Von Willebrand disease (VWD) is the most common inherited disorder that causes bleeding problems. It’s named after Erik von Willebrand, the doctor who first identified it in the 1920s. Your blood has proteins that aid in clotting, one of which is the “von Willebrand factor.” This protein helps blood platelets, the fragments in your blood, to bind and clump together, playing a crucial role in clotting.
If the von Willebrand factor is either low in quantity or not functioning properly, you might experience issues like frequent nosebleeds or heavy menstrual bleeding. Fortunately, effective treatments are available, allowing those who receive treatment to lead healthy, active lives.
There are four types of VWD, and knowing your specific type is important because it determines your treatment plan and outlook. VWD symptoms arise from excessive bleeding and may include frequent bleeding episodes.
People have type 1, 2, or 3 VWD from birth, inheriting the disease genes from their parents, which affect the function of the von Willebrand factor. Usually, inheriting one gene from one parent is sufficient for the disease, but for types 2 and 3, genes need to be inherited from both parents. There’s also acquired von Willebrand disease, which can develop later in life, often as a complication of other medical conditions affecting von Willebrand factor.
If you’re showing symptoms of VWD, it’s crucial to consult a healthcare provider for proper testing. They will inquire about your and your family’s medical history. If family history is uncertain, additional tests will be conducted to rule out other bleeding disorders like hemophilia. A series of specialized blood tests is also used to diagnose VWD, often in consultation with a hematologist.
The treatment plan for VWD depends on the type and severity. Mild cases might not require therapy, whereas others with severe symptoms might need treatment to control bleeding. DDAVP (desmopressin), a synthetic hormone, is often the first line of therapy, administered via veins or a nasal spray. This helps the body release more von Willebrand factor. However, it’s mainly effective in type 1 and sometimes type 2 VWD.
For those not responding well to DDAVP or with severe symptoms, replacement clotting proteins created in a lab may be necessary. Vonvendi, a drug for injecting von Willebrand protein into the vein, is one example. Antifibrinolytics, like Lysteda and Amicar, are pills that help prevent excessive blood loss and are generally affordable and insurance-covered.
For those with menstrual periods, birth control or IUDs might be recommended to manage bleeding. As types 1, 2, and 3 are inherited, there’s no prevention. However, following the treatment plan closely can help manage the condition and reduce bleeding. Some individuals with VWD may also face anemia due to heavy menstrual bleeding, requiring additional treatment like iron supplements.
Most people with VWD can enjoy normal, active lives. Even those with mild forms should maintain communication with healthcare providers to manage symptoms effectively and reduce risk during surgeries or dental procedures. Women with VWD can have healthy pregnancies but should work closely with specialists to minimize risks for both themselves and their babies.
