The von Willebrand factor is a protein that helps blood clot. Von Willebrand disease (VWD) is the most common of all the inherited bleeding disorders, occurring in about 1 out of every 100 to 1,000 people. This disease decreases levels of this protein, or makes it work improperly, affecting your blood's ability to clot. If your blood doesn't clot, you can have heavy, hard-to-stop bleeding after an injury. The bleeding can damage your internal organs and in rare cases may be life-threatening.
Under normally circumstance, if you get a cut, you start to bleed. Small blood cells called platelets clump together (clot) to stop the bleeding. Von Willebrand factor acts like glue to help the platelets stick together and form a blood clot. Von Willebrand factor also carries another important protein, factor VIII, that helps your blood clot. People with hemophilia are missing this protein, or it doesn’t work properly. VWD is more common and usually milder than hemophilia. VWD affects both males and females, while hemophilia mainly affects males.
There are three major types of VWD. In type 1 VWD, you have a low level of the von Willebrand factor, and you may have lower levels of factor VIII than normal. This is the mildest and most common form of the disease. About 3 out of 4 people who have VWD have type 1. Type 2, the von Willebrand factor doesn’t work the way it's supposed to. Type 2 has four subtypes for the different gene mutations that cause each type. Each is treated differently. This makes knowing the exact type of VWD that you have very important. Type 3 VWD patients 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. Early diagnosis is important. With the right treatment plan, even people with type 3 VWD can be helped to live normal, active lives.