmental stage brings new challenges and, the early teen years are no different. Challenges that
will be faced as the teen starts to mature include the following:
Bleeding
Menorrhagia for females
Heavy and/or prolonged periods can occur in females with von Willebrand disease. Contact
your treatment center for a plan. There are journals to keep track of the bleeding,
medicines to stop the bleeding, and other measures that can be provided by your doctor
or nurse. These measures very individualized to the patient and the treatment center.
What is important to remember is that you can receive help for heavy or prolonged
periods. Work with your treatment center team to develop a plan. See section on
menorrhagia.
Joint and muscle bleeds
It is very rare for VWD patients to experience joint bleeds unless they have severe Type
1 or Type 3 VWD. For those who have experienced joint bleeds: target joints that have
developed previously may continue to cause problems and may present with arthritic
changes. Arthritis pain is very similar to joint bleed pain and even adults have trouble
telling the difference.
Blood in the urine
Seeing blood in the urine is very scary A little blood looks like a lot when mixed with urine. Increasing clear
fluid intake to 4 cups an hour will help Then call your treatment center for further directions. Avoid the use
of Amicar®.
Gastrointestinal bleeds
During this age the preteen or teen can learn to be aware of what blood in stool looks like and should notify
a parent if they see blood in the stool and/or have abdominal pain. Bleeding ulcers can occur and need
immediate treatment.
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Head
Any blows to the head need to be reported to the patient’s parents for aggressive treatment if necessary.
Nosebleeds
Nosebleeds may occur when the air is dry, or during allergy season. Teens may develop a nose picking habit
that makes them worse. Efforts to prevent nose bleeding include: using a humidifier to moisten the air and/or
a saline nose spray to keep the inside of the nose from cracking. Teens need to be reminded not to pick their
noses. Persistent nosebleeds may require treatment to control. Refer to section on nosebleeds.
Dental care
A treatment plan may be needed for any dental procedure other than routine cleaning. If your teen needs
fillings, extractions, root canals, or other procedures, your hematology team should be involved in planning for
the procedure to avoid bleeding.
Orthodontia
Braces are fine for patients with bleeding disorders. The patients and parents need to stay in contact with the
treatment center to have a plan in place in case bleeding occurs.
School
Some teens may stop doing work, skip classes, or start receiving poor grades. Be aware of your teen’s school
performance and, if necessary, intervene quickly with tutors, mentors, etc.
The teen may or may not want to tell others about the bleeding disorder. It is a personal decision and needs to
be respected. The teacher, principal, and school nurse are the only ones who need to know.
Activities
Teens should be encouraged to remain active through individual recreational activities or team sports. See
sports section.
Independence
By the time the child reaches adolescence, he or she needs to know his or her diagnosis, medications, and
what to do for injuries/bleeding. This knowledge helps the teen transition into adulthood.
The teen should be encouraged to interact more with caregivers, including doctors, nurses, physical
therapists, and social workers. Allow your child time alone with personnel to ask questions.
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Spending time away from family, on their own, is important to teens. Sleepovers, summer
camp, travel for sports activitiess etc. will encourage independence and self confidence.
Knowing how to self-infuse (if applicable) and taking responsibility for the disorder is a
major part of becoming independent and is encouraged.
MedicAlert identification
All persons with bleeding disorders should wear medical alert identification.
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Teen 16 to 19 years old
Challenges
Teens need to start to take control of their lives - especially their medical treatment. Under the supervision
(always!) of a parent, the young adult should be the person who orders factor, performs the infusion (as
applicable), makes appointments with medical personnel, calls the nurse when injured and does most of the
talking to the doctor and nurse at comprehensive visits.
In order to make this possible, parents have to start to give up some of their role. It
may be hard, especially for mothers, but it is the only way your child will become an
independent adult, capable of self-care and self-advocacy.
Subjects to discuss:
Life after high school
The time for teens to begin to planning their futures is during the high school years.
Topics that parents should address with their teens include:
•Further education (college or tech school)
•Scholarships, which are available from many sources for persons with bleeding disorders
•Job choices that are safe for people with bleeding disorders
•Insurance availability
Responsibility
By the late teens, your child should be doing almost all of his or her own medical care and follow-up with
minimal supervision. There are additional areas of responsibility though, like driving a car, staying in school,
keeping a job, and saving money.
Insurance
Know your coverage and how long your child will be covered under your policy or under a government program.
Do not wait until your policy is nearly expired to find other coverage. Talk about health insurance as a family.
Again, check with your hematology team about options.
Dating/partner in life
As teens start to date, they may not want to tell everyone immediately about their health. This is normal and
expected. Helping teens know when and who to tell as relationships develop is where parents and treatment
centerstaff can really be supportive. Learn about the genetics of VWD from your hematology team so that you
can make future decisions. See section on genetics.
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Medications
Aminocaproic acid (Amicar®)
Aminocaproic acid or Amicar® is a medicine that helps keep bleeding in the mouth from restarting, once it has
stopped. It is NOT a substitute for factor. When Amicar® is used correctly, it allows the injured area or tooth socket
in the mouth to heal from the inside to the surface.
•Amicar® syrup is not readily available at most pharmacies, so, like factor, you should keep an emergency supply at home and take it along when you travel
•Amicar® is usually given every 6 hours. It is important to stay on schedule so that it will continue working until the area has healed, usually around 3-5 days
•Amicar® syrup comes in a brown bottle that should be stored away from bright light. It should NOT be refrigerated
•Always call treatment center before starting Amicar® to be sure that it is safe for you or your child to take it at the time, and also so that the provider can calculate the proper dose based on current weight
•NEVER give yourself or your child Amicar® if there is blood in the urine, from, for example, an infection, injury or surgery. Tell the treatment provider if you or your child has had blood in the urine recently.
Amicar® helps prevent the breakdown of blood clots. It is most useful with mucous membrane bleeding, (such as
nose, mouth, and gastrointestinal tract bleeding), and is usually not helpful with joint bleeding or other internal
bleeding.
How to give:
Liquid/syrup: Use an oral syringe to measure the amount of medication needed. Take the medicine as directed by your doctor. Be sure to swallow the medicine.
Tips for taking Amicar®:
Fill plastic popsicle mold with Kool-Aid®, then squirt in the Amicar® syrup dose. Freeze. Most children will eat a popsicle even if they won’t swallow the syrup from a teaspoon.
Another way to “disguise” the taste of Amicar® syrup would be to squirt the Amicar® in “ice pop”? or acknowledge Otter Pop trademark? and stand upright to freeze.
Tablets: Swallow as directed. Tablets can be crushed and mixed with a liquid or soft food.
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Intravenous (IV): If you or your child is unable to swallow, Amicar® can be given intravenously.
Special instructions:
Amicar® can be taken with or without food. Give with food if an upset stomach occurs after taking the medication.
It is important to take the medication as directed by your doctor.
Amicar® is dosed by weight. The dose will change as you or your child grows. It should be taken
every 6 hours (6am, noon, 6pm and midnight, for example), not 4 times daily.
If you miss a dose of Amicar®, take it or give it to your child as soon as you remember and do not try to
“make up” a missed dose.
Do not take Amicar® for kidney or bladder bleeding (blood in the urine) unless directed by your doctor.
Amicar® should be used with caution by people who have kidney or liver disease.
Side effects: (that do not need to be reported)
Mild upset stomach
Mild diarrhea
Mild headache
Side effects that must be reported:
Skin rash
Chest pain
Difficulty breathing
Slurred speech
Vomiting
Special considerations:
Tell your doctor if you or your child takes other medications, vitamins, or herbal products.
Keep this medication out of the reach of children.
Keep the medication in its original container and store as instructed.
Call 911 or go to the emergency room if you or your child has:
Chest pain
Slurred speech
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Bleeding disorder terms
acquired von Willebrand disease development of VWD later in life as a result of other
medical conditions
activated partial thromboplastin time
a test that measures clotting time
anemia a decrease in red blood cells that may result in iron deficiency
antifibrinolytics medications that prevent clots that have formed at the site of blood
vessel injury from being prematurely broken down
arthropathy
permanent joint disease caused by repeated bleeding into a joint
autosomal dominant an inheritance pattern where the gene from only 1 parent is needed
to cause a trait or condition
autosomal recessive an inheritance pattern where the gene from both parents is needed
to cause a trait or condition
carrier a person who has 2 different forms of a recessive gene; the carrier
is not affected by the trait or condition but can pass it on to his/her
children
clot the interaction of blood proteins with platelets, resulting in the
formation of a gel-like substance that stops blood vessel bleeding
clotting/coagulation factors proteins in blood that combine to form a clot that stops bleeding
coagulation clotting
complete blood count measures the number of red blood cells, white blood cells,
and platelets
cryoprecipitate a product created from human plasma that is rich in VWF, FVIII, and
other clotting factors but does not undergo viral inactivation and
removal
desmopressin (DDAVP) a synthetic version of antidiuretic hormone that causes the body to
release stored VWF and FVIII into the bloodstream
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dysmenorrhea painful menstruation
endothelium the lining of blood vessels
factor VIII a clotting factor that is protected by von Willebrand factor
factor VIII assay a test that indirectly assesses the severity of VWD and the likelihood of
bleeding by measuring FVIII levels
fibrin a protein involved in blood clotting
hemophilia a bleeding disorder affecting males and caused by a deficiency of
factor VIII (hemophilia A) or factor IX (hemophilia B)
hemophilia treatment center a clinic where a team of doctors, nurses, social workers, and other
specialists work together to deliver comprehensive care to people
with bleeding disorders
hematologist a medical doctor who diagnoses and treats diseases
and conditions associated with the blood system
hormonal therapy estrogen- and/or progesterone-containing products used to reduce
heavy menstrual bleeding and raise levels of von Willebrand factor
and factor VIII in women with von Willebrand disease
intravenous injection into a vein
menorrhagia heavy and prolonged menstrual bleeding
multimer a string of the von Willebrand factor protein
perioperative period the time period surrounding a patient’s surgery; this typically
includes hospital admission, anesthesia, surgery, and recovery
plasma the liquid portion of blood that does not contain blood cells
platelets disc-shaped blood cells that adhere to the site of blood vessel
injury, forming a plug that slows bleeding
postpartum hemorrhage excessive blood loss after childbirth
prothrombin time a test that measures clotting time
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qualitative defects refers to abnormalities in the functioning of the von Willebrand
factor protein; associated with type 2 von Willebrand disease
quantitative defects refers to low levels or the complete lack of von Willebrand factor
and, often, factor VIII; associated with type 1 and type 3 von
Willebrand disease
spontaneous von Willebrand disease the development of a new mutation in a person without a family
history of von Willebrand disease
subendothelium the tissue beneath the endothelium; the site of platelet adhesion
following blood vessel injury
thrombin a clotting factor
thrombocytopenia abnormally low levels of blood platelets
topical agent a medication that is applied directly to tissue to stop bleeding
von Willebrand disease a bleeding tendency caused by low levels or the complete
absence of von Willebrand factor or by abnormal functioning of the
von Willebrand factor protein
von Willebrand factor a protein that is essential for normal blood clotting
von Willebrand factor antigen a test that measures the amount of von Willebrand factor in a
person’s blood
von Willebrand factor/ factor VIII concentrate a replacement therapy that is made from human plasma and
contains both von Willebrand factor and factor VIII
von Willebrand factor multimer test a test that shows the structure of von Willebrand factor; used to
identify the type of von Willebrand disease
von Willebrand ristocetin cofactor activity a test that measures the ability of a person’s von Willebrand factor
to interact with normal platelets; also called von Willebrand factor
activity or simply ristocetin cofactor test
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Medications
Common preparations containing aspirin
Why can’t I take aspirin?
Persons who have bleeding disorders are told not to take aspirin for fever or pain and are usually given a list of
over-the-counter medications that contain aspirin. This does not mean that you have an allergy to aspirin (unless
you have taken aspirin and had a bad reaction such as hives or difficulty breathing, etc).
Aspirin causes platelets to lose the ability to stick together and form a plug so that a blood clot can build up on it.
Persons with von Willebrand disease (VWD) have platelets that function normally, which is why bleeding from just a
scratch or scrape or a nose bleed may stop without taking a dose of factor. If you have VWD and then take aspirin
too, you will lose any help from platelets when you have a bleeding episode.
See below to review the common preparations containing aspirin to avoid.
Please note that this is a partial list. ALWAYS check the ingredients in both over-the-counter and prescription
medications for aspirin, also listed as acetylsalicylic acid or ASA. In addition, always check with your physician,
treatment center, and/or pharmacist before starting any new medication for interactions with your bleeding disorder.
Medications obtained outside the United States are not subjected to the same US regulatory/ labeling processes
and may contain aspirin. Thus, use them only if absolutely needed, and with caution.
Common preparations containing aspirin
Alka Seltzer Plus Cold (Bayer)
B-C Tablets (Block)
Alka Seltzer w/Aspirin (Miles)
Bufferin (Bristol-Myers) - all types
Anacin ( Whitehall) - all types
Buffets II Tabs (JMI)
Arthritis Pain Formula (Whitehall)
Buffex (Hauck)
Ascriptin (Rhone-Poulenc Rorer)
Cama (Sandoz)
Aspergum (Schering-Plough)
Carisoprodol Compound (Various)
Axotal (Adria)
Cope Tablets (Mentholatum)
Azdone (Central)
Damason-P (Mason)
B-A-C (Mayrand)
Dolobid (MSD)
Bayer (Glenbrook) - all types
Easprin (Parke Davis)
Bayer Childrens (Glenbrook)
Ecotrin (Smithkline Beecham)
B-C Cold-Sinus-Allergy Powder (Block)
Empirin (Burroughs Wellcome)
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Empirin w/Codeine (Burroughs Wellcome)
Norgesic Forte (3M)
Equagesic (Wyeth)
Norwich Aspirin (Chattem)
Excedrin (Bristol-Myers) - all types
Oxycodone w/Aspirin (Various) - all types
Fiorinal (Sandoz)
Pain Reliever Tabs (Rugby)
Fiorinal w/Codeine (Sandoz)
Panasal 5/500 (Robins)
Genprin (Alra)
Percodan (Dupont)
Genprin (Goldline)
Percodan Demi (Dupont)
Goody’s (Goody)
Robaxisal (Robins)
Halfprin (Kramer)
Roxiprin (Roxane)
Isollyl Improved (Rugby)
Saleto (Mallard)
Lortab ASA (Whitby)
Sodol Compound (Major)
Magnaprin (Rugby)
Soma Compound (Wallace)
Menoplex Tabs (Fiske)
Soma Compound w/Codeine (Wallace)
Methocarbamol w/ASA (Various)
Synalgos-DC (Wyeth Ayerst)
Momentum (Whitehall)
Vanquish (Sterling)
Night-Time Effervescent Cold Tabs (Goldline)
Wesprin Buffered (Wesley)
Norgesic (3M)
ZORprin (Boots)
There are other medications that also cause platelets to lose their clumping ability. Among these are
common medications that are listed under the category of NSAIDs or non-steroidal anti-inflammatory drugs.
Over-the-counter NSAIDs include ibuprofen, (Advil®, Motrin®, etc) and naproxen (Aleve®, etc). You should not
take these medications without checking with your VWD care provider or treatment center.
There are two NSAIDs that do not cause platelets to lose their clumping ability. These are salsalate (Disalcid®)
and celecoxib (Celebrex®). You need a prescription from your provider for any of these medications for joint
pain relief.
All NSAIDs have the potential of causing stomach irritation. They should be used with caution by persons with
bleeding disorders and discontinued at the first sign of stomach pain or burning.
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Contact information / resources
Contact information for regional treatment centers for bleeding disorders
Centers for Disease Control and Prevention (CDC) supports a network of specialized healthcare centers to
prevent and reduce complications experienced by persons with certain blood disorders. These centers provide
comprehensive health management and prevention services to persons with bleeding disorders. The centers use
multi-disciplinary teams of health-care specialists, as well as state-of-the art clinical research programs, and
outreach and education programs.
Currently, the network consists of approximately 140 hemophilia treatment centers (HTCs).
To locate a regional HTC, please see the following website: https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
The National Hemophilia Foundation is dedicated to finding better treatments and cures for bleeding and clotting
disorders and to preventing the complications of these disorders through education, advocacy, and research.
Established in 1948, the National Hemophilia Foundation has chapters throughout the country. Its programs and
initiatives are made possible through the generosity of individuals, corporations, and foundations as well as through
a cooperative agreement with the CDC.
To find your local chapter, please see:
National Hemophilia Foundation (NHF) Chapters
http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=51&contentid=193
The World Federation of Hemophilia (WFH) is an international not-for-profit organization dedicated to improving
the lives of people with hemophilia and related bleeding disorders. Since it was established in 1963, the WFH has
grown into a truly global network with member organizations in more than 113 countries and official recognition from
the World Health Organization. WFH works in partnership with healthcare professionals and people with hemophilia,
governments and regulators, industry, and foundations, to improve hemophilia care throughout the world.
Passport from World Federation of Hemophilia
The Passport, is a directory of HTCs and hemophilia organizations worldwide. The electronic Passport is updated
daily (as changes are received). You can use the various search features to get a list of all the centers in a country
you will be visiting or to find an address of an organization or a person. Please refer to this website.
http://www.wfh.org/en///page.aspx?pid=1264
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Medications
DDAVP®/ Stimate®
DDAVP® causes the release of Factor VIII and von Willebrand factor from the cells lining the blood vessels into
the blood stream. This prevents, or helps stop, bleeding. DDAVP®is used to treat people with bleeding disorders
such as von Willebrand disease (VWD) or mild hemophilia A.
How to administer:
Intravenous (IV): DDAVP® can be given into a vein.
Intranasal (IN): Stimate® is given as a nose spray.
Subcutaneous (Sub Q or SQ): DDAVP® can also be given subcutaneously.
Stimate® is a name brand medicine; there is no generic form of the medicine. Concentration is 1.5 mg/mL.
Generic forms (used for the prevention of bed wetting) of the medication have lower concentration and will not
help stop bleeding.
Special instructions:
DDAVP®/Stimate® may not work for all people with VWD or hemophilia A. Your doctor may recommend a DDAVP®/Stimate® challenge test to see if this medicine works for you or your child. The test is performed
by giving a dose of the medication and testing the blood before and afterward to see if the circulating factor levels rise. Not all hospitals or pharmacies stock Stimate®. You should keep a supply of the medication
for your own use. Order from the pharmacy in advance of running out, to ensure a supply.
After each dose of DDAVP®/Stimate®, you or your child should take just enough fluids to satisfy
thirst for about 24 hours. Ask your hematology doctor or nurse for more specific instructions. There
are no restrictions for foods taken with the medicines.
DDAVP®/Stimate® is dosed by weight. Dosing will change as a child grows. Be sure to speak with your
hematology team regarding dosing instructions.
Before using Stimate®, blow your nose or have your child blow his or her nose to remove any discharge
or blood clots. Then use the nasal spray (1 or 2 sprays) depending on weight.
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Side effects (that do not need to be reported):
Redness and warmth of the face and neck
Mild fluid retention
Mild headache
Side effects that must be reported immediately:
Very rapid heart rate
Fever of 101.5 ºF (38.6 ºC) or greater
Chills
Dizziness
Seizures
Report use of other medications, vitamins, and herbal products to your doctor.
Keep this medication in its original container and store as instructed.
Call 911 or go to the emergency room if you or your child has:
A seizure
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Dental care and VWD
People with von Willebrand disease (VWD) have the same types of dental problems that other people have.
No treatment is required for routine cleaning. However, if either you or your child needs dental work such as
fillings, extractions, root canals, or other procedures, your hematology team should be involved in planning
for the procedure so that you don’t have bleeding. Please contact your team at least 3 weeks prior to the
procedure.
Good dental and mouth care will help prevent mouth bleeding. You can also take steps to prevent mouth
injuries when possible. Even with the best dental care, mouth bleeds may still occur. You should learn how
to recognize and treat mouth bleeds quickly so that you or your child will have fewer complications from the
bleeding.
Prevention
•Oral hygiene- Brush your teeth twice a day. Daily flossing removes food and bacteria that your toothbrush can’t reach. Regular brushing and flossing will help your gums remain healthy - and healthy gums bleed less!
•Healthy diet- Eat a variety of foods every day. A balanced diet includes meat, vegetables, fruit, grains, and dairy products. Calcium-containing foods like cheese, yogurt, and milk help to keep teeth and bones strong.
•Regular dental care- Visit the dentist every 6 months. Regular checkups and cleaning help keep your teeth and gums healthy.
•Don’t use tobacco- Don’t smoke or chew tobacco. People who use tobacco are more likely to have gum disease and plaque on their teeth, which that causes cavities. If you don’t currently smoke - don’t start! If you already smoke, consider a smoking cessation program.
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Dental Issues for People with VWD
•Routine dental care- Most of the time, people with VWD don’t have bleeding with routine
dental procedures like prophylactic cleaning. It can be helpful to have Amicar® available if you
experience oozing from the gums after dental work.
•Tooth eruption- Usually there is no bleeding with tooth eruption. If the bleeding is prolonged, call
your hematology team. Amicar® or Cyklokapron® may help control this bleeding.
•Tooth loss- Usually there is no bleeding with the loss of “baby teeth.” Again, if the bleeding is prolonged, call the hematology team.
•Tooth extraction- When primary or “baby teeth” are taken out, bleeding is often minimal because
the roots of these teeth are not very deep. As a person ages and gets permanent teeth, the roots go much deeper. Sometimes it is necessary to extract teeth due to crowding in the mouth,
as part of orthodontic treatment, or if the tooth is diseased. A treatment plan to prevent bleeding
is usually necessary for this type of procedure. Call your hematology team before having any teeth pulled out.
•Orthodontic treatment- People with VWD are able to wear braces. Discuss with your hematology team.
•Mouth injury- Injury to the lip, gum, or tongue can produce an injury that bleeds. Try to remember
that a little blood looks like a lot when mixed with saliva. Drinking cold liquids may help control
oral bleeding. If you can apply pressure to the site of bleeding with a piece of gauze or moist tea
bag, this may be helpful. If the bleed keeps recurring, Amicar® may be helpful in controlling it. Call your hematology office for advice.
Bleeding of the tongue or throat can be very serious if it produces swelling that blocks the airway.
Call 911 if you have or your child has difficulty breathing or swallowing following mouth or throat injuries.
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Disaster preparedness
It is important to keep this flash drive in a safe, but readily accessible (easy-to-locate) place. If you choose to
have your health information on this drive, it can then be used during a bleeding emergency (at the emergency
department) or for another emergency in disaster situation.
The best thing you can do in case of a disaster or emergency is to be prepared.
Preparation
Designate a clean, safe place to keep a duffel bag, filled with important items, such as important local and out
-of-state phone numbers (including hemophilia treatment centers), a water bottle, flash light, first aid kit, flash
drive (or other material) with health information, a dose* or 2 of factor concentrate and supplies for infusion
(if you treat with factor), bandages, activating ice pack, and other identifying information. Think of this as your
“To GO” bag!
*If you do set aside a dose of factor, make sure to monitor the expiration date and rotate as necessary.
Ideas from the CDC website regarding other items to prepare in the event of an emergency:
http://emergency.cdc.gov/
Water
Store 3 gallons of water per person and 4 additional gallons per person if you are confined to the home
during the emergency.
Food
Stock up on non perishables: a 3- day supply with an additional 4-day supply per person for use at
home.
Other items**
Prescription and non prescription medicines, kitchen accessories, battery-powered radio or TV,
several flashlights, batteries, change of clothing and shoes, blankets, sleeping bags, hygiene items,
entertainment, paper, pencil, medicine dropper, spare keys, cash, credit card, matches in waterproof
container, compass, photo identification and passport.
**As with the factor, be sure to rotate the emergency supplies and replace expired items. A 6 month time frame
for replacement of these items should be sufficient.
CDC link to list of HTCs in the United States: https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
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Emergency identification
Wearing a MedicAlert® bracelet or necklace is the best way to identify that you or your child has a bleeding
disorder. Young children can wear a bracelet around either a wrist or and ankle. Older children and adults can
wear a bracelet or necklace. Sports wrist bands are available for children who are very active. Your treatment
center can assist you in getting medical alert identification with accurate information.
If your treatment center or homecare company provides you with an Emergency or Bleeding Alert card, please
be sure, as parents, that you carry the card for your child. Teens and adults should always keep the card in
their wallet.
Check out this website for more information:
http://www.medicalert.org/home/Homegradient.aspx
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Medications
Factor facts
By Susan C. Zappa RN CPN
Cook Children’s Medical Center
You have been given a very important job.
You are storing an intravenous replacement factor product in your home. This is a privilege.
Learning about this factor product will assist you and your bleeding disorders center to make this a successful
combined effort to enhance the care of yourself or your child.
Factor products are very expensive: The cost can be approximately $300 per dose for the smallest baby
and up to $4000-$7000 per dose for an adolescent or a child with an inhibitor to factor VIII or factor IX.
Due to the expense and because we do not want to waste a precious resource, we treat factor products as if they were “gold.” Never throw away factor without first talking to your nurse
coordinator. No matter what you think, or what has happened, call your nurse first! On the factor box and the bottle with the powder you will find these four important items:
•Factor name: important to remember this and the company
•Amount of international units (IU): this is the actual dose - written 287 IU or IU 287 (examples). Factor is measured in international units, abbreviated IU on the box,
but called “units” by your doctor and nurse. Your factor may be measured in IU of factor VIII, FVIII or
AHF, IU of ristocetin cofactor or IU of VWF:RCo.
•Lot number: important to record so you can track the factor given.
•Expiration date: best used by, but if expired, again, call your nurse before throwing away.
Sample box label:
Antihemophilic Factor/ von Willebrand Factor Complex (Human)
Brand Name
Lot 27NO5R1
Exp. 12 Oct 12
IU AHF/Vial: 605
IU VWF:RCo/Vial: 1600
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Factor is calculated by body weight and the amount of percent increase the doctor wishes to accomplish
with the factor level.
Factor comes in vials that have different amounts of IU in them, and they are not usually the exact dose
calculated.
Ex: your doctor orders you or your child to have 500 IU of VWF:RCo. The factor bottle has
555 IU VWF:RCo in it. We give the entire amount.
If the bottle has 478 IU of VWF:RCo in it, that’s okay, too, the amount is an approximate amount, not exact.
Doses are usually 10% above or 10% below the actual calculated dose.
Storage of factor products:
Place in refrigerator in a plastic bag to keep the box intact and clean.
Keeping factor in the refrigerator helps to make sure it will stay at the exact potency on the box until the
expiration date.
Factor can be stored at room temperature, though, for different lengths of time (months to years). Each
product is different, so read the package insert or check with your nurse.
Factor does not need to be continually refrigerated, just kept out of extreme hot temperatures
(temperatures that may happen in a car in the summer).
People have thought they needed to keep it cold for the trip to the hospital - not true. People
have thought they needed to keep it cold for a day of travel in an airplane (see also ‘Travel’ section) - not true.
People have thrown factor away that was in the refrigerator, because the power went off for several days - it
is still good.
As you can see, you need to talk to
bad. Factor may lose a little of it’s
is intact and the rubber stopper is
your nurse before you think that factor has “spoiled” or gone
potency, but it does not spoil or go bad as long as the container
not punctured.
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You may have enormous amounts of factor to keep refrigerated:
You can take the bottles with the powder in them out of the box and just refrigerate those
bottles. You need to know how to read the units (IU) on the bottle, so you can mix up the correct dose, and
you need to keep the box and bottle with water in a clean, dry place. Mixing the factor:
Mix the factor using the directions given by the manufacturer. Use the mixing tool or needle-less system
provided. Each brand of factor has its own system to mix. Each brand also has different water amounts. You
must use all of the water provided. You may have to mix two or more vials of different strengths to reach your
child’s dose. You can draw up all of the factor in one syringe, after you have mixed all of the vials.
Once you mix the factor it should be given within the manufacturer’s recommended time frame (usually 3
hrs.). Again, the factor can still be okay to use, especially if refrigerated after being mixed, for longer periods
of time, so check with your nurse.
Traveling with factor:
Keep factor with you at all times.
Pack it in your carry-on luggage only; keep it in the passenger part of the car (not the trunk).
Do not allow it to become really hot.
It is best to have a letter from the doctor explaining the factor and syringes when you travel
through airports.
Copyright 2010
Printed with permission from Susan Zappa, RN. CPN
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Medications
Factor products
How is von Willebrand disease (VWD) treated?
The treatment of VWD varies according to its type and severity. Most people have mild VWD and may need
treatment only at the time of injury, surgery, or dental procedures. Such treatment is often provided at
treatment centers for VWD patients, which are staffed by a team of doctors, nurses, social workers, and other
specialists who work together to deliver comprehensive care to people with bleeding disorders.
When medications are required to treat VWD, they are used to:
• Increase the release of stored von Willebrand factor (VWF) and FVIII into the circulation
• Replace VWF
• Prevent the breakdown of clots
• Control heavy menstrual bleeding in women
How is VWD replaced?
VWF is replaced by plasma-derived FVIII clotting factor concentrates that also contain VWF (FVIII/VWF
concentrates). Originally developed for patients with hemophilia A, FVIII/VWF concentrates are also used by
people with VWD who do not respond to DDAVP® or who are experiencing serious bleeding, undergoing major
surgery, or giving birth and require extended therapy to control hemorrhage. FVIII/VWF concentrates are
given by intravenous infusion and, like DDAVP, can be used at home.
FVIII/VWF concentrates are made from human plasma. Products made from human plasma may carry a risk
of transmitting infectious agents, e.g., viruses and, theoretically, the Creutzfeldt-Jakob disease (CJD) agent.
FlashLINK to VWD
Genetics of von Willebrand disease (VWD)
How is VWD inherited?
VWD is a genetic disease that can be inherited from either parent.1 It affects males and females equally. A
man or woman with VWD has a 50% chance of passing the gene on to his or her child. There are no racial or
ethnic associations with the disorder. A family history of a bleeding disorder is the primary risk factor.
VWD subtype I and II are usually inherited in what is known as a “dominant” pattern. This means that if even
one parent has the gene and passes it to a child, the child will have the disorder.
Inheritance Diagram - Autosomal Dominant
Mother with gene
Father with gene
Mother with gene
Father with gene
Inheritance Diagram - Autosomal
Dominant
= Female
= Male
= Female with affected VWD gene
= Female
Male with affected VWD gene
= Male
= Male with affected VWD gene
VWD Type III von Willebrand disease, however, is usually inherited in a “recessive” pattern. This type occurs when
the child inherits
the gene from bothDiagram
parents. Even if both
parents have mild or Dominant
asymptomatic disease, their
Inheritance
- Autosomal
children are likely to
be severely
affected.
BothFather
Parents
Mother
with gene
with gene with
VWD - Autosomal Recessive
= Female
Both Parents= Female
with = Male
Male
VWD - Autosomal ==Recessive
Female with affected VWD gene
=
= Female
=
= Male
Female with two affected VWD genes and severe VWD
=
with
affected
VWD
gene
= Female
Male with
two
affected
VWD
genes and severe VWD
= Female with two affected VWD genes and severe VWD
= Male with two affected VWD genes and severe VWD
25% no VWD
25% severe VWD
50% VWD
25% no VWD
25% severe VWD
50% VWD
Both Parents with
VWD - Autosomal Recessive
How did my child get VWD if I have no family history?
= Female
Neither Parents with
VWD
= Male
Occasionally, a person without a family history will develop VWD.
Spontaneous
= Female
with affected VWDVWD
gene results from a new mutation
=
Male
with
affected
VWD
gene
= Female
25% no
VWDinherited VWD, it can be passed on.
in the VWF gene and,
like
SpontaneousNeither Parents with VWD
25% severe VWD
50%
VWD
Mutation
= Female with two
affected VWD genes and severe VWD
= Male
= Male with two=affected
genes and
severe
Female VWD
with affected
VWD
geneVWD
= Female
= Male
In rare cases, VWD develops later in life due to other medical conditions (such as autoimmune diseases, diabetes,
Spontaneous
etc.).2 This form of the disease is referred to as acquired VWD, and it cannot be passed on to the person’s
Mutation
children.
Neither Parents with VWD
= Female
= Male
Spontaneous
Mutation
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How to draw your family tree
If you want to form your family tree, you can easily do so with the help of a free family tree maker. If you do
not know how to get one “Here are some tips to finding a family tree software program that’s right for you:”
• The easiest way to get a free family tree maker is to search online. Go to a search engine like
Google or Yahoo! and simply search for “free family tree maker.” You will likely get hundreds of
responses and a lot of options to download free software. You can check out the various options
and see what suits you the best.
• Ask your friends and family members. As soon as you get some names, look for theirfree trial versions. Simply go through their features and choose what you think will best suit your needs.
• You can also look for the reviews of the various software packages. These reviews will help you
decide which one is the best for you. Decide upon one and download the free trial version. This is a
safe and secure way to get a family tree maker for free.
Even if you get the free family tree maker easily, you should look for certain qualities, so that you choose the
right one for yourself.
• Ease of use - It should be easy to use and help to make the complex tasks simple. It should let you manage thousands of individual pieces of information in an easy and proper manner. Keep it in mind that you should enjoy working on the family tree.
• Face recognition technology - It should be able to recognize faces easily. It should help you identify
unknown individuals and help you track related photos.
• Multilingual - It should support a lot of different languages and should allow for bi-lingual data entries.
It should also be capable of making intelligent translations.
• Excellent visual interface - It should have a good visual interface that should make the task lively so
that it seems as if you are drawing it yourself.
• It should let you publish your family tree online on the website of your choice.
Remember free does not always equal good, so keep looking for the right option, and do not settle for just
anything that comes free.
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References:
1. NHF. National Hemophilia Foundation. von Willebrand disease. Available at:
http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=182&contentid=47&rptname=bleeding.
Accessed September 24, 2010.
2. NHLBI. National Heart, Lung, and Blood Institute. von Willebrand disease. Available at:
http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html
Accessed September 24, 2010.
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Female issues in von Willebrand disease (VWD)
Gynecologic complications
Gynecologic complications may occur in women with VWD. Menorrhagia (heavy menstrual cycle), is the most
common symptom of this bleeding disorder. Women with VWD or similar bleeding disorders are susceptible
to gynecologic issues that all women face. In particular, they may have a higher risk of developing those
gynecologic conditions that are associated with bleeding (for example, hemorrhagic ovarian cysts).
For optimal management of these challenges, a close collaboration between the obstetrician/gynecologist and
hematologist is needed.
Gynecologic complications may include:
Endometriosis
Endometriosis is a condition in which the tissue that behaves like the cells
lining the uterus (endometrium) grows in other areas of the body, causing
pain, irregular bleeding, and possible infertility.
The tissue growth typically occurs in the pelvic area, outside of the uterus,
on the ovaries, bowel, rectum, bladder, and the delicate lining of the pelvis.
However, this can occur in other areas of the body, too.
Dysmenorrhea
Primary dysmenorrhea is common menstrual cramps. Cramps usually begin 1 to 2 years after a woman
starts getting her period. Pain is usually felt in the lower abdomen or back and can range from mild to severe.
Common menstrual cramps usually become less painful as a woman ages, and may stop entirely if the woman
has a baby.
Secondary dysmenorrhea is pain that is caused by a disorder in the woman’s reproductive organs. Pain from
secondary dysmenorrhea usually begins earlier in the menstrual cycle and lasts longer than common menstrual
cramps.
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Mittelschmerz
Mittelschmerz is characterized by lower abdominal and pelvic pain that occurs roughly midway through a
woman’s menstrual cycle. The pain can appear suddenly and usually subsides within hours, although it
may sometimes last 2 or 3 days. In some cases it can last up to the following cycle. In some women, the
mittelschmerz is localized enough so that they can tell which of their 2 ovaries provided the egg in a given
month. Because ovulation occurs on a random ovary each cycle, the pain may switch sides or stay on the same
side from one cycle to another.
Diagnosis of mittelschmerz is generally made if a woman is mid-cycle and a pelvic examination shows no
abnormalities. If the pain is prolonged and/or severe, other diagnostic procedures such as an abdominal
ultrasound may be performed to rule out other causes of abdominal pain.
Hemorrhagic ovarian cysts
Hemorrhagic cysts occur in women of all ages and often forms during the most fertile years. This is due to
the fact that ovarian cysts are formed when the egg is not released properly from the ovaries and fluid buildup
occurs. Usually if a woman is taking contraceptives, the cyst never appears because the woman is missing
her regular menstrual cycles that create the egg and release it from the ovaries.
Another name for a hemorrhagic ovarian cyst is a blood cyst. They resemble a
normal benign functional cyst, but the only difference is that it forms because
the blood vessels in the walls of the cysts burst, causing blood to enter into the
cyst.
Uterine fibroids
Uterine fibroids are benign tumors that originate in the uterus (womb). Although they are composed of the same
smooth muscle fibers as the uterine wall (myometrium), they are many times denser than normal myometrium.
Uterine fibroids are usually round
or semiround in shape.
Most women with uterine fibroids
have no symptoms.
However,
abnormal uterine bleeding is the
most common symptom of a fibroid. If
the tumors are near the uterine lining or
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interfere with the blood flow to the lining, they can cause heavy, painful, or prolonged periods or spotting
between menses. Women with excessive bleeding due to fibroids may develop iron deficiency anemia. Uterine
fibroids that are deteriorating can sometimes cause severe, localized pain.
Uterine polyps
Uterine polyps are growths attached to the inner wall of the uterus
and protruding into the uterine cavity. Overgrowth of cells in the lining
of the uterus (endometrium) leads to the formation of uterine polyps.
The size of uterine polyps ranges from a few millimeters — no larger than
a sesame seed — to several centimeters (golf ball-sized or larger). They are
attached to the uterine wall by a large base or a thin stalk.
You can have one or many uterine polyps. They usually stay contained within your
uterus, but occasionally, they may slip down through the opening of the uterus into your vagina. Although they
can happen in younger women, uterine polyps most commonly occur in women in their 40s and 50s.
Infertility
Infertility is a malfunction of the reproductive system that impairs the body’s ability to reproduce.
About 6.1 million women and their partners in the United States are affected by infertility. This number
translates to about 10% of the reproductive-age population.
References
Demers C et al. Gynecological and Obstetric Management of Women with Inherited Bleeding Disorders. JOGC
2005; 163:707-718.
Lee CA et al. The obstetric and gynaecological management of women with inherited bleeding disorders –
review with guidelines produced by a taskforce of UK Haemophilia Centre Doctors’ Organization.
Haemophilia 2006; 12:301–336.
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How to back up your flash drive
The data on your flash drive may be safe and secure now; but take a moment to think of all the potential
disasters that could strike at any time: like losing your flash drive, theft, operator error, fire, or damage due to
water or other accidents. Backing up your flash drive is a necessary safeguard against those potential threats.
To back up important medical information that you have entered into your interactive forms, simply following
these steps:
1. After you have entered personal information into one of the three interactive forms (Individual emergency medical information, Clinic follow up sheet, or your Log Sheet), you must save your edited file. To save your file, select or click on the “Save” button in the lower left corner of the page. Make sure you have seen the “Save successfully” message pop up to confirm that you have successfully saved your information.
2. Select or click on the “Printer Friendly” button in the upper right corner. You will now see a preview of your printed page.
3. Select or click on the “Backup” button in the upper left corner of this page.
4. Save your file to your preferred location on your PC, not to your flash drive if you wish to keep a copy of this file in a separate secure place. You may keep the given name of this file or rename it at this time. Save your file as an html file by selecting or clicking on the “Save” button in the Window.
5. Select the “Back” button on the upper right corner of your preview page to return to the Table of Contents.
6. Your file will be saved on your computer, following your directions as to your preferred location and drive.
Regular back up of files is encouraged especially if your medical information has changed or is changing. You’ll
also want to keep your clinic follow up sheets and infusion records up to date.
FlashLINK to VWD
von Willebrand Disease (VWD) and your child’s development
Infancy – the first 6 months
Relationships
Your baby learns and grows through strong, supportive relationships with his or her
family. Parents provide the foundation through which your baby can explore and learn
about the world. Get to know your infant, and learn to tell the difference between his or
her “hungry cry” and “pain cry”. This will help you as you learn to respond to injuries
and bleeding.
Use the bath time to check out your baby- looking for bruising, tender spots, swelling or
anything unusual. Call your hematology team with concerning findings.
The World
Your baby learns about the world by exploring it with his or her senses. This means the infant needs to touch,
taste, look at and smell everything he or she can find! You have to allow this, and at the same time protect him
or her from being harmed. The bleeding disorder can make this more challenging.
Bleeds are very rare in the first few months, because your baby isn’t moving very much yet. Among the most
frequent bleeds are:
Bleeding with circumcision
This is extremely rare. There should be no more than a few drops of blood with circumcision.
Immunizations
Bruising
A baby with a bleeding disorder will bruise more easily than other children. Baby may develop bruises from
being picked up, or where the car seat strap crosses his or her shoulder, or on the knees with crawling. Bruises
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are unsightly, but not too concerning unless they grow larger and cause pain to the baby. It may be helpful to
draw a line around a concerning bruise so that you can tell if it is growing. Ice or cold packs can be applied to
help treat a bruise. If a bruise causes your baby to stop using his or her arm, or to stop crawling, call the HTC
for advice.
Head bumps
If your baby experiences a “head bump” (if he or she rolls off the bed, hitting his or her head for instance) you
should contact your VWD care provider to discuss whether or not an evaluation is necessary.
Mouth bleeds
Mouth bleeds are common and sometimes difficult to control. They may occur when your baby gets a new
tooth, or when he or she falls with something in his or her mouth. It is difficult, if not impossible, to apply
pressure or ice to an injury in the mouth. It may help to give the baby cold liquids to drink. Amicar® can often
be used by itself to treat a mouth bleed. If your child has severe type 1 or type 3 VWD, factor infusion may be
necessary, with Amicar used to help keep the clot in place. Call your hematology team for directions on how to
treat mouth bleeds.
Nose Bleeds
Infants with VWD may have nosebleeds that are difficult to control. With a nosebleed, hold the baby upright
so that blood will come out of the nose and not drain down the throat. Hold the nose to apply pressure
if possible. Amicar may be used to help control nosebleeds that recur. Call your hematology team for
directions on how to treat baby’s nosebleeds.
Nosebleeds can often be prevented with good nasal hygiene. It is helpful to keep nasal passages moistened
to prevent cracking of fragile membranes in dry weather. A humidifier can be helpful to prevent problem
nosebleeds. Refer to the Nosebleed section!
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von Willebrand Disease (VWD) and your child’s development
Relationships
her “hungry cry” and “pain cry”. This will help you as you learn to respond to injuries
and bleeding.
Use the bath time to check out your baby- looking for bruising, tender spots, swelling or
anything unusual. Call your hematology team with concerning findings.
The World
Your baby learns about the world by exploring it with his or her senses. This means the infant needs to touch,
taste, look at and smell everything he or she can find! You have to allow this, and at the same time protect him
or her from being harmed. The bleeding disorder can make this more challenging.
Immunizations
Bruising
A baby with a bleeding disorder will bruise more easily than other children. Baby may develop bruises from
being picked up, or where the car seat strap crosses his or her shoulder, or on the knees with crawling. Bruises
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are unsightly, but not too concerning unless they grow larger and cause pain to the baby. It may be helpful to
draw a line around a concerning bruise so that you can tell if it is growing. Ice or cold packs can be applied to
help treat a bruise. If a bruise causes your baby to stop using his or her arm, or to stop crawling, call the HTC
for advice.
Head bumps
If your baby experiences a “head bump” (if he or she rolls off the bed, hitting his or her head for instance) you
Mouth bleeds
treat mouth bleeds.
Nose Bleeds
directions on how to treat baby’s nosebleeds.
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Relationships
her “hungry cry” and “pain cry.” This will help you as you learn to respond to injuries
and bleeding.
Use the bath time to check out your baby - look for bruising, tender spots, swelling or
anything unusual. Call your hematology team with concerns.
The world
Babies learn about the world by exploring it with their senses. This means they need to touch, taste, look at,
and smell everything they can find! You have to allow this, and at the same time protect your infant from being
harmed. The bleeding disorder can make this more challenging.
Immunizations
Bruising
A baby with a bleeding disorder will bruise more easily than other children. Your baby may develop bruises from
being picked up, or where the car seat strap crosses his or her shoulder, or on the knees from crawling. Bruises
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are unsightly, but should not cause you concern unless they grow larger and cause your baby pain. It may be
helpful to draw a line around a worrisome bruise so that you can tell if it is growing. Ice or cold packs can be
applied to help treat a bruise. If a bruise causes your baby to stop using his or her arm, or to stop crawling, call
the HTC for advice.
Head bumps
If your baby experiences a “head bump” (if he or she rolls off the bed, hitting his or her head, for instance), you
Mouth bleeds
treat mouth bleeds.
Nosebleeds
directions on how to treat your baby’s nosebleeds.
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Infusion record
Why keep records of infusions?
In individuals with VWD, von Willebrand Factor (VWF) may need to be replaced by infusing FVIII/VWF complex
concentrates, often called FVIII/VWF concentrates. Originally developed for patients with hemophilia A, FVIII/
VWF concentrates are also used by people with von Willebrand Disease (VWD) who do not respond to DDAVP®
or who are experiencing serious bleeding, undergoing major surgery, or giving birth and require extended
therapy to control hemorrhage. FVIII/VWF concentrates are given by intravenous infusion and, like DDAVP, can
be used at home. FVIII/VWF concentrates are expensive.
When FVIII/VWF concentrates are given to a patient in the hospital or clinic, the number of units and the lot
number used are recorded in the patient’s chart along with the reason it was given. When your hematology
team decides that your family is ready to have factor at home and someone learns to infuse factor without a
nurse present, it is important to continue the recordkeeping part of home therapy.
How are these records used?
1.The record of infusions lets the team know if you or your child is at risk of developing a target joint.
The earlier that a target joint is treated, the less likely it is to cause permanent damage. Bring in your infusion records every time you visit the clinic!
2.If you or your child is on prophylaxis and the number and frequency of? the infusions were the same
for the past 6 months, a written record allows the team to know if there was any breakthrough
bleeding or procedure that might require more infusions. Maybe the dose needs to be increased or the
days of the week need to be changed….OR…..maybe you can decrease the number of days of prophylaxis each week.
3.If there is ever a need to see if you or your child had an infusion from a particular lot number, you and
the treatment center will have that information. These records were critical for people to have during
the days before the factor products were free from viruses.
4.Insurance companies/Social Security Disability base payment on the record of factor used. This is the only way that the treatment center can prove that you or your child has bleeding episodes and
is using factor!
5.It is possible to have your home therapy privilege taken away if you do not keep accurate records of
infusions.
Discuss any number of infusion recordkeeping methods - including web based programs - with the
nurse.
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Joint bleeding or hemarthrosis treatment options
Introduction
Joint bleeding or (hemarthrosis) is very rare in those with von Willebrand disease (VWD) unless they are a
severe Type 1 or Type 3. As with hemophilia, the most common joints that persons with severe VWD may bleed
into are the ankles, knees, and elbows. But joint bleeding is not limited to just these joints. Joint bleeding can
occur with significant orthopaedic trauma in any person with a bleeding disorder, but it is rarely spontaneous
in VWD patients.
Treatment
The number one treatment for a joint bleed is factor replacement. This should be instituted as soon as the
person/child notes a problem. The optimal time for treatment is 2 hours or less from the onset of symptoms to
the infusion of factor. This is not always possible, if the family needs to access the emergency room, but every
effort should be made to try. Most joint bleeds will require follow-up treatment (this will depend on the joint
involved and the type and severity of the bleed).
In addition to factor replacement, RICE is an important adjuvant treatment for a joint bleed. RICE stands for
rest, ice, compression, and elevation.
Rest
Rest entails staying off the joint if it is a weight-bearing joint (ankle, knee) or simply not using a joint if the
bleeding is in the upper extremity. This can be accomplished in a number of ways: using crutches, staying
home on the couch, using a sling, etc. It is often difficult to have young children rest so having a new video or
movie on hand to introduce at the time of the bleed may be helpful.
Ice
Ice should be used intermittently for the first 24 hours after a bleed occurs. It should not be left on for longer
than 20 minutes at a time. Do not apply ice directly to the skin; make sure that there is a towel or other cloth
(like a sock) in between the skin and the ice.
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Compression
Compression can be applied using an elastic bandage or support. It should not be so tight as to cut off
circulation.
Elevation
The joint should be elevated higher than the heart to help reduce swelling. Keep in mind that you cannot
adequately elevate a knee or ankle from a sitting position-you must be lying down.
Joint bleeds can be very painful. It is important to address this along with other treatment aspects. Tylenol®
can be given to most people to control their pain. It is important to remember to give it to young children as
well because they do not always verbalize the pain they are having. NSAIDs can be used safely in persons
with hemophilia on the advice of your treatment center. Not all treatment centers allow the use of these
medications. Rarely a person having a joint bleed might require a narcotic for pain relief. Narcotics require a
prescription from your physician.
Chronic hemarthrosis
Chronic hemarthrosis is very rare in VWD but can occur after repeated bleeding episodes into the same joint.
Over time the body will breaks down and absorbs the blood left in a joint following a hemarthrosis. The blood
that is absorbed and iron left behind are irritants and can cause the synovial lining of the joint to become
inflamed and swollen. This swollen synovium bleeds easier so the person with hemophilia may note frequent
bleeds into this joint. This is called a “target joint”. This can cause a loss in motion of the joint and can
eventually lead to chronic joint disease or arthritic changes. Early treatment of joint bleeds is the best way to
prevent these complications.
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Links to other resources
The National Hemophilia Foundation (NHF)
NHF seeks to promote and enhance the quality of life for all people with coagulation disorders through education,
advocacy, and research. Towards these ends, NHF and its 48 chapters nationwide engage in a number of program
initiatives. NHF supports important clinical research, funds educational outreach, issues medical advisories,
convenes educational meetings and symposia, advocates for improved treatments, makes policy recommendations,
and promotes communication and cooperation among all members of the community.
NHF also offers a wide range of educational services and publications through HANDI, its information resource
center.
NHF Member Chapter Organizations
NHF’s 48 state and local chapters are wonderful resources for networking with people affected by bleeding disorders.
Many chapters provide valuable information, materials, advocacy, and support to area families. Some chapters assist
individuals with accessing appropriate healthcare and support groups, become involved in state-based advocacy
issues, and provide financial assistance and other special services. While many people with bleeding disorders
have found the services provided by their chapters indispensable, and still others have built upon their chapter
experiences to take on leadership roles within the community.
For more information about NHF, go to: http://www.hemophilia.org.
Hemophilia Treatment Centers (HTCs)
There are approximately 140 hemophilia treatment centers in the United States that offer comprehensive medical
care to people with coagulation disorders. Each of these federally funded hospitals is staffed by at least a
hematologist, nurse, social worker, and physical therapist. These medical professionals work together as a team to
provide quality treatment, education, and psychosocial support services for patients and their families.
To find the HTC located closest to you, click on the U.S. Centers for Disease Control and Prevention link below:
https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
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Listed below are direct links to several websites related to VWD.
American Thrombosis & Hemostasis Network (ATHN)
http://athn.org/
Bloodline
www.bloodline.net
Centers for Disease Control and Prevention (CDC), Bleeding Disorders in Women Facts
http://www.cdc.gov/ncbddd/hemophilia/women.html
Factor Foundation of America
www.factorfoundation.org
Hemophilia Alliance
www.hemoalliance.org
Hemophilia Federation of America
www.hemophiliafed.org
Hemophilia Forum
http://www.factorfacts.com/en/hemophilia-forum.html
Hemophilia Region VI
www.hemophiliaregion6.org
Hemophilia Region IX
www.hemophiliaregion9.org
Inalex Communications
www.inalex.com
LA Kelley Communications
www.kelleycom.com
Lady Bugs Foundation
www.ladybugsfoundation.org
MyGirlsBlood
www.mygirlsblood.org
National Heart, Lung, and Blood Institute (NHLBI) / VWD
http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html
Patient Services Incorporated
www.uneedpsi.org
Plasma Protein Therapeutic Association
www.pptaglobal.org
www.donatingplasma.org
US Health and Human Services/ Office of Women’s Health
www.womenshealth.gov
Victory for Women with Blood Disorders (formerly Project Red Flag)
www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=314&contentid=1564&rptname=women
Women with Rare Bleeding Disorders
www.wrbd.org
World Federation of Hemophilia
www.wfh.org
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Female issues in von Willebrand disease
Menorrhagia
Overview
Women who have von Willebrand disease (VWD) may experience several symptoms. However, for the
majority of women, the presenting and most problematic symptom is menorrhagia. As many as 90% of
women who have VWD suffer from menorrhagia.
 • Menorrhagia is defined as heavy (excessive) menstrual loss.  • Menometrorrhagia is characterized as irregular and excessive menstrual bleeding.
The hallmark of menorrhagia associated with VWD is the onset of heavy bleeding at menarche (first
menstrual period), or during the initial years of menstruation. While there are exceptions, the onset during
menarche helps to distinguish menorrhagia associated with VWD compared to menorrhagia secondary to
other health issues.
Many women who suffer from menorrhagia have to change their sanitary protection very frequently
(sometimes more than once an hour) and/or double up on the protection by using tampons and pads at the
same time. The reason for this is the amount of blood that is lost during menstruation. On average, the
typical woman loses 35-40 milliliters (2-3 tablespoons) each cycle, in comparison to a woman who has
menorrhagia who loses more than 80 milliliters (more than 6 tablespoons) during her cycle.
Another important clinical characteristic of menorrhagia is impaired quality of life. The heavy bleeding may
stain clothing, which can result in avoidance of certain activities during menstruation. These problematic
symptoms have been shown to greatly impact the woman with menorrhagia’s quality of life and life
perceptions. For example, attending school or work regularly may be complicated during menstruation,
as it is difficult to accommodate enough bathroom breaks. The same is true with participating in leisure
activities.
Treatment
Treatment options for menorrhagia vary from one individual to another. Please keep in mind that all
menorrhagia in women with VWD may not be caused by their bleeding disorder. The hematologist will
work with the woman’s gynecologist to determine the best treatment option.
 • Hormonal treatments to help control menstrual bleeding may include, use of oral contraceptives (birth control pills), progesterone only therapy, and the levonorgestrel intrauterine device.
 • Hemostatic therapies include desmopressin (IV), nasal Stimate®, antifibrinolytic therapy, including tranexamic acid and aminocaproic acid, and factor concentrates.
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 •
 •
Surgical treatments for women that are done with child rearing include endometrial ablation or, in some cases, hysterectomy.
Iron supplements may also be prescribed for women who experience anemia (or in order to prevent
anemia) as a result of the increased blood loss. Signs or symptoms of anemia include fatigue,
shortness of breath, lightheadedness, heart palpitations (racing heart/irregular beat), pallor,
weakness, and in more extreme cases, fainting or chest pain.
So what does this mean for you if you have VWD and menorrhagia?
Pictorial charting/menstrual calendar
Once you are diagnosed with VWD, it is essential to complete a pictorial chart or menstrual flow calendar
each month. Charting is helpful to observe menstrual details such as length of cycle, heaviest bleeding days,
how much sanitary protection used, clots, and other information that may be forgotten if not recorded. An
application for iPhone or iPad-such as Period Tracker Deluxe for $1.99-may be a useful tool to help you chart
your menstrual flow details.
Keeping a menstrual chart and bringing it with you to treatment center visits and OB/GYN visits allows the
practitioner another way to evaluate your cycle and the effectiveness of treatment. It also helps to identify
other trends in your cycle. See sample charts at the end of this section.
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Pain relief during menstruation
Pain relievers
It is important to avoid pain relievers that impair platelet function (interference with proper blood clotting).
Medications such as aspirin (acetylsalicylic acid) or aspirin-containing products have an atiplatelet effect
that interferes with appropriate clotting and therefore should be avoided. Certain over-the-counter (OTC)
medications for PMS also contain aspirin, so they should be avoided. Please see section under Medications
called “Common preparations containing aspirin to avoid.”
Herbal preparations should also be used cautiously because they may interfere with blood clotting. Consulting
your hematologist or treatment center with any questions or concerns before taking a new over-the-counter
medication or preparation is recommended.
Acetaminophen (Tylenol®) does not impair platelet function and is generally recommended, unless it is
contraindicated for other coinciding health issues. Consulting with your individual practitioner again is very
important before taking any new medication.
Foods to avoid during menstruation
Foods that contain wheat and dairy can cause fluid retention and can intensify cramping.
Other foods to avoid during menstruation, as they can intensify symptoms, (irritability and bloating), are
alcohol, caffeine, refined sugars and fatty foods.
If you are taking nasal Stimate® please refer to your VWD care provider regarding fluid restriction
instruction(s).
Foods to enjoy during menstruation
Eat lots of fruits and vegetables and foods high in fiber (especially if you experience constipation or indigestion).
Ideas for dealing with menstrual cramping:
•
•
•
•
•
Increase exercise, even if it means just taking a quick walk.
Avoid caffeine. It constricts blood vessels and increases tension.
Try relaxation techniques (deep breathing or meditation) or splurge on a massage…you’re worth it!
Get enough sleep
Keep a diary or ournal
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Dealing with your feelings during menstruation:
There is an enormous value and benefit in journaling, charting, and making notes
of changes in your menstrual cycle (utilizing the menstrual chart and/or
pictorial blood accounting chart). There are also electronic applications on
your cell phone that can help you. Journaling your feelings can be a great
stress reliever.
Journaling notepaper:
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________________________
___________________________________________________________________
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The following charts are examples of methods to help keep track of your menstrual cycle and symptoms. You
may wish to make copies so that you can maintain your logs long term.
Monthly Menstrual Flow Chart
The numbers 1-8 represent the consecutive days of your menstrual period. For each day,
record the number of pads or tampons you usually use that matches each illustration.
Then check out your final score at the bottom of the chart.
PAD
1
2
3
4
5
6
7
8
TAMPON
1
2
3
4
5
6
7
8
TOTAL SCORE
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Menstruation log
Instructions: you may wish to make copies of this sheet, so that you can maintain your log long term.
Month:
Mon
Tue
Wed
Thu
Fri
Sat
Sun
Number of pads/tampons
used
Need to change pad/tampon
every 30 minutes to 2 hours
on heaviest day (Y/N)
Need to use pad and tampon
together
Presence of clots >1 inch in
diameter
Staining through clothes/
nightclothes
Severe cramps
Anemia (specify amount of
iron therapy/ tansfusion)
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11
My Diary
Year ______________
Menstrual Chart and
Your Symptoms
1
2
3
4
5
6
7
8
9
10
11
12
13
14
15
16
17
18
19
20
21
22
23
24
January
February
March
April
May
June
July
August
September
October
November
December
You can use this menstrual chart for each day of your period as well as for nonmenstrual spotting. To record flow levels, you can devise system such as:
H= Heavy Flow
M=Moderate Flow
L=Light Flow
B=Breakthrough Bleeding
S=Mi-cycle or nonmenstrual spotting
1. Add your own symptoms, and rate whenever they occur. For Example, you might use:
1=Very Mild 2=Mild 3=Moderate 4=Severe 5=Very Severe
Or MI=Mild MO=Moderate SV=Severe
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26
27
28
29
30
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Nosebleeds
Basic information
Nosebleeds can be very common in children and adults who have bleeding disorders. Often a person is diagnosed with the bleeding disorder because they are having
frequent, hard - stop nosebleeds.
Nosebleeds occur for different reasons. One reason is that an area inside the nose has
become irritated. This area then has a hard time healing, especially when breathing
dry air, nose picking, etc. Trauma to the nose can also cause nosebleeds. Or a blood vessel can be close to
the surface and bleed easily from irritation.
There are many risk factors that can cause nosebleeds, including allergies, smoking, and infections, in addition
to dry air and trauma as mentioned earlier. Trying to eliminate any irritation to the lining of the nose is your
best plan.
Your treatment center can tell you when to call them for nosebleeds. Generally, if the nosebleed lasts for 20-30
minutes (despite applying continuous pressure), or if it occurs frequently (2-3 times per day for several days
in a row), you need to contact your hematologist or nurse.
Always sit up or have your child sit up and lean slightly forward during a nosebleed.
This keeps you or your child from swallowing the blood. Apply continuous pressure
by squeezing just below the bridge of the nose. Cold cloths can be put on the back
of the neck or bridge of the nose. If the person who is bleeding can hold ice in his
or her mouth, this will help (but the pressure needs to be continued on the outside
of the nose at the same time).
Some over-the-counter treatments are:
•Afrin® or Neo-Synephrine® nasal spray - one spray into the nostril that’s bleeding
•QR Powder- apply as directed
•Allergy medicine, eg, Claritin®
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For prescription treatment options check with your treatment center.
Some preventative measures are:
•Apply olive oil to the openings of the nose each night and in the morning (use olive oil only, dip a cotton swab into the olive oil and apply)
•Keep air cool at night with a cool mist humidifier
•Increase fluid intake
•Sleep in a cool room
•Use a saline nasal spray 3-4 times a day
•Cover the nose when outside in the winter
•Do not take hot showers right after a nosebleed or after swimming
For more information, contact [email protected] and ask for the Nosebleed Booklet available in English
and Spanish.
Reference: ‘Nosebleeds’, Gue D, Munn J, Purcell S, Zappa S, Copyright 2007, National Hemophilia Foundation. 2
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Patient Notification System
The Patient Notification System is a free, confidential, 24 hour communication system providing information on
factor concentrate therapy withdrawals and recalls.
The system was created to provide consumers with a single, convenient, and confidential source for up-todate withdrawal and recall information. Led by the Plasma Protein Therapeutics Association (PPTA), the Patient
Notification System was developed by the manufacturers of plasma therapies with direct input from consumers.
Everyone should register with the Patient Notification System so they can be directly notified when adverse
conditions occur with blood products.
The system is being administered by the National Notification Center. The identity of those individuals who have
registered is completely confidential.
Each person who registers with the Patient Notification System will select how they wish to be notified—
telephone, express delivery letter, fax or e-mail. All participants will then receive a follow-up first-class letter
back-up. Participants can also select which products they wish to be notified about.
The system is available by telephone for updates and news at 800-UPDATE-U. There is also a Web site at
www.patientnotificationsystem.org
Register now!
• By phone: 888-873-2838 (888-UPDATE-U)
• Online Enrollment: www.patientnotificationsystem.org
• Fax registration forms to: 1-800-442-2906
• Mail registration forms to:
National Notification Center
20 N. Meridian Street, Suite 300
Indianapolis, IN 46204.
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Medications
Plasma safety
Because VWD treatment products sourced from human blood have been responsible for transmitting bloodborne infectious agents (such as HIV and hepatitis) in the past, it is very important to ensure that products being
considered for use are safe and free from viral infection. Since the 1980s, manufacturers and the agencies
regulating the manufacture of fractionated plasma products have responded to concerns about transmission
of blood-borne viruses by developing a comprehensive set of measures designed to reduce, if not eliminate,
infectious risk. These measures are based on the following principles:
1) Selection of appropriate blood and plasma donors
2) Screening of the plasma raw material with laboratory tests
3) Elimination of any potential contaminating viruses through the manufacturing process
Of these three principles, the elimination of potential viruses through the manufacturing process has enhanced
the safety of VWD treatment products the most.
Measures to enhance the viral safety of plasma products include:
• Selection procedures which ensure that donors with high-risk behavior are excluded
• Mandatory serological testing on all plasma donations for HIV, hepatitis B, and hepatitis C
• Plasma inventory hold and exclusion based on post-donation information
• Nucleic acid testing (NAT) of minipools for HCV-RNA (and increasingly for other viruses • Testing starting-manufacturing plasma pool samples for viral markers and viral genomic material
• Inclusion of one or more validated specific viral inactivation and/or removal steps in the including HIV, HBV, B19, and HAV) and exclusion of reactive donations
manufacturing process
• Full traceability of plasma from donors to end products
In addition, some agencies and manufacturers also test finished products for viral markers and genomic
material.
The combination of appropriate donor selection procedures, screening with the current generation of standard
serological tests, and, in particular, the inclusion of measures to inactivate or remove potential viruses has
made fractionated plasma products free from serious known blood-borne viruses such as HIV, HBV and HCV. Fractionated plasma products manufactured by today’s processes, and manufactured with attention to good
manufacturing practices (GMPs), are among the lowest risk therapeutic products in use today.
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Female issues in von Willebrand disease (VWD)
Pregnancy and post partum
Pregnancy and VWD
Pregnancy can offer a new set of challenges for women with VWD. Thus, planning for pregnancy should begin
before conception if possible. This planning should include consulting with a hematologist and an obstetrician.
In cases where a severe form of VWD might be inherited, it is also important to meet with a genetic counselor
prior to conception or during pregnancy to review the genetics and inheritance of VWD. The hemophilia treatment
center (HTC) will have a counselor who is familiar with VWD on staff or available by referral.
Each type of VWD has a unique response to changes in the body during pregnancy. It is important that each
woman be evaluated by an HTC to determine her VWD type. Then, based on the typing and baseline factor
levels, HTC professionals and the obstetrician will make a plan for the pregnancy, delivery and post-partum
periods. von Willebrand factor (VWF) levels may rise during pregnancy, and it is important to know levels prior
to delivery.
Your hematologist should be involved as soon as you learn you are pregnant. There are several things that will
need to be discussed regarding plans for delivery. These items include:
•Laboratory blood work to check VWF levels
•The different hemostatic agents that may need to be used to prevent or treat bleeding
•Discussion of the plan for any invasive procedures during pregnancy. You should also discuss with your hematologist and obstetrician:
•Modes of delivery (vaginal vs cesarean)
•Use of different types of anesthesia
•Other details of delivery. 1
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Women with Type 1 VWD
•Factor 8 and VWF protein usually rise and normalize during pregnancy
•You may not require any factor support or desmopressin (DDAVP®) during the time around the baby’s birth
•Epidural anesthesia may be used without treatment if clotting factor levels have been tested during pregnancy and are known to be in the normal range
•DDAVP is not generally given during active labor and delivery
•Women with severe Type 1 VWD will not have an increase in VWF levels that is sufficient to deliver or have epidural anesthesia and will require FVIII/VWF concentrate for safe delivery
•This subtype has a defect in the quality of the VWF protein. Even though the levels of these proteins increase they will not be functional.
•Most women will likely require factor concentrate during the time of delivery or with any complication of pregnancy that results in bleeding
•These women will not have an increase in VWF levels that is sufficient to deliver or have epidural anesthesia
•These women will require FVIII/VWF concentrate for safe delivery
Post partum
Your hematologist needs to be aware of your delivery. This is because women with VWD are at an increased
risk for postpartum hemorrhage (PPH), particularly delayed PPH. This can occur from the date of delivery to
several weeks after delivery. PPH happens when the uterus does not contract quickly and completely enough
after vaginal delivery to slow or stop bleeding, and your factor levels have returned to your prepregnancy
baseline state.
If you have experienced excessive bleeding, your hematologist or treatment center may have you come in to
have a hemoglobin level and/or VWF level checked.
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Postpartum facts in women with VWD
•All women bleed after delivery for 21 - 27 days
•Factor levels in women with VWD remain elevated for a period of time (7 - 21 days following delivery)
•Women with VWD are vulnerable to hemorrhage after factor levels fall, usually around day 15
Additional facts
•For women who undergo a D&C (dilation and curettage) or have a miscarriage, a plan of treatment will need to be made and will require close follow-up by their hematology/OB team
•If there is a possibility that the infant will have a severe form of VWD, a pediatric hematologist should be consulted prior to delivery to arrange a plan for evaluation and neonatal testing
•Because of the possible need for a blood transfusion during or after delivery, women with VWD who have not already been vaccinated should be immunized against hepatitis A and hepatitis B
References
Lee CA, Kadir RA, Kouides PA. Inherited bleeding disorders in women. West Sussex: Wiley-Blackwell; 2009.
National Heart Lung and Blood Institute. The diagnosis, evaluation and management of von Willebrand disease.
National Institutes of Health publication 08-5832; US Bureau of Printing: Washington, DC 2007. Also found at:
http://www.nhlbi.nih.gov/guidelines/vwd/.
Demers C, Derzko C, David M, Douglas J. Gynaecological and Obstetric Management of Women With Inherited
Bleeding Disorders. JOGC No.163, July 2005; 707-718.
3
FlashLINK to VWD
Role of Hemophilia Treatment Center (HTC)
Having a chronic disease means spending a lot of time and energy negotiating the healthcare system. The
hematologists, nurses, psychosocial professionals, and physical therapists not only help consumers with
their medical care issues, but also lend tremendous emotional support. Many people with bleeding disorders,
including von Willebrand disease (VWD), use the resources of their hemophilia treatment center (HTC), also
called treatment centers, for many years because the staff understands their unique needs.
Individuals who go to hemophilia treatment centers will not only find state-of the art medical care but will
also benefit from an experienced, caring staff that takes time to develop comprehensive treatment care plans
for patients and families.
Hemophilia treatment centers are located in cities across the United States.
To find the one closest to you, go to the CDC website at https://www2a.cdc.gov/ncbddd/htcweb/Main.asp
The treatment center not only provides specialty care but can also act as a resource to your regular family
physician or dentist.
Members of the care team at HTCs include:
• Hematologists—specialists in blood disorders.
• Pediatricians—specialists in caring for infants, young children, and teenagers
• Nurses—specialists in bleeding disorders. The nurse is probably the person you will see most frequently.
• Social Workers—specialists who assist you with the
issues of daily living, such as adjusting to your
bleeding disorder and locating resources (eg,
insurance, transportation, housing, etc).
• Physical therapists—specialists in activity, exercise, and rehabilitation.
• Orthopedists—specialists in disorders of the bones and joints.
• Dentists—specialists in disorders of the teeth and gums. The dentists at HTCs are experts in
treating children with oral bleeding problems.
• You are also an important member of the treatment team. The staff needs your input to develop a
plan of care that will ensure you remain healthy, active, and able to live successfully
with the added challenge of a bleeding disorder.
FlashLINK to VWD
Sample travel letter
(On HTC letterhead)
Please notify your HTC at least 2 weeks before travel to get your customized Travel Letter.
D
I
O
Patient Name:_____________________________________________________________________
DOB:____________________________________________________________________________
Diagnosis:_____________________ Baseline Levels: VWF Activity ____________________________
VWF Antigen ____________________________
Factor VIII ______________________________
V
____________________________ has a diagnosis of _____________________________________
von Willebrand Disease. He/She will be traveling with medications and/or factor products to treat his/her
disorder. The products need to be kept in carry-on luggage.
His/her current treatment plan is as follows:
Nosebleeds: _______________________________________________________________
Menorrhagia: _______________________________________________________________
Minor bleeds: _______________________________________________________________
Major bleeds: _______________________________________________________________
Prophylactic dose: ____________________________________________________________
Please contact the HTC at ______________________________________ with any questions or concerns.
FlashLINK to VWD
Sports ratings by risk
1
Safe
1.5
Safe to Moderate
2
Moderate
2.5
Moderate-Dangerous
3
Dangerous
Archery
Aquatics
Elliptical machine
Stationary bike
Fishing
Frisbee
Golf
Hiking
Snorkeling
Swimming
Tai Chi
Walking
Bicycling
Rowing machine
Treadmill
Circuit training
Body sculpting
Physio-ball
Spinning
Frisbee golf
Pilates
Weightlifting/
resistance training
Ski mach.
Aerobics
Bowling
Stepper
Dance
Diving
Cardio kick boxing
Ultimate Frisbee
Jumping rope
Rock climbing (indoor)
Rollerskating
Rowing/crew
Running/jogging
Skiing-Cross Country
T-ball
Tennis
Yoga
Baseball
Basketball
Canoeing
Cheerleading
Gymnastics/
track & field
Horseback riding
Ice skating/
In-line skating
Water skiing
Jet skiing
Kayaking
Karate/
Tae Kwon Do
Mountain biking
Racquetball
River rafting
Scooter (non motorized)
Scuba diving
Skate boarding
Skiing/downhill/
telemark
Surfing
Snowboarding
Soccer
Softball
Volleyball
BMX racing
Boxing
Diving/comp.
Football
Hockey-all
Lacrosse
Rodeo
Rugby
Motorcycling/motor
cross racing
Power Lifting
Rock climbing-natural
Scooter-motorized
Wrestling
NOTE: Trampoline is NOT recommended for ANY child ever.
References: Anderson, A, Forsyth, A. Playing it Safe, NHF 2005 and the American Academy of Pediatrics.
FlashLINK to VWD
Surgery and von Willebrand disease
Persons with von Willebrand disease (VWD) are not at any higher risk of needing surgery than the general
population. They do require treatment to prevent bleeding before most surgical procedures as well as invasive
procedures (eg. colonoscopy, breast biopsy, and endoscopy), and dental work. The treatment prescribed is
dependent on the type of surgery as well as the type of VWD.
The best thing to do when planning a surgery of any type is to contact your von Willebrand care provider for
recommendations. It is your responsibility to contact your treatment center. This should be done well in advance (2-3 weeks) to allow time to arrange for necessary treatment prior to your surgery.
FlashLINK to VWD
Medications
Tranexamic acid (Cyklokapron® or Lysteda®)
Tranexamic acid is an antifibrinolytic agent (that is, a medicine that slows the breakdown of blood clots). It can
help keep bleeding in the mouth and mucous membranes from starting up again, once it has stopped. It is often
used along with factor or DDAVP® to treat bleeding with surgeries in the mouth and nose, dental procedures
and/or mouth injury.
• Cyklokapron® (tranexamic acid) tablets are currently not available for sale in the United States. They may be available through Canadian Pharmacy websites. Speak with your hematology team about how to obtain this medication.
• Cyklokapron® is available for use in the IV form. Your doctor might want to use this form of the medication when you or your child is in the hospital.
• Bulk powder of the generic form of Cyklokapron® (tranexamic acid) is available in the US. Your specialty pharmacy may be able to obtain it for compounding into an oral liquid medicine for you.
• Cyklokapron® is usually given every 8 hours. It is important to take the medicine on a scheduled basis around the clock, as the doctor prescribes it.
• NEVER take Cyklokapron® (or give it to your child) for blood in the urine. Report any blood in the urine to your hematology team.
• Lysteda® (tranexamic acid) tablets (650 mg) were approved by the FDA for sale in the United States in December 2009 to treat menorrhagia. This is the same agent, tranexamic acid, as contained in Cyklokapron® tablets.
Special Instructions:
• Can be taken with or without food. Give with food if you or your child have an upset stomach after
taking the medication.
1
FlashLINK to VWD
• If you or your child misses a dose of the medication, take it as soon as you remember. Do not try to “make up” a missed dose.
• Do not use for kidney or bladder bleeding (blood in urine) unless directed by your doctor.
• Should be used with caution by people with kidney or liver disease.
Side Effects (do not need to be reported):
Mild upset stomach
Mild diarrhea
Mild headache
Side effects that must be reported:
Skin rash
Chest pain
Slurred speech
Vomiting
Visual changes
Tell your doctor of other medications, vitamins, and herbal products that you or your child is taking.
Keep the medication in its original container and store as instructed.
Call 911 or go to the Emergency Room if you or your child has:
Chest pain
Slurred speech
2
FlashLINK to VWD
Universal Data Collection or UDC
Today, clotting factor concentrates are considered safe from contamination with known viruses. However, continued
monitoring of blood products provides an additional measure to ensure their safety. With this in mind, the Centers
for Disease Control and Prevention (CDC), in cooperation with federally funded hemophilia treatment centers (HTCs),
established the Universal Data Collection (UDC) project.
Hemophilia Treatment Centers (HTCs) across the US are helping the Centers for Disease Control and Prevention (CDC)
to learn about how to prevent joint damage from hemophilia, and they are also studying possible viral contamination
of factor products. This study will help doctors and nurses to find out what treatments are best.
How the UDC study works:
• Every year, at the Comprehensive Visit, you will be asked if you want to participate in the UDC
“study.” Someone who can answer your questions will ask you to read and sign a paper called an “Informed Consent.” That will give the HTC permission to ask the questions needed, collect the lab specimens needed, and send all of it to the CDC in Atlanta
• The information that you give will go to the CDC with a number in place of your child’s name - the only thing that the CDC will know is what HTC you are attending
• You can refuse if you want- it is your right and nothing negative will happen
• The information is used to help the treatment and care of those with bleeding disorders
• When your child is less than 2 years old, only very basic information is asked
• When your child is more than 2 years old
o The physical therapist will measure his joints to see if there has been any change in how well he can bend or straighten each joint. This will take some time, but it doesn’t hurt at all
o Questions will be asked about treatment and how many bleeds your child has had in 6 months
o Blood will be drawn and the specimens sent to the CDC for testing for Hepatitis A, B, C and
HIV- this is done for free
o Some of the blood specimen is stored at the CDC, in case further testing is needed in the future
Your HTC will answer all your questions.
If you give permission for your child to be in the study, you will be given a copy of the informed consent that you
signed. This will give you more information and whom to call if you ever have a question or concern about the study.
This paper will also help remind you of what we mean by the “UDC study” or the “CDC study.”
FlashLINK to VWD
Emergency Care for Patients
with von Willebrand Disease
A n i n s t r u c t i o n al m a nu al fo r M e d i c al P ro fe s s i o n al s
Authors and Editors: Susan C. Zappa RN, Lucie Lacasse RN, Rose Jacobson RN, Sherry Purcell RN and Karen Wulff RN
Medical Reviewers: David Lillicrap MD, FRCPC and Marcela Torres MD
von Willebrand Disease (VWD) is
classified by 'type 1, 2, or 3' If the
type is unknown proceed as if type 1,
if bleeding continues consult a
hematologist.
Mucous membrane
bleeds (pg. 6)
Administer the
recommended
treatment and
anti-fibrinolytics
Gynecological bleeds (pg. 12)
Immobilizers
p.r.n. for joint bleeds
Abdominal bleeds (pg. 10)
Trauma (pg. 23)
Administer the recommended
treatment
Ice pack
for soft tissue, muscle,
joint bleeds
For VWD type 3: Avoid intramuscular injections due to the
possibility of causing a muscle
bleed
Minor cuts / bruises
no treatment
Head Injury (pg. 4)
Always treat
immediately with
the recommended
treatment
Treatment and Management Guidelines for von Willebrand Disease
Type of von
Willebrand
Disease
Type 1 or Type 2
The type 2 VWD
known as ‘pseudo
VWD or VWF
platelet type' will
only respond
to a platelet
transfusion - call
a hematologist.
Major life-threatening bleeds
(ex. - head injury, GI bleeding, severe
menorrhagia, etc.)
Factor concentrate containing both
FVIII (8) and von Willebrand factor
(eg. Humate P®, Alphanate® , wilate®):
60-80 Ristocetin cofactor units/kg IV
Package insert will instruct as to rate per
volume.
Note: monoclonal or recombinant
factor VIII (8) products do NOT have
von Willebrand factor in them and
will not stop the bleeding.
Other bleeds
(ex. - sutures, nosebleed, mouth bleed, dental
extractions etc.)
Known to respond to desmopressin
(DDAVP®):
Desmopressin 0.3 mcg/kg IV in 50 ml of
Normal Saline over 30 minutes or
subcutaneously if volume can be given
safely. Recommendation: a maximum dose
of 20 mcg.
Mucosal bleeding - anti-fibrinolytics (pg. 9)
For patients who do not respond to desmopressin:
Give a factor concentrate containing both
(eg. Humate P®, Alphanate®, wilate® ):
40-60 Ristocetin cofactor units/kg IV
Package insert will instruct as to rate per volume.
Note: monoclonal or recombinant factor
VIII (8) products do NOT have von Willebrand factor in them and will not stop
the bleeding.
Type 3
Most severe form
of VWD.
60-80 Ristocetin cofactor units/kg
40-60 Ristocetin cofactor units/kg
Package insert will instruct as to rate per
volume.
Package insert will instruct as to rate per volume.
Note: monoclonal or recombinant
factor VIII (8) products do NOT
have von Willebrand factor in them
and will not stop the bleeding.
Note: monoclonal or recombinant factor
VIII (8) products do NOT have von Willebrand factor in them and will not stop the
bleeding.
Dose Chart
If your institution does not have Humate-P®, or Alphanate®, wilate®, but does have Koate DVI® available,
consult a hematologist for guidelines and instructions.
Per the Medical and Scientific Advisory Council of the National Hemophilia Foundation:
Because of the increased risk of HIV and hepatitis A, B, and C transmission, cryoprecipitate should not be used (for the treatment of von Willebrand
Disease) except in an emergency situation where one of the above products is not available and delay of treatment would be life or limb threatening.
Centers
Content
Table of Contents
Intro
Basics
Emergency Care for Patients
with von Willebrand Disease
Head
2
Head Injury
4
Mucous Membrane Bleeding
6
14
Desmopressin Acetate
(DDAVP®, Stimate® and Octostim®)
16
Factor Medications & Administration
18
Other Medications
19
Invasive Procedures / Labs / X-rays
20
Trauma / Emergencies
22
Bibliography / References
23
Factor
Meds
Joint /Muscle / Soft Tissue Bleeding
Desmopressin
12
Joint / Muscle
Soft Tissue
Gynecological Bleeding
Gyn.
10
GI / GU
GI/Urinary Tract Bleeding
Mucous
Membr.
Introduction / von Willebrand Disease Basics
Labs
X-rays
Copyright © 2006, Updated January 2011 Susan C. Zappa, RN, CPN, CPON, Cook Children's Medical Center;
Lucie Lacasse, RN, BScN, The Ottawa Hospital; Rose Jacobson, RN , Winnipeg, Health Sciences Centre; Sherry L.
Purcell, RN, Kingston General Hospital; Karen Wulff, RN, Tulane University School of Medicine.
All rights reserved. Permission granted to photocopy for educational purposes only. For further information or
inquiries, contact Susan Zappa at Cook Children's Medical Center, [email protected] (682) 885-2587.
1
Intro
Centers
Basics
Content
Introduction & von Willebrand Disease Basics
Head
Purpose
Mucous
Membr.
This manual contributes to von Willebrand Disease (VWD) care by enhancing the emergency department personnel’s
understanding of this disorder and its treatment. The goals of this manual are to:
•promoteunderstandingof thecomplexitiesof vonWillebrandDiseasewithanemphasisonrapidtreatmentfor
correction of the hemostatic abnormality
•provideareferencefortheemergencycenterstaff
•promoteaconsultativedialoguewiththeemergencydepartment(ED),treatmentcenter,andpatient/family
GI / GU
Early triage and treatment reduce morbidity.
Use
Gyn.
This manual provides a standardized format for evaluation and treatment of VWD emergencies. The content is segmented
by systems and complications of VWD. Turn to an area of interest. The illustration on the left page provides information
points for quick review. The text on the right page gives further detail of bleeding presentations, their possible complications and treatment. The treatment varies to the type and the severity of VWD. Treatment and management information is
provided on the inside cover of the manual as a reference.
Joint / Muscle
Soft Tissue
It is suggested that the patient’s treatment center or hematologist be consulted for final management of bleeding complications.
To The Attending Medical Staff
Desmopressin
This manual is a guide for medical personnel who may be less familiar with VWD treatment. The content consists of
guidelines, recommendations and suggestions only. The attending physician has the final responsibility for appropriate
diagnosis and treatment.
Definition
von Willebrand Disease is an autosomally-inherited bleeding disorder caused by the quantitative deficiency or dysfunction of von
Willebrand factor, a large multimeric glycoprotein. It is non-sex linked. Therefore, it can occur equally in both men and women.
Factor
Meds
Effects of von Willebrand Disease
von Willebrand factor is essential for platelet-plug formation as an adhesion protein that diverts circulating platelets to the sites of
vascular injury, particularly through larger multimers. It also forms a non-covalent complex with coagulation factor VIII in plasma,
thereby protecting it from inactivation and clearance.
Labs
X-rays
Even though the primary deficiency or defect in von Willebrand Disease is that of von Willebrand factor, the secondary deficiency
of factor FVIII, which is dependent on von Willebrand factor as its naturally occurring plasma carrier and stabilizer, leads to a
defect both in platelet-plug formation and in fibrin formation.
Prevalence
Trauma
Bibliography
The prevalence is as high as 1 to 2 percent in the general population.
2
Centers
Content
Introduction & von Willebrand Disease Basics
Types of von Willebrand Disease
Intro
Basics
The type of VWD determines the treatment - see inside of the front cover for treatment options. von Willebrand Disease
is classified by ‘type 1,2 or 3’. If the type is unknown, proceed as if type 1. If bleeding continues contact a hematologist.
von Willebrand Disease is classified into three main phenotypes and each have subtypes based on the quantity and quality of the von
Willebrand factor (VWF):
Head
• Type1:whichaccountsfor60to80percentof cases,resultsfromadecreasedproductionof normalvonWillebrandfactor
and factor VIII; typically transmitted as an autosomal dominant trait in the heterozygous state.
Mucous
Membr.
• Type2:whichaccountsfor10to30percentof casesischaracterizedbyqualitativeabnormalitiesof vonWillebrandfactor
and is further divided into subtypes 2A, 2B, 2M and 2N. Inheritance is generally autosomal dominant.
GI / GU
• Type3:Accountsfor1to5percentof casesandistransmittedasanautosomalrecessivetraitinhomozygousorcompound
heterozygous persons. This severe form of the disease is characterized by a very low or undetectable von Willebrand factor in
plasma with a low, usually detectable factor VIII activity. It is in these rare cases of type 3 (1 in 1 million people) that
symptoms are more frequent and severe, similar to those cases of severe hemophilia.
Acquired von Willebrand Disease: This is an acquired syndrome that resembles von Willebrand Disease in its clinical manifestation
and laboratory patterns. It occurs in rare instances in association with clinical conditions such as lymphoproliferative and autoimmune diseases, hypothyroidism, essential thrombocythemia, cancer, Wilm’s tumor and valvular heart disease.
Gyn.
Bleeding episodes
Joint / Muscle
Soft Tissue
The hallmark of von Willebrand Disease is mucosal bleeding. Mucous membrane bleeds such as bleeding from the nose, mouth,
gastrointestinal tract, genitourinary and vaginal bleeding are the most common. If left untreated, these mucous membrane bleeds
can become acute and sometimes life-threatening emergencies. Serious bleeding resulting from untreated trauma and/or post-surgical bleeding can also become life or limb-threatening in these patients.
Serious bleeding sites
The major sites of serious bleeding which threaten life, limb, or function are:
•oropharynx
•vaginalbleeding
•ocular
•spinalcord
•gastrointestinal
•intra-abdominal
Desmopressin
•intracranial
Treatment
Factor
Meds
The mainstay of treatment is the replacement of the deficient/defective protein at the time of bleeding or before invasive procedures are performed. This may require desmopressin (subcutaneous, intranasal, or intravenous) or an infusion of commercial von
Willebrand factor/FVIII concentrate such as Humate-P®, Alphanate® and wilate®. Specific doses, additional drugs and medical
interventions depend upon the type of VWD and the site and severity of bleeding. Please refer to the inside cover of the manual
for more detailed information on the recommended treatment. Once treatment has been given, emergency diagnostic procedures
can begin.
Family
Labs
X-rays
Patients living with VWD or their parents are often knowledgeable about the management of their disorder and their input
should be sought and heeded. Interview the family about whether any medication has been administered prior to arriving at the
ED; if so, determine when and what dose. Additional treatment may be required, dependent on the time lag and severity of the
bleed. Determine the treating hematologist or treatment center, and contact them for assistance and follow-up as needed.
3
Centers
Content
Head Injury
Mucous
Membr.
Head
Intracranial hemorrhage (ICH) is a
potential for all head injuries.
GI / GU
Administer recommended
treatment first*, and then
perform diagnostic studies
such as CT scan.
Desmopressin
Joint / Muscle
Soft Tissue
Gyn.
If an ICH is diagnosed, the
patient should be admitted
and the hematologist
contacted immediately. If no
ICH is diagnosed, the patient
may be discharged.
Factor
Meds
Discharge Instructions
Labs
X-rays
Call the treatment center or the
patient’s hematologist for follow-up
treatment recommendations*.
Trauma
Bibliography
Report any signs or symptoms of an
ICH to the treatment center or the
patient’s hematologist.
Head injury instructions should
be given for a two week period
(instead of the usual instructions for
24 - 48 hour period).
4
Recommended treatment table inside front cover
*
Intro
Basics
Intracranial hemorrhage (ICH) is a potential risk for individuals with von Willebrand Disease, and is most
commonly associated with injury. The risk of intracranial hemorrhage is increased with the more severe
types of VWD. Without early recognition and treatment, death or severe neurologic impairment can occur.
Early neurologic symptoms may not always be evident.
Centers
Content
Head Injury
Head
Treatment
Mucous
Membr.
All significant head trauma, with or without hematoma, should be treated promptly with the appropriate
treatment* before any diagnostic tests. A hematologist should be contacted.
Diagnostic imaging
GI / GU
Obtain an emergency CT scan to rule out ICH after the appropriate treatment* has been given. Notify the
patient’s hematologist or treatment center as soon as possible.
Possible admission
Desmopressin
If the patient is discharged home, instruct the family to monitor the patient for signs and symptoms of
neurologic deterioration and report any abnormalities to the hematologist. Consult the treatment center to
arrange for follow-up treatment if the patient is discharged home from the emergency department.
Joint / Muscle
Soft Tissue
Instructions
Gyn.
The patient should be admitted to the hospital for observation if he/she has suffered a severe blow to
the head or exhibits any neurologic symptoms. Symptoms can include headache with increasing severity,
irritability, vomiting, seizures, vision problems, focal neurologic deficits, stiff neck, or changes in level of
consciousness. Patients with a past history of ICH are at increased risk of repeated head bleeds.
Factor
Meds
Labs
X-rays
Recommended treatment table inside front cover 5
*
Centers
Content
GI / GU
Mucous
Membr.
Head
A Dental or E.N.T. consult may be needed.
Joint / Muscle
Soft Tissue
Gyn.
Nose bleeds may respond
to other measures. Refer to
“Controlling Epistaxis” table on
pg. 8.
Factor
Meds
Desmopressin
Mouth bleeds (gum, tooth, frenulum
or tongue laceration) may require
treatment * and the use of antiIf the bleeding is minor,
local measures such as topical thrombin
(if available) or fibrin glue in conjunction
with anti-fibrinolytics can be used. Refer
to the anti-fibrinolytics on pg. 9 .
Labs
X-rays
Trauma
Bibliography
Patients should follow-up
with their treatment center or
hematologist the next day.
6
*
Assess for anemia if there has been
prolonged mucosal bleeding.
Instruct the patient on how to
control epistaxis, the use of antifibrinolytics, and the importance
of a modified diet. Consult the
Diet Modifications table on pg. 9 as
needed.
•havesustainedaknowninjurytothemouth,tongue,ornose
•havesevereswellinginthemouthorthroatarea
•areexperiencingrespiratorydistress
•havedifficultyswallowingorspeaking
Mucous
Membr.
•areexperiencingprofuseand/orprolongedbleeding
Head
Intro
Basics
Mucous membrane bleeding may require medical care in the emergency department. Treatment may be required
for patients who:
Centers
Content
The patient may not know the reason for the symptom or bleeding. It may have been caused by trauma, infection, or the bleed may be spontaneous. If airway blockage is suspected, prompt treatment* is required prior to
any invasive procedures.
GI / GU
Remember prompt treatment will greatly reduce the bleeding, often preventing serious complications. The longer the patient waits, the more bleeding takes place. If the bleed is in a closed space, the accumulation of blood will cause surrounding tissue damage, airway obstruction and pain.
Gyn.
Epistaxis
Joint / Muscle
Soft Tissue
Uncontrolled epistaxis may require treatment in conjunction with anti-fibrolytics. Be sure the patient knows
how to control and stop the bleeding (see pg. 8)
Oral Cavity
Desmopressin
Bleeding in the mouth can be hard to control. A frenulum or tongue laceration may respond to topical thrombin
or other similar agents. If the bleed continues, the patient will probably need further treatment. A single treatment may temporarily stop the bleeding, but clot lysis from saliva enzymes often results in re-bleeding. Rebleeding is most commonly seen on days 3-5. An anti-fibrinolytic may be indicated to maintain hemostasis. A
modified diet should be started at the same time as treatment (see Diet Modifications pg. 9).
Factor
Meds
Bleeding may occur with extracted, erupting or exfoliating teeth. It is more common with extracted and exfoliating teeth. A dental consult may be needed to extract the tooth. Treatment* to increase the von Willebrand
factor will be necessary prior to extraction. A frenulum or tongue laceration will require treatment*.
Retropharyngeal
Labs
X-rays
After the recommended treatment*, further observation, X-rays and admission may be required depending upon
the specific circumstance.
*
7
Centers
Content
Mucous
Membr.
Head
Controlling Epistaxis
Instruct the patient:
Mucous
GI / GU
Membr.
1. To gently blow his/her nose to remove mucus and unstable
clots that will interfere with hemostasis.
2. Tilt the head forward so any blood will come out the nares and not down the back of
the throat.
Gyn.
3. Apply firm constant pressure to the entire side of the nose that is bleeding for 15
minutes.
Joint / Muscle
Soft Tissue
4. Release the pressure to see if bleeding has stopped, gently blow out and remove any
soft clots.
5. If the bleeding continues, reapply pressure for another five minutes.
Desmopressin
6. Recommended treatment* and/or anti-fibrinolytic agents (see next page) may be
needed.
Factor
Meds
7. During active bleeding, NoseBleed QR® powder
an over-the counter
preparation can be utilized. The powder needs to be mixed with blood, as per the
manufacturer's directions. The powder will solidify the blood, form a crust and bleeding
may stop.
8. During active bleeding, or when the bleeding has stopped, you may spray or apply
two drops of oxymetazoline (eg. NeoSynephrine®, Dristan® or Afrin®) nasal spray/drops
to the side that was bleeding. These can be used at home PRN for epistaxis.
Trauma
Bibliography
Labs
X-rays
9. Instruct the patient to use mucosal membrane moisturizer (eg. Vaseline®, Secaris®) in
the nares to keep the membranes soft and moist, and prevent the formation of hard
crusts which might crack and restart bleeding. Adequate humidification in the home is
also helpful.
10. An Ear Nose Throat (ENT) consult may be required for possible cauterization of a
vessel.
8
Intro
Basics
Anti-Fibrinolytics
Anti-fibrinolytics may be indicated in nasal or oral bleeding. Amicar® and
Cyklokapron® are both anti-fibrinolytic agents. Either may be prescribed for
mucous membrane bleeding to promote clot stabilization in conjunction with
the recommended treatment*. In some cases they may be prescribed
independently.
Head
Mucous
Membr.
Amicar - epsilon aminocaproic acid
Recommended dosage:
Child: oral dose 50-100 mg/kg (not to exceed 4 g) every 6 hours for 3 - 10 days
Adult: oral dose 3-4 g every 6 hours for 3 -10 days
Supplied: Tablet: 500 mg or 1000 mg per tab
Syrup: 250 mg per ml
Injectable: 250 mg /ml available in 20 ml vial
*
Contraindicated if hematuria present
GI / GU
Gyn.
Cyklokapron - tranexamic acid
Recommended dosage:
Child: oral dose 25 mg/kg every 6- 8 hours for 3 - 10 days
Adult dose: oral dose 1000 mg-1500 mg tid for 3 - 10 days
Supplied: Tablet: 500 mg tranexamic acid per tab
Injectable: 100 mg/ml available in 5 and 10 ml ampules
*
Contraindicated if hematuria present
Desmopressin
Follow-up care per the treatment center or patient’s hematologist. Topical agents such as topical
Thrombin® and Gelfoam® may also be used to help control mucous membrane bleeding.
Diet Modifications
Directions for the patient:
Factor
Meds
1. Diet should be restricted to soft, cool, or lukewarm foods until the area is fully
healed. Suggested foods: flavored gelatin, non-carbonated drinks, sherbet,
lukewarm soups (no cream soups), baby foods, blenderized or pureed foods,
pasta.
Labs
X-rays
2. Avoid using a straw, chewing gum, and do not smoke. Negative pressure
from the sucking action can dislodge the clot and aggravate the bleeding site.
3. Foods to avoid include hard foods like chips, nuts, popcorn, tacos, etc.
4. If Desmopressin (DDAVP®, Octostim®, or Stimate®) has been utilized for treatment,
the patient has fluid restrictions for 24 hours.
Joint / Muscle
Soft Tissue
These medications must be given as ordered to keep blood levels constant. They are not readily
available through local pharmacies (they must be ordered). If possible, dispense the amount for
2-3 days from the hospital pharmacy to allow time for the local pharmacy to order. Other options
are the family’s home supply, bleeding disorders treatment center or (U.S.) home care companies.
*
Centers
Content
9
Nausea and vomiting may indicate
intracranial hemorrhage as well as
gastrointestinal problems.
Joint / Muscle
Soft Tissue
Gyn.
GI / GU
Mucous
Membr.
Head
Centers
Content
Gastrointestinal / Urinary Tract Bleeding
Hematuria
- bed rest for 24 hours
- force fluids
- consult the treatment center or
the patient’s hematologist
- avoid anti-fibrinolytics
Labs
X-rays
Factor
Meds
Desmopressin
Iliopsoas bleeding
- flexed hip
- pain on extension
- Management: Treatment
product* as per
hematologist
Abdominal pain
Treat immediately* as per
hematologist for:
- flank pain
- melena
- vomiting blood
Trauma
Bibliography
- increase fluids
- rest
- no heavy lifting
10
- report any symptoms such
as fever, pain, or increased
hematuria, melena,
hematemesis
*
- follow-up with the
treatment center or the
patient’s hematologist
Centers
Content
Gastrointestinal / Urinary Tract Bleeding
Intro
Basics
Initial presentation
Acute abdominal pain in a patient with von Willebrand Disease may have many origins, such as gastrointestinal
(GI) tract hematomas (both spontaneous or trauma induced), iliopsoas or retroperitoneal bleeding.
Head
Bleeding may also occur with hemorrhoids or the passage of kidney stones. Notify the treatment center or the
patient’s hematologist.
Mucous
Membr.
Patients who present to the emergency department with abdominal or flank pain, melena or hematemesis should
be triaged for immediate examination and the recommended treatment* should be initiated. Once this is done,
then diagnostic x-rays, scans and endoscopy procedures can be carried out.
GI / GU
Abdominal trauma and benign events such as forceful coughing or vomiting can precipitate an abdominal bleed.
Blood loss can be significant before outward signs and symptoms appear. Infants can have bleeds with gastroenteritis, intussusception or Meckel's Diverticulum.
Symptoms
Desmopressin
For nausea and vomiting without an obvious cause, consider that these may be symptoms of intracranial bleeding.
Inquire about head injury, mental status changes, and other neurologic signs and symptoms, and consider CT scan
of the head.
Joint / Muscle
Soft Tissue
Symptoms of abdominal muscle bleeding (rectus, pectorals, latissimus, obliques) are a palpable mass, rigidity, and
pain. Concurrent bleeding in the abdominal cavity may be present and go unnoticed for days with a steadily
dropping hemoglobin. Rupture of the liver, spleen, or pancreas should be considered when the hemoglobin falls
dramatically following trauma.
Gyn.
A history of lifting heavy objects, weight lifting, falling on a bicycle crossbar or stretching the groin can precipitate abdominal wall, iliopsoas (see pg. 14 and 15), or retroperitoneal bleeding. These types of bleeds can occur
more commonly in individuals with type 3 VWD, and are rarely seen in type 1 and type 2 VWD.
Genitourinary bleeding
Factor
Meds
Hematuria is often frightening to the patient but not a serious event. Instruct the patient to remain at bed rest and
to increase fluids to 16 oz or 500 ml every hour over the next 24 hours. Protracted hematuria may require treatment.
Anti-fibrinolytics are contraindicated with hematuria. Contact the hematologist.
*
11
Labs
X-rays
Scrotal bleeding may occur after trauma, especially in toddlers. Treatment will be required and follow-up with the
hematologist or treatment center should be arranged.
Centers
Content
GI / GU
Mucous
Membr.
Head
Assess for signs of anemia
Obtain accurate menstrual history
- check hemoglobin
- pad and/or tampon count per
- check ferritin level
hour or per 24 hr time period
(include nights)
Gyn.
- frequency of changing protection
- amount of blood on each pad
(use a pictorial chart if available)
- number of overflow or flooded
pads
- length, regularity of menses
- missed days at school/work due
to menses
For active menorrhagia:
In addition to Humate-P ® , Alphanate ®
and wilate® or Desmopressin
(DDAVP ® or Stimate®/Octostim ® as
preparation available), start an antifibrinolytic (pg. 9).
- need for iron therapy either
currently or in the past
Consider prescribing birth control
therapy or IV Premarin as adjunctive
therapy to prevent more bleeding.
Trauma
Bibliography
Labs
X-rays
Factor
Meds
Desmopressin
Joint / Muscle
Soft Tissue
- presence of clots, size of clots
- follow-up with the
treatment center or the
patient’s hematologist
(within one week)
12
- Instruct patient to accurately
record bleeding, menstrual
history
- Recommend rest, drinking
fluids, eating iron rich foods
(or use supplemental iron
preparations)
Centers
Content
Intro
Basics
Menstrual Bleeding
Prolonged and heavy menstrual bleeding is one of the most common symptoms for females with bleeding
disorders.
Factor
Meds
Labs
X-rays
*
Desmopressin
The von Willebrand factor levels will decrease 24 to 48 hours following delivery, thereby increasing the risk of post-partum bleeding. In the event of a post-partum hemorrhage, treatment* should
be initiated immediately to elevate the von Willebrand factor levels. Life-threatening post-partum
hemorrhage will require treatment with a von Willebrand factor/FVIII concentrate (ex. Humate-P®*,
Alphanate® and wilate®) Adjunctive treatment with intravenous or oral anti-fibrinolytics may be useful
(see pg. 9).
Joint / Muscle
Soft Tissue
Postpartum Bleeding – During pregnancy, the majority of women with von Willebrand Disease,
type 1, will have normal von Willebrand factor and factor VIII levels due to increased estrogen levels.
“There are very few published data on the use of desmopressin during pregnancy, but there are some
concerns that desmopressin causes uterine contraction with premature labour, intrauterine growth
retardation and hyponatremia. For these reasons, it is advisable to be cautious about the use of desmopressin during pregnancy. Once the cord is clamped, desmopressin can be used if necessary. It is
also probably reasonable to use desmopressin before a caesarean. Desmopressin is not contraindicated
during lactation.”
Kouides, P.A., Phatak, P. D., Burkart, P., et al. (2000). Gynecological and obstetrical morbidity in women with type
1 von Willebrand disease : results of a patient survey. Hemophilia, 6(6), 643-648.
Gyn.
Assess for signs of anemia, as the patient's hemoglobin can drop to 2-3 g/ml Hgb, in just a few days,
from prolonged menses.
GI / GU
Menses - Some women bleed excessively through their menstrual cycle. Others bleed between cycles
or continuously through the month. These women may present to the ED with menorrhagia, iron
deficiency, anemia, or mittelschmerz due to increased bleeding with ovulation. Obtain an accurate
menstrual history and contact the patient’s hematologist for treatment recommendations.
Mucous
Membr.
Assess for signs of anemia, as the patient's hemoglobin can drop 2-3 g/ml Hgb, in just a few days,
from prolonged menses.
Head
Menarche – a teenage girl with von Willebrand Disease can present to an emergency department at
menarche or soon after with a severe, occasionally life-threatening hemorrhage. Appropriate treatment should commence immediately. Major vaginal bleeding requires treatment with a von Willebrand
factor concentrate (ex. Humate-P®*, Alphanate® and wilate®) Consultation with an OB-GYN specialist and hematologist at a bleeding disorder treatment center is essential for ongoing follow-up. Oral
contraceptives, anti-fibrinolytic treatment and desmopressin (IV, intranasally or subcutaneously) may
be recommended on an ongoing basis.
Early onset joint bleed
- tingling - pain
- limited range of motion
Advanced joint bleed
- heat - pain
- swelling
Management: Treatment product*
as per hematologist. Ice and
immobilization for comfort.
Mucous
Membr.
Head
Centers
Content
Soft Tissue / Muscle / Joint Bleeding
Deltoid / forearm bleed
- increased swelling and bruising
- observe for symptoms of
compartment syndrome
as per hematologist R.I.C.E.**
Soft tissue bleeds and bruising
- no functional impairment
- tenderness, but no severe pain
Management: No treatment,
R.I.C.E.**
Joint / Muscle
Soft Tissue
Gyn.
GI / GU
Neck swelling: EMERGENCY
- potential airway compromise
as per hematologist
Thigh/calf/buttock bleed
- pain
- with/without swelling
- impaired mobility
- observe for signs and symptoms
of compartment syndrome
as per hematologist
Labs
X-rays
Factor
Meds
Desmopressin
Iliopsoas bleeds
- flexed hip
- pain /inability to extend the
leg on the affected side
Management: Treatment
product* as per hematologist
Trauma
Bibliography
14
-
**
RICE - Rest, Ice, Compression
(Ace® wraps), Elevation
-
Sling or splinting if support is needed
(i.e. Aircast® for ankles)
-
Crutches - for weight bearing
joints and crutch instructions
-
follow-up with the treatment center
or the patient’s hematologist
*
Soft tissue bleeds usually do not require aggressive treatment. Superficial hematomas and bruises respond well to rest, ice and elevation. If the hematoma and bruising continue to increase in size, impairing movement or function, treatment may be required.
Intro
Basics
Soft tissue and superficial bleeds
Centers
Content
Soft Tissue / Muscle / Joint Bleeding
Muscle bleeds
Muscles that exhibit warmth, pain, and swelling should be managed with the recommended treatment.* Anti-fibrinolytics may also
be helpful.
Gyn.
Treatment and follow-up care: Occasionally muscle bleeds may require treatment but more often will resolve with conservative treatment such as rest and ice. If compartment syndrome is suspected, appropriate treatment* should be initiated and the patient should
be admitted with an emergency consult to hematology.
GI / GU
Consequences of muscle bleeds: Muscle bleeds can result in serious consequences if not treated promptly. Extensive blood loss may
occur in large muscle groups. Muscle bleeding can place pressure on nerves and blood vessels and, if untreated, may result in permanent disabilities such as foot drop and wrist contracture. It is important that the patient’s hematologist be consulted before any
invasive procedures.
Mucous
Membr.
Persons with the most severe type of von Willebrand Disease, type 3, can experience muscle bleeding spontaneously or with minimal trauma. Any muscle group may be subject to bleeding. Common bleeding sites include the upper arm, forearm, thigh, and calf
muscles.
Head
Muscle bleeding is usually only associated with trauma in persons with mild von Willebrand Disease.
Joint Bleeding
Signs and symptoms: Outward signs of joint bleeding include restriction of movement, swelling, heat, and erythema on and around
the joint. The patient may report symptoms of a bubbling or tingling sensation with no physical signs. Later symptoms include a
feeling of fullness within the joint and moderate to severe pain as the bleed worsens.
Joint Aspiration: Caution!
The aspiration of joint bleeds in VWD is contraindicated unless recommended by the treatment center.
*
Labs
X-rays
Initiate treatment before any diagnostic procedures such as x-ray. Before dislocated joints are reduced, infuse with the recommended treatment*.
Factor
Meds
Treatment: Some patients may present for treatment with no other outward signs of bleeding than decreased range of motion and
a complaint of pain or tingling. This is indicative of an early onset joint bleed and is the optimal time to treat. The patient should
be infused as quickly as possible with the recommended treatment* in order to minimize pain and joint destruction. Extreme pain,
swelling, heat, and immobility are signs and symptoms of an advanced joint bleed which occurs only after blood has filled the joint
space.
Desmopressin
The joints most commonly affected are the elbows, knees, and ankles. Less common sites include the shoulders and hips. As
repeated bleeding occurs, the synovial tissue thickens and develops even more friable blood vessels. A vicious cycle of bleeding
and rebleeding may set in and the affected joint is referred to as a “target joint.” Eventually, repeated bleeding into joints leads to
arthropathy with destruction of cartilage and the eventual erosion of bone. The end result is decreased joint mobility and function.
Joint / Muscle
Soft Tissue
Joint bleeding is uncommon in individuals with type 1 and 2 von Willebrand Disease and is usually associated with trauma. Individuals with type 3 von Willebrand Disease can experience joint bleeding with or without trauma, and bleeding can occur into any
joint space.
Centers
Content
Desmopressin
Desmopressin is a synthetic form of antidiuretic hormone which causes the release of factor VIII and von Willebrand factor from the endothelial cell storage sites. It can increase the VWF level by as much as three to five fold.
Desmopressin is the preferred treatment for type 1 VWD and certain patients with type 2. The response to desmopressin
can vary greatly with each individual. Therefore, prior to use, a desmopressin trial should be done with results reviewed
and recorded by a hematologist. If the patient does respond to desmopressin, the full effect is reached 30 to 90 minutes
after administration and hemostasis is maintained for approximately 24 hours. If the patient does not respond to demopressin, hemostasis can be maintained with infusions of factor concentrates containing both von Willebrand protein and
factor VIII, such as Humate – P®* , Alphanate® , and wilate®.
GI / GU
Head
, STIMATE®
Mucous
Membr.
DESMOPRESSIN - brand names: DDAVP®, OCTOSTIM®
Expected side effects: short term facial flushing, increased heart rate, red conjunctiva, and headache.
Dose
Factor
Meds
Desmopressin
Joint / Muscle
Soft Tissue
Gyn.
SC/IV: 0.3 mcg/kg/dose. Recommendation: a maximum dose of 20 mcg.
IV ROUTE: Dilute with normal saline (50-100 ml). Infuse over 30-60 minutes. No less than 30 minutes. It is
recommended to administer the medication with the individual in a supine position.
SC ROUTE: The subcutaneous route is advantageous to minimize drug side effects.
Supplied
Ampules: 4 mcg/ml DDAVP®,
15 mcg/ml OCTOSTIM®
Nasal Spray
OCTOSTIM®
or STIMATE®
nasal spray must be brand specific to ensure patient receives the correct
®
dose of DDAVP that will stop the bleeding.
150 mcg / 0.1 ml per single spray (OCTOSTIM®
, STIMATE®
)
Recommended dose for patients over 50 kg: 300 mcg (1 spray per nostril, total of two sprays)
Trauma
Bibliography
Labs
X-rays
Recommended dose for patients under 50 kg: 150 mcg (1 spray in one nostril, total of one spray)
Unreliable absorption if the intranasal route is compromised. Hematologist should be consulted for treatment guidelines.
Indications
Treatment of von Willebrand Disease Type 1 and certain forms of Type 2
Contraindications
Hypersensitivity, infants under 3 months, patients suffering from dehydration, history of seizure, coronary artery
insufficiency.
16
*
Centers
Content
Desmopressin
Use With Caution
Intro
Basics
•Elderly •PatientswithvonWillebrandDiseaseType2B
•Youngchildrenespeciallyunder2yearsof age
•Hypertensivecardiovasculardisease
•Individualswithlow-normalbloodpressure
Head
Desmopressin has an antidiuretic effect. Patients should be advised to avoid alcohol and restrict their fluid intake to
thirst only for 24 hours after receiving the drug. Infants and children will require careful fluid intake restriction
to prevent possible hyponatremia and water intoxication. Accurate intake and output should be recorded on any
patients receiving IV fluids.
Mucous
Membr.
Adverse Effects
Cardiovascular: facial flushing, sweating, dizziness, transient hypertension, hypotension, and tachycardia,
hyponatremia.
GI / GU
Gastrointestinal: nausea, vomiting.
Neurologic: headache, tremor, seizures.
Gyn.
Local: pain and erythema at injection site or in nasal mucosa if intranasal spray is used.
Thrombocytopenia: in Type 2B von Willebrand Disease
Joint / Muscle
Soft Tissue
Topical Preparations
Amicar 10% topical
solution
Mix 2 ml Amicar (IV preparation 250 mg/ml), and 3 ml sterile water for
injection
Desmopressin
Soak gauze in solution, squeeze out excess and apply to area.
Discard solution after 24 hours
Tranexamic Acid 5%
topical solution
Mix 5 ml Tranexamic acid, use the IV preparation 100mg/ml (5 ml
ampule size) and 5 ml sterile water for injection
Factor
Meds
Soak gauze in solution, squeeze out excess and apply to area.
Discard solution after 24 hours
Request from Pharmacy
Topical agents such as topical Thrombin®
membrane bleeding.
and Gelfoam® and fibrin glue (Tisseal-R) may also be used to help control mucous
Antibiotics and pain medications may also be indicated in the treatment of mucosal bleeds.
17
Labs
X-rays
Tranexamic Acid 5% Nasal Gel
: To make a 5% Tranexamic Acid Nasal gel, take ten (10) Tranexamic acid 500 mg tablets and
crush with a very small amount of 70% alcohol. Measure out 100 grams of Intrasite gel (methylcellulose). Gradually add the Intrasite
gel to the crushed tablets/paste. Once mixed put in an ointment jar. Stable for 10 days refrigerated (probably much longer). Apply with
Q-tip® or finger once or twice a day.
Factor Administration
Reconstitute per package insert.
Products containing von Willebrand factor are plasma derived products.
Example for dose calculation
Patient’s weight = 50 kilograms
Order: 60 ristocetin co-factor units/kg IV
60 ristocetin co-factor units X 50 kg =
3,000 ristocetin co-factor units
The volume may be
10 ml, 20 ml, 30 ml
Examples: Humate-P® , Alphanate ®, wilate®
Labeled: Antihemophilic Factor/von Willebrand Factor Complex
(Human)
Mixing instructions and the rate of administration are found on the drug insert.
It is best to follow treatment recommendations that the patient may carry or to consult with the patient's
treatment center.
Dosage
Each bottle of factor concentrate is labeled with the activity
expressed in both von Willebrand ristocetin co-factor
international units (VWF: RCo I.U.) and factor VIII international units (F VIII I.U.).
2625 I.U. VWF:RCo/vial
866 I.U. FVIII/vial
Expires: June 14, 2007
Lot Number: 22566611A
Information on front of box
The dosage to be administered is based on the patient’s body weight in kilograms (kg) and is normally ordered in
ristocetin co-factor units (VWF:RCo I.U.). The von Willebrand factor/FVIII concentrate is a plasma-derived factor
that has been virally inactivated.
The ENTIRE contents of all the vials reconstituted for an infusion should be used, even if it exceeds the calculated
dosage (±10% of prescribed dose).
Document the lot number(s), expiration date(s), factor concentrate trade name and total number of units infused.
This information can be found on the factor concentrate’s box.
Some patients are instructed to bring unmixed factor concentrate with them to the ED to minimize treatment delay
and cost. Occasionally, patients will bring prepared factor concentrate after unsuccessful home venipuncture attempts,
Please assist with venipuncture and allow the patient or family to infuse the prepared factor concentrate, if possible,
per your institution's policy.
18
Centers
Content
Other Medications
Intro
Basics
Head
Mucous
Membr.
GI / GU
Pressure for a minimum of 5
minutes after a needle stick
Ice for 15 -20 minutes
Routine medications
Routes of administration
Factor
Meds
Medications which can be given PO, SC, or IV are preferred. If the rabies vaccination series is needed, an experienced hematologist (preferably the patient’s) should be contacted for advice prior to and after the injections in
order to prevent internal bleeding.
Desmopressin
Acetaminophen can be given for fever or pain. Narcotics/opioids can be given to control pain experienced by the
patient with a bleeding disorder. Avoid giving intramuscular injections of pain medications because of the possibility of causing a muscle bleed.
Joint / Muscle
Soft Tissue
Medications for fever or pain
Gyn.
Patients with VWD can receive routine medications (e.g. pain medications, antibiotics, etc.) that do not interfere
with clotting function. Avoid non-steroidal anti-inflammatories (NSAIDS), ASA and any product with aspirin-related ingredients (e.g. Pepto-Bismol®, Excedrin®, Percodan®).
Caution
Some patients with VWD may have liver disease from hepatitis or may have been exposed to HIV. Use caution
when prescribing drugs that may cause liver toxicity or could cause potential serious drug interactions.
19
Labs
X-rays
For any needle stick, pressure for a minimum of 5 minutes afterward will minimize soft tissue or muscle bleeding.
Avoid giving intramuscular injections of antibiotics, pain medications, or immunizations because of the possibility of causing a muscle bleed. You can also apply an ice pack for 15 - 20 minutes.
Treatment should never be delayed for laboratory studies to be
drawn or completed.
Head injury
First give the recommended
treatment*. . .
. . .then perform a
CT scan.
Fracture
Desmopressin
Joint / Muscle
Soft Tissue
Gyn.
GI / GU
Mucous
Membr.
Head
Centers
Content
Invasive Procedures, Labs, X-rays
. . .then immobilize
appropriately.
Labs
X-rays
Factor
Meds
First give the recommended
treatment*. . .
Trauma
Bibliography
Patient should follow-up with the
treatment center or hematologist
the next day.
20
*
Head injury: Discharge with
routine post head injury instructions
(patient should be assessed for two
weeks instead of 48 hours).
Intro
Basics
In general, patients with VWD who are experiencing an acute bleeding episode may need treatment as well as
basic first aid measures. Do not delay treatment to perform testing.
Centers
Content
Invasive Procedures, Labs, X-rays
Laboratory studies
Head
If the only complaint is an acute joint or muscle bleed, no laboratory studies are necessary. If GI, uterine, or
oral cavity bleeding is suspected and has potentially been extensive, a complete blood count may be indicated
to determine if the individual is anemic. Treatment should never be delayed for laboratory studies to be drawn
or completed.
Mucous
Membr.
X-rays and other radiological studies
A CT of the head (see pg. 4) is necessary when dealing with a potential intracranial hemorrhage. Give the maximum recommended treatment* before sending the patient to CT scan.
GI / GU
Remember that a swollen joint or extremity can be the result of internal bleeding. X-rays of the joint can be
used to document a joint bleed, but are generally not useful in detecting early onset bleeds when treatment is
optimal.
Gyn.
Fractures
Give the recommended treatment*, then X-ray and set the bone.
Joint / Muscle
Soft Tissue
Lacerations and sutures
Invasive procedures
Factor
Meds
Invasive procedures should be performed as clinically indicated, i.e. lumbar puncture with symptoms of meningitis. However, factor replacement treatment* should be given prior to the procedure.
Desmopressin
Sutures and staples can be used. If the laceration is significant enough to require sutures, the patient should first
receive the recommended treatment* and then proceed with the procedure. Contact the patient’s hematologist
for follow-up treatment instructions. No treatment is usually needed for suture removal.
Arterial sticks and venipunctures
Labs
X-rays
Do not perform arterial sticks unless no other option is available. If an arterial stick must be done, then
the recommended treatment* and precautions should be taken before the procedure begins.
Venipuncture may be done at any location; hands are generally excellent and no pre-treatment is necessary.
Avoid “digging” for deep veins. Apply pressure for several minutes or until there is no further oozing noted at
the venipuncture and IV removal sites.
*
21
Many different emergencies / trauma may occur to persons with von Willebrand Disease, just as to others.
Centers
Content
Trauma
Bibliography
Labs
X-rays
Factor
Meds
Desmopressin
Joint / Muscle
Soft Tissue
Gyn.
GI / GU
Mucous
Membr.
Head
Trauma / Emergencies
• Animalbites
•Motorvehicleaccidents
• Burns •Gunshotwounds
• Falls
•Ocularinjuries
• Fractures(seepg.20) •Puncturewounds
Treatment
For any serious injury, a major dose of a factor VIII product containing von Willebrand factor (eg. Humate-P®*,
Alphanate®, wilate®) should be infused prior to blood work, CT scan, X-rays or other scans, debriding, sutures,
etc.
For less serious injuries, other treatment options may suffice and can be considered: local treatment (pg. 8), desmopressin (pg.16-17), anti-fibrinolytics (pg. 9).
22
*
Intro
Basics
Association of Hemophilia Clinic Directors of Canada. (1999). Clinical practice guidelines: hemophilia and von Willebrand’s
disease: 1. Diagnosis, comprehensive care and assessment (2nd Edition) . Toronto, Canada : Association Of Hemophilia
Clinic Directors Of Canada
Centers
Content
Selected Bibliography
Association of Hemophilia Clinic Directors of Canada. (1999). Clinical practice guidelines: hemophilia and von Willebrand’s
disease: 2. Management (2nd Edition) . Toronto, Canada : Association Of Hemophilia Clinic Directors Of Canada
Cox Gill, J. (2004). Diagnosis and treatment of von Willebrand disease. Hematology/Oncology Clinics of North America, 18,
1277-1299.
Fressinaud, E., & Meyer, D. (2005). Von Willebrand disease: biological diagnosis. In C.A. Lee, E. E. Berntorp, & W. K. Hoots
(Eds.), Textbook of Hemophilia (pp. 272-278). Malden, Massachusetts: Blackwell Publishing Ltd.
Kadir, R.A. (1999). Women and Inherited Bleeding Disorders: Pregnancy and delivery. Seminars in Hematology, 36, 28 -35.
GI / GU
Gioia, K. P. (2000). Von Willebrand Disease . In K. P. Gioia (Ed.), Congenital Bleeding Disorders: Principles and Practices (pp.
103-109). King of Prussia, PA: Aventis Behring L.L.C.
Mucous
Membr.
Federici, A. B. (2005). Classification and clinical aspects of von Willebrand disease. In C.A. Lee, E. E. Berntorp, & W. K. Hoots
(Eds.), Textbook of Hemophilia (pp. 279-284). Malden, Massachusetts: Blackwell Publishing Ltd.
Head
Canadian Pharmacists Association (2006). Compendium of pharmaceuticals and specialties. 41th ed. Ottawa, Canada.
Kasper, Carol K. (2004). Von Willebrand Disease.[Monograph]. Los Angeles, USA:.Orthopedic Hospital, [Published by Aventis
Behring Foundation for research and advancement of patient care].
Gyn.
Kouides, P.A., Phatak, P. D., Burkart, P., et al. (2000). Gynecological and obstetrical morbidity in women with type 1 von
Willebrand disease : results of a patient survey. Hemophilia, 6(6), 643-648.
Lee, C.A, & Abdul-Kadir, R. (2005). Von Willebrand disease and women’s health. Seminars in Hematology, 42, 42-48.
Lillicrap, D. (2004). The Basic Science: Diagnosis and Clinical Management of von Willebrand disease. (Treatment of
Hemophilia Monograph No. 35). Montreal, Canada: World Federation of Hemophilia.
Joint / Muscle
Soft Tissue
Kirtava, A., Crudder, S., Dilley, A., Lally, C., Evatt, B. (2004). Trends in clinical management of women with von Willebrand
disease: a survey of 75 women enrolled in hemophilia treatment centres in the United States. Hemophilia, 10(2), 158-161.
Mannucci, P.M. (2004). Treatment of von Willebrand’s Disease. The New England Journal of Medicine, 351, 683-694.
Medical and Scientific Advisory Council of National Hemophilia Foundation. (2003). MASAC Recommendations concerning
the treatment of hemophilia and other bleeding disorders – Document # 151. New York, NY : National hemophilia
Foundation.
Factor
Meds
Rivard, G.E., Aledort, L., et al. (2008). Efficacy of factor VIII/von Willebrand factor concentrate Alphanate® in preventing
excessive bleeding during surgery in subjects with von Willebrand disease. Haemophilia, 14, 271-275.
Desmopressin
Mannucci ,P.M. (2001). Treatment of von Willebrand Disease. Thrombosis and Haemostasis, 80, 149-53.
Rodeghiero, F., & Castaman, G. (2005). Treatment of von Willebrand disease. Seminars in Hematology 42, 29-35.
Sindet-Pederssen, S., Ramstrom, G., Bernvil, S., et al. (1989). Hemostatic effects of tranexamic acid mouthwash in
anticoagulant-treated patients undergoing oral surgery. New England Journal of Medicine, 320, 30-3.
Labs
X-rays
Street, A. N., & Leung, W. (1990). Use of Tranexamic acid mouthwash in dental procedures in patients taking oral
anticoagulants. Medical Journal of Australia, 153: 630.
Thomson, A.R., Gill, J.C., Ewenstein, B. M., Mueller-Veltens, G., & Schwartz, B. A. (2004). Successful treatment for patients
with von Willebrand disease undergoing urgent surgery using factor VIII/VWF concentrate (Humate-P ®). Haemophilia, 10,
42-51.
Trauma
Bibliography
23
Acknowledgements
Authors and editors:
Susan C. Zappa, RN, CPN, CPON
Bleeding Disorders Nurse Coordinator
Fort Worth, Texas USA
Lucie Lacasse, RN, BScN
Hemophilia Nurse Specialist
The Ottawa Hospital
Ottawa, Ontario, Canada
Rose Jacobson, RN, Nurse Clinician
MB Bleeding Disorders Program
Health Sciences Centre
Winnipeg, Manitoba, Canada
Sherry L. Purcell, RN
Nurse Coordinator
Bleeding Disorders Clinic
Kingston General Hospital
Kingston, Ontario, Canada
Karen Wulff, RN
Nurse Coordinator
The Louisiana Center for
Bleeding and Clotting Disorders
Tulane University School of Medicine
New Orleans, Louisiana USA
Reviewers:
David Lillicrap, MD, FRCPC
Director, Kingston/Belleville
Regional Hemophilia Program
Professor, Department of
Pathology and Molecular Medicine
Canada Research Chair in Molecular
Hemostasis
Kingston, Ontario, Canada
Marcela Torres, MD
Pediatric Hematology and Oncology
Director of Hematology
Fort Worth, Texas USA
Jim Munn, RN, MS
Program Coordinator
University of Michigan
Hemophilia and Coagulation
Disorders Program
Ann Arbor, Michigan USA
Annette Smith
VWD Type 1
New Mexico, USA
Canadian consumer
VWD Type 3
Toronto, Ontario, Canada
Clare Cecchini
Program Development Coordinator
Canadian Hemophilia Society
Montreal, Quebec, Canada
Production Team- Project Manager: Art Gardner
Illustrations: Bob Aul and Mark Gilmore Colorizing: Heather Swaim Design & Layout: Art Gardner
von Willebrand Fact(or)Sheet
FOR ADULTS WITH VWD
BY SUSAN C. ZAPPA RN, CPN, CPON
Cook Children’s Medical Center, Fort Worth Texas 2003
This fact sheet was created to help you understand von Willebrand disease (VWD), a bleeding disorder.
Blood does a lot of different things as it goes around inside your veins. The veins are like a pipeline
carrying your blood cells filled with oxygen to your heart and lungs. Blood is also
made up of many different cells, like red blood cells (where iron is
stored), white
blood cells (these fight infection), and platelets (one of the parts of
the blood that
helps you to stop bleeding). Sorry, there are no blue blood cells!
Blood is also made up of “factors” that all work together to help you stop bleeding. Some
factors have a number name, like factor 8 or factor 9. If a person is missing one of these
factors, they have the blood disorder called hemophilia.
You are missing, non functioning or only a small amount of a factor called von Willebrand factor (VWF). A
doctor named Eric von Willebrand discovered this factor, so they named it after him. It’s kind of a hard name
to remember. You might think it would have been easier to remember a number like 8 or 9. Sorry, you need to
remember the name: von Willebrand Disease (VWD). It is a bleeding disorder.
There are 3 sub-categories or “Types”:
Type 1 patients have decreased amounts of VWF, Type 2 have non functioning & may have decreased amounts
of, VWF (sub-groups are a, b, n, or m) and Type 3 are missing VWF altogether. You need to know what type of
VWD you have.
How did we know you have von Willebrand disease?
Your doctor tested your blood to see how much VWF you have. Sometimes people have
to be tested a couple of times, because the VWF levels in your blood can briefly (for a
day or two) rise from things like exercise, surgery, certain medicines, illness, or if you
are upset or stressed -like when someone is sticking you with a needle!
1
FlashLINK to VWD
How do you get von Willebrand disease?
You inherited the gene from your family (like eye or hair color). OR there could also be a chance of a genetic
mutation occurring. You will always have von Willebrand disease: It does not go away and it can be passed on,
in your genes, to your children (there’s a 50/50 chance with each child).
Both males and females can have von Willebrand disease.
So, what does it mean to be missing or have non functioning or decreased VWF
It means that you have everything else to help you stop bleeding. You have all the other factors (there are 12)
that work together to make a clot or scab. You are just missing or have small amounts of 1 of the factors. But
people need all of the factors to stop bleeding right away. For people with VWD:
They do not bleed more than other people; instead, they bleed We call it oozing, because it happens slowly. You can ooze on the outside, where you see the blood (like a
nosebleed). You can ooze just under the skin (like in a bruise). Or, you can ooze deep inside your
body (internally). If someone is bleeding internally, the person will have pain and they may (or
may not) see swelling. This is the most serious type of bleeding, especially if it happens inside
the head (lots of damage can be done in there), joints, or muscles.
What does this mean for you?
It means that you will get bigger bruises than people who don’t have VWD. You may get nosebleeds that take
a long time to stop, or when you go to the dentist your gums may ooze blood for a long time. For women, they
may experience very heavy bleeding with their periods. If you have to have surgery or if you were hurt, doctors
would need to know that you have von Willebrand disease so bleeding could be taken care of with medicine.
All of your doctors, the dentist, etc. need to know that you have von Willebrand disease so they can care for
you properly.
There are medic alert bracelets, ankle bracelets, chains, etc. you can wear to let medical personnel know you
have von Willebrand disease if you are in an accident or unconscious.
2
FlashLINK to VWD
Can I play sports?
Really you can do almost anything …play basketball, baseball and soccer, and run track. You can be on a
ski team or swim team, and you can do gymnastics. It doesn’t mean you might not accidentally get hurt. You
should always wear protective gear, like helmets (essential in any sport that puts you on wheels) and pads to
try to prevent injuries. And you should know to seek medical care if you get hurt.
We want you to play in non contact sports and be normal.
We can take care of accidents that may happen.
However, should NOT play contact sports, like tackle football, hockey, and boxing. That’s because someone is out to hurt you in those sports. If you get badly hurt, we know you will have more
bleeding than the person who doesn’t have a bleeding disorder. That is too big of a risk to take.
What would we do for bleeding?
That depends on a couple of things, like what type of von Willebrand Disease you have.
The front page describes the three types: Type 1, Type 2 (a, b, m, or n), and Type 3.
For a lot of problems, you can just clean the cut and put pressure on the bleeding with a band-aid or
clean cloth. You can also put ice on an injury, not use or rest the injured area and also raise or elevate
the injury. These simple things will often stop the bleeding.
What medicines are used to stop bleeding?
For some types of VWD, levels of the VWD will increase if the person gets a medicine called DDAVP®. For other
types, the actual VWF is given in a vein (IV). When VWF levels are higher, the bleeding should stop.
3
FlashLINK to VWD
DDAVP treatment is usually given as a spray in the nose with a special medicine called Stimate®. This medicine
helps boost the amount of VWF, so that the blood can clot. It only lasts for 1-2 days and, it is usually
given every 48 hrs. Stimate®
is kept in the refrigerator, and you can have it at your house to give your
– self when you are bleeding (per your doctors orders). Only the nasal spray form of DDAVP named Stimate® will
stop the bleeding. It starts to work in about 1 hour. Ask your nurse about side effects. DDAVP can also be
given by injection in an arm or leg. Or it can be given
IV at the hospital. It may be given by injection or IV for
testing purposes.
A special factor product, administered intravenously, that has VWF (and factor 8) in it can also be used to stop
bleeding. This is given at the hospital or, if the patient been taught to start an IV, at home. Your doctor or nurse
will tell you when to give this factor. This is what is given if you are really bleeding a lot or if the
Stimate® spray doesn’t work for you. This factor product can make your VWF levels go up or be
normal. A dose only lasts for about 12 to 24 hours. It comes as a powder in a little bottle. You
mix the powder with the special water that also comes in a little bottle. Then you draw up the
medicine in a syringe and, using an IV needle, give it into a vein over just a few minutes. It starts to work in about
15 minutes. You can learn to give this factor to yourself per your doctor’s directions.
Other medicines we can prescribe to help mouth, nose, or menstrual bleeding are Amicar® or Cyclokapron®. These
medicines do not make you stop bleeding, but when you do form a clot, they will help keep that
clot from breaking down. They come as a pill or a liquid that you swallows. You have to take these
medicines exactly as the doctor or nurse tells you.
There are a few medicines that you should not take without asking your doctor or nurse first. These medicines can
make anyone who takes them bleed more than usual. You don’t need that! So before taking any aspirin or Advil® or
Motrin®, ask. And, ask your doctor or nurse if there are other medicines that they don’t want you to take.
You can take Tylenol® for a fever or for pain.
4
FlashLINK to VWD
Any questions?
Ask the nurse. Your nurse is a special nurse that takes care of people with bleeding disorders
and is there to help you understand VWD and to answer your questions.
You also have a special doctor, social worker, physical therapist, and other
people who act as a team, working together to help you.
Isn’t it great to have your own team?
Your Diagnosis:
Your doctor’s name: ___________________________________________________________________
Your nurse’s name: ___________________________________________________________________
Telephone number(s): _________________________________________________________________
Emergency number(s): ________________________________________________________________
Your treatment center name: ____________________________________________________________
Other team members: _________________________________________________________________
Edited and printed by the Nursing/Psychosocial Group of Hemophilia Region VI. Special thanks to: LISA LOGAN RN, BSN, CPON
Copyright 2010 May be reproduced with author’s permission for educational use only.
5
FlashLINK to VWD
FOR CHILDREN WITH VWD MIDDLE SCHOOL AGE AND UP
Your blood does a lot of different things as it goes around inside your veins. The veins are like a pipeline
carrying your blood cells filled with oxygen to your heart and lungs. Blood is also made up of many different
cells, like red blood cells (where iron is stored), white blood cells (these fight infection), and platelets
(one of the parts of the blood that help you to stop bleeding). Sorry, there are no blue blood cells!
So what color is your blood?? Take a look at your veins on the tops of your hands. Some people think blood is blue because the veins look blue. Actually, the color of our veins (the
pipelines) are blue or greenish, but the blood INSIDE the veins is red, just like it looks when you
cut yourself and bleed.
Blood is also made up of “factors” that all work together to help you stop bleeding. Some factors
have a number name, like factor 8 or factor 9. If you are missing one of these factors, you have
the blood disorder called hemophilia.
You are missing or have only a small amount of a factor called von Willebrand factor9 (VWF). A doctor named
Eric von Willebrand discovered this factor, so they named it after him. It’s kind of a hard name to remember.
You might think it would have been easier to remember a number like 8 or 9. Sorry, you need to remember the
name: von Willebrand disease (VWD). It is a bleeding disorder.
Your doctor tested your blood to see how much VWF you have. Sometimes you have to be tested a couple
of times, because the VWF levels in your blood can briefly rise from things like exercise, surgery, certain
medicines, if you are sick, or if you are upset or stressed -like when someone is sticking you with a needle!
1
FlashLINK to VWD
How did you get von Willebrand disease?
You got it from your mother or father. You inherited the gene from your family. Just like you got brown hair or
blue eyes, you got the gene that gave you von Willebrand disease: You will always
have von Willebrand disease, it does not go away, and you can pass it on to your
children (there’s a 50/50 chance with each child). Both boys and girls can have
von Willebrand disease.
So, what does it mean to be missing or have small amounts of VWF?
that work together to make a clot or scab. You are just missing or have small amounts of 1 of the factors. But
people need all of the factors to stop bleeding right away.
They do not bleed more than other people; instead, they bleed We call it oozing, because it happens slowly. Like ketchup oozing from your hamburger bun. Can you picture
it? You can ooze on the outside, where you see the blood (like a nosebleed). You can ooze just
under the skin (like in a bruise). Or you can ooze deep inside your body (internally). If you are
bleeding internally, you will have pain and you may (or may not) see swelling. This is the most
serious type of bleeding, especially if it happens inside your head (lots of damage can be done
in there).
What does this mean for you?
It means that you will get bigger bruises than people who don’t have VWD. You may get nosebleeds that take a
long time to stop, or when you go to the dentist your gums may ooze blood for a long time. If you have to have
surgery or if you were hurt, doctors would need to know that you have von Willebrand disease so they could
take care of you and stop the bleeding. All of your doctors, your dentist, plus your teachers need to know that
you have von Willebrand Disease.
There are cool medic alert bracelets, ankle bracelets, chains, etc. you can wear. If for some reason you were
2
FlashLINK to VWD
hurt and couldn’t talk, wearing a medical alert would let the doctors know about your von Willebrand disease.
Can I play sports?
Really you can do almost anything …play basketball, baseball and run soccer, and track. You can be on a swim
team and you can do gymnastics. It doesn’t mean you might not accidentally get hurt. You should always wear
protective gear, like helmets and pads to try to prevent injuries. And you should know to tell your mom or dad
or an adult if you get hurt. You are responsible.
We want you to play in non contact sports and be normal. We can take care of accidents that may happen.
You should NOT play contact sports, like tackle football, hockey, and boxing. That’s because someone is out
to hurt you in those sports. If you get badly hurt, we know you will have more bleeding than the person who
doesn’t have a bleeding disorder. That is too big of a risk to take.
That depends on a couple of things like what type of von Willebrand disease that you have.
There are 3 types: Type 1, Type 2 (a, b, m, or n), and Type 3. What type are you???
For a lot of problems, you can just clean the cut and put pressure on the bleeding with a bandage or clean cloth.
You can also put ice on an injury, not use or rest the injured area and also raise or elevate the injury. These
simple things will often stop the bleeding.
For some types of von Willebrand disease, levels of the missing factor will go higher if you get a medicine called
DDAVP®. For other types, you get the actual VWF in your vein (IV). When VWF levels are higher, the bleeding
should stop.
3
FlashLINK to VWD
helps boost the amount of von Willebrand factor, so that you are like normal. It only lasts for 1 or 2
days, but it can
stop the bleeding.
Stimate® is kept in the refrigerator and you can have it at your house for you to use when you are bleeding. Only the special nasal spray form of DDAVP is called Stimate®. It starts to work in about 1 hour.
You can also get DDAVP in a shot in your arm or leg or in an IV (in your vein) at the hospital. It’s lots easier
to spray something in your nose, though.
A special factor product, administered intravenously, that actually has von Willebrand factor (and factor 8) in it can
also be used to stop bleeding. This is given at the hospital or at home, if you or your mom or dad can start an IV.
It has to be given in a vein. This is what you would get if you were really bleeding a lot or if the
Stimate® spray doesn’t work for you. This factor product can make your VWF levels or numbers
go up and be normal. A dose only lasts for about 12 to 24 hours. It comes as a powder in a little
bottle. You mix the powder with the special water that also comes in a little bottle. Then you draw
up the medicine in a syringe (a shot) and using an IV needle, give it into a vein over just a few minutes. It starts to
work in about 15 minutes.
Other medicines we can give you to help mouth or nose bleeding are Amicar® or Cyclokapron®.
These medicines do not make you stop bleeding, but when you do form a clot, they will help keep
that clot from breaking down. They come as a pill or a liquid that you swallow. You have to take these
medicines just like your doctor tells you.
There are a few medicines that you should not take without talking to your doctor or nurse first.
These medicines can make anyone who takes them bleed more than usual. You don’t need that! So before taking
any aspirin or Advil® or Motrin®, ask. And, ask your doctor or nurse if there are other medicines that they don’t
want you to take.
4
FlashLINK to VWD
Any questions?
Ask your nurse. Your nurse is a special nurse that takes care of people with bleeding
disorders and is there to help you understand VWD.
You also have a special doctor, social worker, physical therapist and other
people who act as a team, working together to help you.
Your Diagnosis:
Your nurse’s name: ___________________________________________________________________
Edited and printed by the Nursing/Psychosocial Group of Hemophilia Region VI. Special thanks to Lisa Logan, RN, BSN, CPON
Copyright 2010 May be reproduced with the author’s permission for educational use only.
5
FlashLINK to VWD
FOR PARENTS AND CAREGIVERS
This fact sheet was created to help you understand von Willebrand Disease (VWD), a bleeding disorder.
made up of many different cells, like red blood cells (where
iron is stored), white
blood cells (these fight infection), and platelets (one of the
parts of the blood that
helps you to stop bleeding). Sorry, there are no blue blood cells!
Your student is missing, non functioning or only a small amount of a factor called von Willebrand factor (VWF).
A doctor named Eric von Willebrand discovered this factor, so they named it after him. The kids always think
it would have been easier to remember a number like 8 or 9, but they need to remember the name: von
Willebrand Disease (VWD). It is a bleeding disorder.
There are 3 sub-categories or “Types”:
Type 1 patients have decreased amounts of VWF, Type 2 have non-functioning & also may have decreased
amounts of VWF and Type 3 are missing VWF altogether. You need to learn what type of VWD your child has,
this is important.
Your doctor tested your child’s blood to see how much VWF they have. Sometimes people have to be tested a
couple of times because the VWF levels in your blood can briefly (for a day or two) go higher from things like
exercise, surgery, certain medicines, if you are sick, or if you are upset or stressed -like when someone is
sticking you with a needle!
1
FlashLINK to VWD
How did you get von Willebrand disease?
You inherit the gene from your family (like eye or hair color). OR there could also be a chance of a genetic
mutation occurring. Your child will always have von Willebrand Disease, it does not
go away and it can be passed on, in their genes, to their children (there’s a 50/50
chance with each child). Both boys and girls can have von Willebrand Disease.
So, what does it mean to be missing or have non-functioning or
decreased VWF?
that work together to make a clot or scab. You are just missing or have small amounts of 1 of the factors. But,
body (internally). If someone is bleeding internally, the person will have pain and you may (or
What does this mean for your child?
It just means that they will get bigger bruises than other people who don’t have VWD. They may get nosebleeds
that take a long time to stop, or when they go to the dentist their gums may ooze blood for a long time. For
girls, they may experience very heavy bleeding with their periods. If your child has to have surgery or if they
were hurt, doctors would need to know that they have von Willebrand Disease so bleeding could be taken
care of with medicine. All of your child’s doctors, the dentist, plus teachers need to know that your child has a
bleeding disorder.
There are cool medic alert bracelets, ankle bracelets, chains, etc. you can buy for your child to wear to let
2
FlashLINK to VWD
medical personnel know they have von Willebrand Disease.
Can my child play sports?
Really he or she can do almost anything …play basketball, baseball, soccer, and track. They can be on a ski
team, swim team and they can do gymnastics. It doesn’t mean they might not accidentally get hurt. They
should always wear protective gear, like helmets (essential in any sport that puts the child on wheels) and pads
to try and prevent injuries. And they should know to tell you or an adult if they get hurt
We want your child to play in non-contact sports and be normal.
We can take care of accidents that may happen.
Your child should NOT play contact sports, like tackle football, hockey and boxing, though. That’s because someone is out to hurt them in those sports. If they get badly hurt, we know they will have
more bleeding than the person who doesn’t have a bleeding disorder. That is too big of a risk to take.
That depends on a couple of things, like what type of von Willebrand Disease the person has.
The front page describes the three types: Type 1, Type 2 (a, b, m, or n), and Type 3.
For a lot of problems, you can just clean the cut and put pressure on the bleeding with a band-aid or
clean cloth. You can also put ice on an injury, not use or rest the injured area and also raise or elevate
the injury. These simple things will often stop the bleeding.
For some types of VWD, levels of the VWF will go higher if the person gets a medicine called DDAVP®. For other
3
FlashLINK to VWD
helps boost the amount of VWF, so that the blood can clot. It only lasts for 1-2 days, and it is usually
given every 48 hrs. Stimate® is kept in the refrigerator, and you can have it at your house to give your
child when they are bleeding (per your doctors orders). Only the nasal spray form of DDAVP named
Stimate® will stop the bleeding. It starts to work in about 1 hour.
Ask your nurse about side effects. DDAVP can also be given by injection in an arm or leg. Or it can be given IV
at the hospital. It may be given by injection or IV for testing purposes.
bleeding. This is given at the hospital or, if the patient been taught to start an IV, at home. Your doctor or nurse will
tell you when to give this factor. This is what is given if your child is really bleeding a lot or if the Stimate® spray
doesn’t work for your child. This factor product can make VWF levels go up or be normal. A dose only lasts for
about 12 to 24 hours. It comes as a powder in a little bottle. You mix the powder with the special
water that also comes in a little bottle. Then you draw up the medicine in a syringe and, using an
IV needle, give it into a vein over just a few minutes. It starts to work in about 15 minutes. If old
enough, your child can learn to give this factor to themselves.
Other medicines we can prescribe to help mouth, nose, or menstrual bleeding are Amicar® or
Cyclokapron®. These medicines do not make you stop bleeding, but when you do form a clot, they will
help keep that clot from breaking down. They come as a pill or a liquid that your child will swallow. You
have to give these medicines exactly as the doctor or nurse tells you.
There are a few medicines that your child should not take without asking your doctor or nurse first.
These medicines can make anyone who takes them bleed more than usual. You don’t need that! So before givng
any aspirin or Advil® or Motrin®, ask. And, ask your doctor or nurse if there are other medicines that they don’t
want you to give.
You can give Tylenol® for a fever or for pain.
4
FlashLINK to VWD
Any questions?
Ask the nurse. Your child’s nurse is a special nurse that just takes care of people with
bleeding disorders and is there to help you and your child understand
VWD.
You also have a special doctor, social worker, physical therapist and other
people who are a TEAM, working together to help you.
Your Diagnosis:
Your nurse’s name: ___________________________________________________________________
5
FlashLINK to VWD
von Willebrand Fact(or)sheet
FOR SCHOOL PERSONNEL
Cook Children’s Medical Center, Fort Worth Texas
This fact sheet was created to help you understand von Willebrand disease (VWD), a bleeding disorder.
made up of many different cells, like red blood cells (where
iron is stored), white
blood cells (these fight infection), and platelets (one of the
parts of the blood that
helps you to stop bleeding).
Your student is missing, non functioning or only a small amount of a factor called von Willebrand factor (VWF).
A doctor named Eric von Willebrand discovered this factor, so they named it after him. The kids always think
it would have been easier to remember a number like 8 or 9, but they need to remember the name: von
Willebrand Disease (VWD). It is a bleeding disorder.
There are 3 sub categories or “Types”:
Type 1 patients have decreased amounts of VWF, Type 2 have non functioning, and may also have decreased
amounts of, VWF, and Type 3 are missing VWF altogether.
How do we know a person has von Willebrand disease?
The doctor tested the child’s blood to see how much VWF they have. Sometimes they have to be tested a couple
of times, because the VWF levels in your blood can briefly (a day or two) rise from things like exercise, surgery,
certain medicines, illness, or if you are upset or stressed-like when someone is sticking you with a needle!
1
FlashLINK to VWD
How does a person get von Willebrand disease?
You inherit the gene from your family. OR there could also be a chance of a genetic mutation occurring. The
child will always have von Willebrand disease: it does not go away, and it can be
passed on, to their children (there’s a 50/50 chance with each child).
Both boys and girls can have von Willebrand disease.
So, what does it mean to be missing or have non-functioning or
decreased VWF?
that work together to make a clot or scab. You are just missing or have small amounts of 1 of the factors. But,
body (internally). If someone is bleeding internally, the person will have pain and you may (or
What does this mean for your student?
It means that they will get bigger bruises than people who don’t have VWD. They may get nosebleeds that
take a long time to stop, or when they go to the dentist their gums may ooze blood for a long time. Girls may
experience very heavy bleeding during their periods. They may need to change their sanitary protection hourly
and still may experience break through bleeding. Please keep this in mind and help us work out the best
solutions to help the student.
We hope the school will be understanding of students who may have nosebleeds or other bleeding problems.
Keeping the child in class and and on as normal a schedule as possible is the goal. Working together, we hope
to achieve that goal. Contact the student’s nurse (information on last page) for any questions or concerns.
2
FlashLINK to VWD
Can my student play sports?
Really he or she can do almost anything …play basketball, baseball and soccer, and run track. They can be on
a ski team or swim team, and they can do gymnastics. It doesn’t mean they might not accidentally get hurt.
They should always wear protective gear, like helmets (essential in any sport that puts the child on wheels)
and pads to try to prevent injuries. And they should know to tell an adult if they get hurt or think they are hurt.
Their parent or nurse should then be contacted for instructions.
We want the student to play non-contact sports. We can take care of accidents that may happen.
However, student should NOT play contact sports, like tackle football, hockey, and boxing. That’s because
someone is out to hurt them in those sports. If they get badly hurt, we know they will have more bleeding than
the person who doesn’t have a bleeding disorder. That is too big of a risk to take.
That depends on a couple of things, like what type of VWD the person has.
The front page describes the 3 “types”: Type 1, Type 2 (a, b, m, or n), and Type 3
For a lot of injuries, you can just clean the cut and put pressure on the bleeding with a bandage or clean cloth.
You can also put ice on an injury. The student can rest the injured area and also raise or elevate the injury.
These simple things will often stop or reduce the bleeding.
For some types of VWD, levels of the VWF will increase if the person gets a medicine called DDAVP®. For other
3
FlashLINK to VWD
helps boost the amount of VWF, so that the blood can clot. It only lasts for 1-2 days, and it is
usually given every
48 hrs. Stimate® is kept in the refrigerator, and you can have it at your house
to give your child when they are bleeding (per your doctors orders). Only the nasal spray form of DDAVP named
Stimate® will stop the bleeding. It starts to work in about 1 hour.
Ask your nurse about side effects. DDAVP can also be given by injection in an arm or leg. Or it can be given IV
at the hospital. It may be given by injection or IV for testing purposes.
bleeding. This is given at the hospital or, if the patient been taught to start an IV, at home. Your doctor or nurse will
tell you when to give this factor. This is what is given if your child is really bleeding a lot or if the Stimate® spray
doesn’t work for your child. This factor product can make VWF levels go up or be normal. A dose only lasts for
about 12 to 24 hours. It comes as a powder in a little bottle. You mix the powder with the special
water that also comes in a little bottle. Then you draw up the medicine in a syringe and, using
an IV needle, give it into a vein over just a few minutes. It starts to work in about 15 minutes. If
old enough, your child can learn to give this factor to themselves.
Other medicines we can prescribe to help mouth, nose, or menstrual bleeding are Amicar® or
Cyclokapron®. These medicines do not make you stop bleeding, but when you do form a clot, they
will help keep that clot from breaking down. They come as a pill or a liquid that the child swallows.
You have to give these medicines exactly as prescribed. The student may have to take them at
school.
There are a few medicines that the child should not take without asking their doctor or nurse first.
These medicines can make anyone who takes them bleed more than usual. They don’t need that! So before givng
any aspirin or Advil® or Motrin®, ask. And, ask the doctor or nurse if there are other medicines that they don’t want
you to give.
You can give Tylenol® for a fever or for pain.
4
FlashLINK to VWD
Any questions?
Ask the parent or nurse. The child’s nurse is a special nurse that just takes care of people
with bleeding disorders and is there to help you and the child understand
VWD.
The child also has a special doctor, social worker, physical therapist, and
other people who act as a team, to help take care of them.
The school is also a part of the child’s ‘team’. We want to work with you.
Student’s Diagnosis:
Your nurse’s name: ___________________________________________________________________
Emergency number(s): _________________________________________________________________
Your treatment center name: _____________________________________________________________
Other team members: __________________________________________________________________
Telephone number(s): __________________________________________________________________
5
FlashLINK to VWD
von Willebrand disease
What is von Willebrand disease?
VWD is the most common bleeding disorder, affecting approximately 1% of the population.1 VWD is caused by
low levels or a complete lack of VWF (quantitative defects) or by abnormalities in the functioning of the VWF
protein (qualitative defects) that impair the formation of a platelet plug. Insufficient or ineffective VWF
also results in inadequate levels of FVIII, and this combination can lead to bleeding.2
Unlike hemophilia, a bleeding disorder that generally occurs in males only, VWD affects males and females
equally.3
What are the types of von Willebrand disease?
VWD is classified into 3 types based on the quantity or quality of VWF produced. It is important for a person to
know which type of VWD he or she has because each type is treated differently.
Type 1 VWD (a quantitative defect)
In type 1 VWD, levels of VWF are mild to moderately low—typically 20%-50% of normal.4 FVIII levels may also
be reduced.5 Type 1 is the mildest and most common form and affects approximately 80% of people with
VWD.4
Type 2 VWD (a qualitative defect in the VWF multimer)
Type 2 VWD accounts for about 20% of cases.4 Type 2 VWD is divided into 4 subtypes.
•Type 2A. Type 2A is caused by a deficiency of normal VWF multimers.
•Type 2B. In type 2B, VWF binds too tightly with platelets, thereby decreasing the number of platelets available to stop bleeding. The term for low platelet levels in the blood is thrombocytopenia.
•Type 2M. In type 2M, VWF doesn’t bind tightly enough with platelets, thus preventing platelets from clumping
together to form a plug.
•Type 2N. In type 2N, VWF does not bind effectively with FVIII and does not protect FVIII from being destroyed
in the bloodstream. The resulting low levels of FVIII seen in type 2N VWD also occur in mild hemophilia A.
1
FlashLINK to VWD
Type 3 VWD (a quantitative defect)
Type 3 is the most serious form of VWD, and it is very rare, occurring in less than 1% of individuals with this bleeding
disorder.4 In type 3, VWF levels are extremely low or completely absent, and FVIII levels are also very reduced.
References:
1.
Pruthi RK. A practical approach to genetic testing for von Willebrand disease.
Mayo Clin Proc. 2006;81(5):679-691.
2.
Mannucci PM. Treatment of von Willebrand’s Disease. N Engl J Med. 2004;351(7):683-694.
3.
NHF. National Hemophilia Foundation. von Willebrand disease. Available at:
http://www.hemophilia.org/NHFWeb/MainPgs/MainNHF.aspx?menuid=182&contentid=47&rptname=blee
ding. Accessed September 24, 2010.
4.
Lillicrap D. The basic science, diagnosis, and clinical management of von Willebrand disease. World Federation of Hemophilia. April 2008, number 35.
5.
NHLBI. National Heart, Lung, and Blood Institute. von Willebrand disease. Available at:
http://www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html Accessed September 24, 2010.
2
FlashLINK to VWD

- Alphanate

Transcription

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