Your Guide to von Willebrand
If you often have large, lumpy bruises; frequent or
difficult-to-stop nosebleeds; bleed a lot after a fairly minor cut; or are a
woman who has very heavy or long menstrual periods, you should ask your doctor
about an inherited bleeding disorder called von Willebrand disease (VWD). Named
for Dr. Erik von Willebrand, a Finnish doctor who first described the condition
in 1926, VWD affects your bloods ability to clot and can lead to heavy,
hard-to-stop bleeding after an injury. The bleeding from VWD can lead to damage
of your internal organs or even be life threatening, but this is rare.
In VWD, you either have low levels of a certain
protein in your blood or the protein doesnt work the way it should. The
protein is called von Willebrand factor (VWF). It is made in the walls of your
blood vessels and released into your blood.
Normally, when one of your blood vessels is injured,
you start to bleed. As soon as this happens, small cells in your blood that are
called platelets clump together to plug the hole in the blood vessel and stop
the bleeding. Von Willebrand factor acts like glue to help the platelets stick
together and form a blood clot.
Von Willebrand factor also carries with it clotting
factor VIII (8), another important protein that helps your blood clot. Factor
VIII is the protein that is inactive or missing in hemophilia, another clotting
Von Willebrand disease, like hemophilia, is an
inherited bleeding disorder, but VWD is more common and usually milder. In
fact, VWD is the most common of all the inherited bleeding disorders. It occurs
in about 1 out of every 100 to 1,000 people. It also affects both males and
females, while hemophilia mainly affects males.
There are three major types of von Willebrand
- In type 1 VWD, you have a low level of the von
Willebrand factor, and you may have lower levels of factor VIII than normal.
This is the mildest and most common form of VWD. About 3 out of every 4 people
with VWD have type 1 VWD.
- In type 2 VWD, the von Willebrand factor does not
work the way its supposed to. Type 2 VWD is divided into subtypes 2A, 2B,
2M, and 2N. Each type is caused by different gene mutations and treated
differently. This makes knowing the exact type of VWD that you have very
- In type 3 VWD, you usually have no von Willebrand
factor and low levels of factor VIII. Type 3 is the most serious form of VWD,
but very rare.
Von Willebrand disease cannot be cured, but it can be
treated. Early diagnosis is important, and with the right treatment plan, even
people with type 3 VWD can be helped to live active lives.
What Causes von Willebrand disease?
Von Willebrand disease is almost always inherited.
Your parents pass the gene for the disease on to you. You can develop types 1
or 2 VWD when only one of your parents carries the gene for it. You usually
inherit type 3 VWD only if both of your parents pass the gene on to you. Your
symptoms may be different from your parents.
Some people develop a form of the disease later in
life as a result of other medical conditions. This is called Acquired von
Willebrand Syndrome (AVWS).
What Are the Signs and Symptoms of von Willebrand
The signs and symptoms of VWD depend on the type and
severity of the disease. Many people have such mild symptoms that they may not
know they have the disorder. Some people have the gene for the disease but
dont have any symptoms.
If you have type 1 or type 2 VWD, you may
have the following mild to moderate symptoms:
- Frequent large bruises from minor bumps or
- Frequent or difficult-to-stop nosebleeds.
- Extended bleeding from the gums after a dental
- Heavy or extended menstrual bleeding in
- Blood in your stools from bleeding in your
intestines or stomach.
- Blood in your urine from bleeding in your kidneys
- Heavy bleeding after a cut or other accident.
- Heavy bleeding after surgery.
*Heavy menstrual bleeding is the most common symptom
in women. If it isnt treated, it can lead to iron deficiency and anemia.
(Not all heavy menstrual bleeding is due to VWD.)
If you have type 3 VWD, you may have any or
all of the symptoms listed above, as well as the following:
- Severe bleeding episodes for no reason. These
bleeding episodes can be life threatening if not treated right away.
- Bleeding into soft tissue or joints, causing severe
pain and swelling.
How is von Willebrand Disease Diagnosed?
Von Willebrand disease is sometimes difficult to
diagnose. People with type 1 or type 2 VWD may not have major bleeding
problems; as a result, they may not be diagnosed until they have heavy bleeding
after surgery or some other trauma.
On the other hand, type 3 VWD can cause major bleeding
problems during infancy and childhood. As a result, children with type 3 VWD
are usually diagnosed during their first year of life.
To find out if you have VWD, your doctor will take a
complete medical history and do a physical exam. For the history, he or she
will likely want to know about your personal and family historyin
- Any episodes of bleeding from a small wound that
lasted more than 15 minutes or started up again within the first seven days
following the injury.
- Any episodes of extended, heavy or repeated
bleeding after surgery or dental extractions that required medical
- Any episodes of bruising with little or no apparent
trauma, especially if you could feel a lump under the bruise.
- A nosebleed that occurred for no apparent reason
and lasted more than 10 minutes despite pressure on the nose or a nosebleed
that needed medical attention.
- Any episode of blood in your stool for no apparent
- Any heavy menstrual bleeding in women (usually with
clots or lasting longer than 7 to 10 days).
- Any history of muscle or joint bleeding.
- Any medicines youve taken that might cause
bleeding or increase the risk of bleedingfor example, aspirin, other
nonsteroidal anti-inflammatory drugs (NSAIDs), clopidogrel
(Plavix®), warfarin, or heparin.
- Any history of liver or kidney disease, blood or
bone marrow disease, or high or low blood platelet counts.
The doctor will also do a physical examination to look
- Unusual bruising or other signs of recent
- Evidence of liver disease or anemia.
No single test exists for diagnosing VWD.
As a result, your doctor will order a combination of
blood tests to diagnose the disease. These tests may include:
- Von Willebrand factor antigen. This test measures
the amount of von Willebrand factor in your blood.
- Von Willebrand factor ristocetin (ris-toe-SEE-tin)
cofactor activity. This test shows how well the von Willebrand factor
- Test for factor VIII clotting activity. Some people
with von Willebrand disease have low levels of factor VIII activity, while
others have normal levels.
- Von Willebrand factor multimers. This test is
performed if one or more of the 3 tests above are abnormal. It shows the makeup
or structure of the von Willebrand factor. It helps your doctor diagnose what
type of VWD you have.
- Platelet function test. This test measures how well
your platelets are working.
Your doctor may order these tests more than once to
confirm the diagnosis. He or she may also refer you to a hematologist (a doctor
who specializes in treating blood diseases) to confirm the diagnosis and for
Early diagnosis is important to make sure you are
treated effectively and can live a normal, active life.
How is von Willebrand Disease Treated?
Your doctor will decide what treatment you need, based
on the type of VWD you have and how severe it is. Most cases of von Willebrand
disease are mild, and you may need treatment only if you have surgery, tooth
extraction, or an accident.
Treatments for von Willebrand disease include
- Increase release of von Willebrand factor and
factor VIII into the bloodstream.
- Replace von Willebrand factor.
- Prevent breakdown of clots.
- Control heavy menstrual bleeding in women.
Specific treatments include:
- Desmopressin (DDAVP). This is a synthetic hormone
that you usually take by injection or nasal spray. It makes your body release
more von Willebrand factor and factor VIII into your bloodstream. DDAVP works
for most patients with type 1 and some with type 2 VWD.
- VWF replacement therapy. This involves getting an
infusion of a concentrate of von Willebrand factor and factor VIII into a vein
in your arm. This treatment can be used if you:
- Cant take DDAVP or need extended
- Have type 1 VWD that doesnt respond to
- Have type 2 or type 3 VWD.
- Oral contraceptives, or birth control pills, can
help women who have heavy menstrual bleeding. The hormones in the pills can
increase the amount of VWF and factor VIII in the bloodstream.
- Antifibrinolytic drugs help prevent the breakdown
of blood clots. They are used mostly to stop bleeding after minor surgery,
tooth extraction, or an injury. They may be used alone or together with DDAVP
and replacement therapy.
- Fibrin glue is medicine that is placed directly on
a wound to stop the bleeding.
Tips for Living with von Willebrand Disease
Its important that you try to prevent bleeding
and stay healthy. You should:
- Avoid over-the-counter medicines that can affect
blood clotting, including aspirin, Ibuprofen, and other nonsteroidal
anti-inflammatory drugs (NSAIDs).
- Always check with your doctor before taking any
- Tell your doctor or dentist that you have von
Willebrand disease. Your dentist can talk to your doctor about whether you need
medicine before dental work to reduce bleeding.
- Consider wearing a medical ID bracelet or necklace
if you have a serious form of VWD (for example, type 3 VWD), so that in case of
serious injury or accident the doctors caring for you will know you have
- Exercise regularly and maintain a healthy weight.
Exercise helps keep muscles flexible. It also helps prevent damage to muscles
and joints. Always stretch before exercising.
Some safe exercises or
activities are swimming, biking, and walking. Football, hockey, wrestling, and
lifting heavy weights are not safe activities if you have bleeding problems.
Always check with your doctor before starting any exercise program.
Frequently Asked Questions
- Is there a cure for von Willebrand
No, von Willebrand disease is a lifelong disorder.
However, most people have a mild form that causes little or no change in their
lives. Even those with type 3 von Willebrand disease can live normal, active
lives once they are treated.
- Who should know that I have von Willebrand
People like your doctor, dentist, employee health
nurse, gym trainer, and sports coach should be aware of your condition. If you
have a severe form of VWD (for example, type 3 VWD), consider wearing a medical
ID bracelet or necklace that states you have von Willebrand disease. In case of
an accident or emergency, this will be very helpful to the health care team
Since your parents, brothers and sisters, and children
may also have von Willebrand disease, you should consider telling them about
your diagnosis and suggesting they consider getting tested.
- Who should know that my child has von
Anyone who is responsible for your child should know
about his or her condition. For example, his or her teacher, school nurse,
daycare provider, coach, or any after-school program leader should know,
particularly if your child has one of the more severe forms of VWD (for
example, type 3 VWD).
- Is von Willebrand disease life
Most people with von Willebrand disease have a mild
form of the disease, which usually does not cause bleeding that is life
threatening. But any bleeding that cant be controlled can be life
threatening. Some people with severe forms of von Willebrand disease need to
seek emergency treatment to stop bleeding before it becomes life
- Do all people who have the gene for von
Willebrand disease have bleeding problems?
No. Some people carry the genes for the disease but do
not have symptoms. They still can pass the disease on to their children.
- How will I know if I have von Willebrand
The first step is a thorough history and physical
examination. If you or anyone in your family has a history of bleeding, your
doctor can order a number of blood tests to find out whether you have the
disease. These tests will check how quickly your blood clots and the levels of
clotting factors in your blood. Your doctor can use these test results to
diagnose your exact type of VWD.
For Women Only
Heavy menstrual bleeding is often the main symptom of
von Willebrand disease for women. Doctors call this menorrhagia. They define it
- bleeding with clots larger than about 1-inch in
- anemia or low blood iron
- need to change pad or tampon more than every
Of course, heavy menstrual bleeding can also be a sign
of a gynecological disorder, so its important to have a complete
gynecological exam before you seek testing for VWD.
If you have VWD and heavy menstrual bleeding, there
are treatments to help you. They include:
- Combined oral contraceptives. They increase VWF and
factor VIII in your blood and reduce menstrual blood loss. They are the most
often recommended birth control method for women with VWD.
- A levonorgestrel intrauterine device. This is a
contraceptive device that contains progestin.
- Aminocaproic acid or tranexamic acid are
antifibrinolytic drugs. They can reduce bleeding by slowing the breakdown of
- Desmopressin (DDAVP).
For some women who no longer want children,
endometrial ablation is performed. This procedure, which destroys the lining of
your uterus, has been shown to reduce menstrual blood loss in women with
If you need a hysterectomy, or surgical removal of the
uterus, for another reason, it will do away with menstrual bleeding altogether
and possibly improve your quality of life. However, hysterectomy carries its
own risk of bleeding complications.
Pregnancy can be a challenge for women with VWD.
Although blood levels of VWF and factor VIII tend to increase during pregnancy,
women with VWD can have bleeding complications during delivery. They also are
likely to have heavy bleeding for an extended period after delivery.
However, there are things you can do to minimize the
chances of complications in pregnancy:
- Consult a hematologist and an obstetrician who
specializes in high-risk pregnancies before you become pregnant.
- Consider using a center that specializes in
high-risk obstetrics and has a hematologist with expertise in bleeding
disorders on the staff for prenatal care and delivery.
- Before you have any invasive procedure, such as
amniocentesis, ask your doctor whether anything needs to be done to prevent
serious blood loss.
- During your third trimester, you should have blood
tests to measure VWF and factor VIII to help plan for delivery.
- You should also meet with an anesthesiologist to
review your choices for anesthesia and to discuss taking medicine to reduce
your bleeding risk.
With these precautions, most women with VWD can have
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