What is von Willebrand's disease?

Most of us have factors in our blood which help to form blood clots, which in turn stem bleeding. One of these is von Willebrand factor, and people with von Willebrand disease (vWD) have limited or no von Willebrand factor in their blood. vWD is the most common bleeding disorder, and is thought to affect approximately one in every hundred people.

vWD is similar to haemophilia, but people with vWD usually suffer more from prolonged external bleeding, rather than internal bleeds. The disorder is passed on through an abnormal gene which can be inherited from the mother or father (or both, in some cases). Unlike haemophilia, vWD is not gender-specific, and it affects males and females equally.

How serious is vWD?

People with vWD may have reduced levels of the factor (mild, or vWD type I and type II), or they may have none at all (severe, or vWD type III). Mild vWD is the most common form of the disorder, and many people with vWD will lead normal active lives, with no need for treatment. However, people with severe vWD can suffer from internal bleeding episodes more commonly seen in people with haemophilia.

How is vWD treated?

For those who need treatment there are several options, depending on individual circumstances and the severity of the disorder.

• Tranexamic acid is often used to treat small but persistent bleeds (such as nosebleeds). This drug is taken orally, and works by slowing down the breakdown of clots.
• Desmopressin is also used to treat smaller bleeds, and during minor surgical procedures. It is injected into the vein, and increases the level of von Willebrand factor in the blood. 
• Concentrated von Willebrand factor is used to treat severe forms of the disorder. Again, this is injected and replaces the missing factor in the blood so that clotting takes place at a normal rate.

What are the symptoms of vWD?

The first indications of vWD may be noticed after a minor surgical procedure (such as a tooth extraction), where prolonged bleeding might be observed. Other symptoms may include frequent nose bleeds, heavy menstruation and bleeding after invasive surgery.

How do I find out if I have vWD?

If vWD is suspected, a specialist (haematologist) can do tests to determine how long you bleed before your blood clots. Your doctor will also take into consideration your personal and family medical history.

Diagnosis can be complicated and drawn-out, because some people with vWD have levels of clotting factors in their blood that are nearly normal. Furthermore, stress can encourage a falsely negative result, as it has been known to raise levels of von Willebrand factor in the blood. This is a real risk, as impending blood tests can often make people feel anxious.

Original material provided by the Haemophilia Foundation. Reviewed by everybody in March 2005.