Topic Overview
What is von Willebrand's disease?
Von Willebrand's disease is a
bleeding disorder. When you have this disease, it takes longer for your blood
to form clots, so you bleed for a longer time than other people.
Normally, when a person begins to bleed, small blood cells called
platelets go to the site of the bleeding and clump
together to help stop the bleeding. If you have von Willebrand's disease, your
blood doesn't clot well because you don't have a certain protein in your blood
or you have low levels of it. This protein is called the von Willebrand factor.
It helps your blood to clot by helping the platelets stick together.
The disease is mild in most people. It can stay the same or get better or
worse as you get older.
What are the types of von Willebrand's disease?
There are three major types of the
disease. They range from mild to severe.
- In type 1, you are missing some von Willebrand
factor. This can cause mild to moderate bleeding episodes. About 3 out of 4 people who have von
Willebrand's disease have type 1.1 You may not know
you have the disease, and you may not need treatment.
- In type 2, you
have the von Willebrand factor, but it doesn't work as it should. This usually
causes mild bleeding episodes, but it can cause moderate bleeding episodes.
- In type 3, you don't have
the von Willebrand factor or you have a very small amount. This type can lead
to serious bleeding episodes, but it is very rare. People who have type 3
disease can develop
anemia and can have dangerous bleeding after an
injury or during surgery.
What causes von Willebrand's disease?
Von Willebrand's disease usually is passed down
through families (inherited). If you have the disease, your doctor may
suggest that your family members get tested for it too.
It's possible to get acquired von Willebrand's disease later in life. This rare form of the disease isn't inherited. Instead, it seems to be caused by an immune system reaction in people who have certain diseases or take certain medicines.
What are the symptoms?
Bleeding
a lot is the main symptom of von Willebrand's disease. How severe the bleeding
is will be different for each person.
Symptoms of mild von
Willebrand's disease include:
- Frequent nosebleeds.
- Some bleeding
from the gums.
- Heavy menstrual periods in women (menorrhagia).
- Bruises that appear for no
reason.
- Heavy bleeding after injury or surgery.
Symptoms of more severe von Willebrand's disease include
those listed above and:
- Blood in the urine.
- Bruising
easily.
- Black, tarry, or bloody stools.
- Bleeding into
the joints, which causes stiffness, pain, and swelling. This symptom is rare.
How is von Willebrand's disease diagnosed?
Von Willebrand's disease can be hard to
diagnose. Some people with mild von Willebrand's disease bleed about the same
amount as other people do. You may not notice any symptoms until you bleed a
lot after an injury, dental procedure, or surgery.
Your doctor
will ask you how often and how much you bleed. If your doctor thinks you may
have a problem with clotting, he or she may suggest:
- Blood tests that measure von
Willebrand factor activity levels or von Willebrand factor antigen.
- Genetic testing that shows if you have a defect in your von
Willebrand factor and what type of von Willebrand's disease you have.
How is it treated?
Treatment depends on the type of von Willebrand's disease you have, how
much you bleed, and your risk for heavy bleeding.
If you have a mild type of von Willebrand's disease, you may
need to:
- Avoid
nonsteroidal anti-inflammatory drugs (NSAIDs), such as
aspirin and ibuprofen (Advil or Motrin, for example).
- Take medicine to prevent
heavy bleeding if you have an injury, are going to have surgery, or are about
to give birth.
- Avoid blood thinners, such as warfarin,
and antiplatelet medicines like clopidogrel.
If you have severe von Willebrand's disease, your treatment may include:
- Desmopressin medicine, which helps control
bleeding episodes.
- Replacement therapy, which replaces the
clotting factor in your blood.
- Antifibrinolytic medicines, to help stop blood clots from breaking
down.
- Birth control pills, or an intrauterine device (IUD) that contains hormones, to help control heavy menstrual
periods.
- Fibrin glue or thrombin powder, which you place on a
wound to help control bleeding.
If you have severe von Willebrand's disease, you most likely
will need to take extra care to treat and prevent bleeding episodes. Avoid NSAIDs and blood thinners. Try to
stay at a healthy weight and keep active. This helps prevent bleeding into
muscles and joints. Avoid sports or activities where injury and bleeding are
likely, such as football and hockey.
Tell all your doctors and other health
professionals, such as your dentist, that you have this disease. Doctors need
to know about it before you have any procedures, because you may be at risk for
dangerous bleeding.
Most large hospitals have a "bleeding disorders" resource center. Learn the phone number to the center closest to you, and carry it
with you.