HNC's SOAR — Support • Outreach • Advocacy • Resources — is a special program for women and girls with bleeding disorders
What is von Willebrand disease?
Von Willebrand disease (VWD) is a hereditary deficiency of von Willebrand factor in blood, occurring in 1% to 2% of the population. The factor is a protein that affects platelet function. Von Willebrand disease was first recognized in 1925 by Erik von Willebrand, a Finnish physician, who described a new type of bleeding disorder in inhabitants of the Aland Islands.
VWD occurs in men and women equally, but women are more likely to notice the symptoms because of heavy bleeding during their menstrual periods and after childbirth. Other signs of VWD include frequent or lengthy nosebleeds, easy bruising, and heavy bleeding from the mouth or gums. Sometimes bleeding is caused by injury, or it can have no obvious cause. On average, it takes 16 years for a woman to get a diagnosis of VWD after the onset of symptoms. Undiagnosed women are at risk for serious problems after surgery and childbirth. Every year, 30,000 women in the U.S. have a hysterectomy due to heavy bleeding. A large number of these women may have a bleeding disorder that can be treated without surgery and instead with clotting factor products containing von Willebrand Factor.
According to the National Hemophilia Foundation there are many variations of von Willebrand disease including:
- Type 1: Most common and mildest form of the disease. The von Willebrand factor levels are lower than normal and factor VIII levels may also be low. About 3 out of 4 people who have VWD have type 1.
- Type 2: In type 2 VWD, the von Willebrand factor doesn't work well. Type 2 is divided into subtypes: 2A, 2B, 2M, and 2N (Normandy). Different gene mutations (changes) cause each type, and each is treated differently. Thus, it's important to know the exact type of VWD that you have.
- Type 3: Severe von Willebrand disease. People who have type 3 VWD usually have no von Willebrand factor and low levels of factor VIII. Type 3 is the most serious form of VWD, but it's very rare.
Some pletelet defects are sometimes referred to as pseudo von Willebrand disease, even though the von Willebrand factor is unaffected.