The most prevalent bleeding disorder, von Willebrand disease (VWD), affects more than 3 million Americans, about 1% of the population. Like hemophilia, VWD is a genetic disorder caused by a missing or defective clotting protein in the blood. In VWD, the problematic protein is von Willebrand factor, which plays a key role in the clotting process. Despite VWD being more common than hemophilia, numerous myths and misunderstandings persist about the disease.
Myth: Only women have VWD.
Fact: VWD occurs equally in men and women.
Myth: Women and men with VWD have the same symptoms.
Fact: Symptoms in both men and women vary depending on the severity of a person’s VWD. Both women and men may have frequent and prolonged nosebleeds, bruise easily and have excessive bleeding after surgery or dental work. In addition, women may experience heavy menstrual bleeding (HMB) and bleeding problems during and after childbirth.
Myth: There is only one type of VWD.
Fact: There are three main types of VWD and several subtypes.
Type 1 VWD is the most common and the mildest form. In type 1, the body has low levels of von Willebrand factor. People with type 1 may also have low levels of factor VIII, another blood-clotting protein. According to the Centers for Disease Control and Prevention (CDC), 85% of people treated for VWD in the US have type 1.
In type 2 VWD, the body produces a normal amount of von Willebrand factor, but the clotting protein doesn’t work as it should. There are four subtypes of type 2 VWD—2A, 2B, 2M and 2N—depending on the problem with the von Willebrand factor. Each subtype is treated differently.
Type 3 VWD is the rarest and most severe form. In type 3, the body makes little or no von Willebrand factor and has low levels of factor VIII.
Myth: VWD is easily diagnosed.
Fact: Because most men and women with VWD have type 1 and thus don’t regularly have significant bleeding episodes, they may not receive a diagnosis until they experience a major trauma, either from surgery, injury or dental work. Nosebleeds, bruising and HMB may all be attributed to other causes, further complicating diagnosis. Various tests performed by a hematologist can provide an accurate diagnosis. However, some tests may need to be repeated because von Willebrand factor levels can fluctuate in the body and are influenced by stress and hormones.
Myth: There’s no treatment for VWD symptoms.
Fact: Treatments are available to help control bleeding. The most common treatment is desmopressin (DDAVP), which boosts von Willebrand factor levels in the blood and can be taken by injection or nasal spray. Other treatments may include factor replacement therapy; drugs that help prevent the breakdown of blood clots; and, for women, hormone therapy such as birth control pills. Treatment depends on the type and severity of a person’s VWD, so it’s vital that patients work closely with their healthcare team to establish a management plan.
For more on von Willebrand disease, visit the National Hemophilia Foundation’s Steps for Living website.