Von Willebrand Disease is probably the most prevalent of all the disorders of bleeding. We do not know the actual occurrence rate because many patients go undiagnosed, however, some estimate it may affect as many as 0.1% to1% of people. It can affect both boys and girls and most patients do not experience a single severe episode of bleeding unless a significant injury or surgery occurs but rather frequent episodes of bruising, nosebleeds, or menstrual periods that are heavy or prolonged throughout their life.
The von Willebrand protein, or factor, is a partner to factor 8, another key piece of our body that forms blood clots quickly and properly. The two proteins, when held together, protect each other from destruction. They survive in the blood together for a longer time like this. The von Willebrand factor’s function is to help bring platelets to an area that has been injured and bleeding to start the clotting process. It also helps all the platelets to stick to one another while a clot is forming. Lastly, it helps deliver enough factor 8 to a clot so that a forming clot can be strengthened.
Due to its close relationship with factor 8, many patients with von Willebrand disease were mistaken as hemophilia patients. In fact, the disease was even once known as pseudo-hemophilia. However, it appears to happen just as often in girls, unlike the majority of hemophilia patients who are boys. A Finnish doctor, Erik von Willebrand, described the disease that would come to bear his name as a disease that was passed on from parents to their children and distinct from hemophilia in 1926.
Today, we understand the disorder much better. Many people can have very mild or minimal bleeding symptoms, but some can have severe and frequent bleeding. Some of the bleeding symptoms include excessive bruising, unexpected and prolonged nosebleeds, frequent bleeding from the mouth and gums, bleeding after an injury or surgery, and heavy and prolonged menstrual periods. Childbirth may also be a risky time for some women, and frequent bleeding can often lead to iron deficiency anemia. There is, in fact, more than one type of von Willebrand disease requiring specialized testing in order to complete the diagnosis of von Willebrand disease.
Treating von Willebrand disease is tailored to the severity and type of bleeding. Many patients respond well to care of the local area with pressure, ice, and elevation. Women with heavy menstrual bleeding may see improvement with the help of a gynecologist using hormonal therapies. Still, other patients may respond well to medicines taken by mouth, in a nasal spray, or under the skin. When severe or life-threatening bleeding occurs, the deficient von Willebrand factor can be replaced with one of several forms of replacement medicine given into the vein.