Topic Overview
What is von Willebrand's disease?
Von Willebrand's disease is a
bleeding disorder in which a protein in your body's blood system is missing or
does not work well. The protein is called von Willebrand factor. If the von
Willebrand factor is working properly, it helps your blood clot. Clotting stops
the bleeding when you are cut or injured.
The von Willebrand
factor usually carries another blood-clotting protein called factor VIII. If
you have von Willebrand's disease, the two proteins may not attach properly to
each other. Your blood will not clot as well as it should.
There
are three major types of the disease:
- Type 1: A shortage of von Willebrand factor (mild to moderate). This type of von Willebrand's disease can result in mild
to moderate bleeding episodes, depending on how much von Willebrand factor is
missing. About 3 out of 4 people with von Willebrand's disease are type
1.1 Some people with type 1 disease do not need
treatment and may not be aware that they have a blood disorder.
- Type 2: A flawed von Willebrand factor (usually mild). Although the body produces normal amounts of the von Willebrand
factor, it doesn't work properly. Type 2 is further classified (such as 2a or
2b) depending on the type of flaw.
- Type 3: A complete lack of von Willebrand factor (more severe). Type 3 von Willebrand's disease is very rare. When the
body does not produce von Willebrand factor, the cells (platelets)
needed to form a clot do not work properly and clotting factor VIII levels are
low, both of which can lead to severe bleeding. People with type 3 disease are
in more danger of
anemia and excessive bleeding after an accident or
during surgery.
What causes von Willebrand's disease?
Von Willebrand's disease usually is passed down
through families. It is the most common bleeding disorder present at birth
(congenital), but most cases are mild. About 1% of people may inherit this
disease. Men and women are equally likely to have von Willebrand's disease.
What are the symptoms?
Excessive bleeding is the main symptom of von Willebrand's disease. The
severity of the condition varies from person to person, even within the same
family. Many women with von Willebrand's disease have very
heavy menstrual bleeding. Other symptoms include
frequent nosebleeds and heavy bleeding after injury or surgery.
Von Willebrand's disease causes symptoms similar to the bleeding disorder
hemophilia.
How is von Willebrand's disease diagnosed?
Von Willebrand's disease
can be hard to diagnose. If you have symptoms that suggest a blood clotting
disorder, your doctor will ask about your medical history, especially about
episodes of excessive bleeding. Tests that can help with the diagnosis
include:
- Blood tests that measure bleeding time, von Willebrand factor
activity levels, or von Willebrand factor antigen.
- Genetic testing that shows a defect in your von Willebrand
factor.
How is it treated?
If
you have a mild type of von Willebrand's disease, you may only need home
treatment like not using
nonsteroidal anti-inflammatory drugs (NSAIDs), such as
aspirin.
But if you have a more severe type of von Willebrand's
disease, you probably will need to prevent and treat bleeding episodes
throughout your life. The course of von Willebrand's disease is difficult to
predict because it may stay at the same level of activity or get better or
worse as you get older.
Treatment may include:
- Desmopressin medicine, to help increase the production of von
Willebrand factor.
- Replacing the clotting factor in your blood.
- Medicine that helps prevent the breakdown of blood clots
(antifibrinolytic agents).
- Hormone therapy for women, to control heavy menstrual
periods.
- Topical medicine to control bleeding (thrombin powder).
- Avoiding medicines, such as aspirin, that can affect
bleeding.
Frequently Asked Questions
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