What Is von Willebrand Disease?
Von Willebrand disease is an inherited
bleeding disorder. Children born with the disease have one or both of the following:
- Low levels of a protein that helps the blood to clot, or
- The protein does not work as it should.
This clotting protein is called
von Willebrand factor. When some of this factor is missing or defective it can cause prolonged bleeding after an injury or accident.
What is clotting?
An injury (like a cut) to a blood vessel causes a complex chain of events called the clotting process, which results in a blood clot.
Clotting is your body's reaction to bleeding to keep you from losing too much
blood. Losing too much blood can be life threatening and can damage your internal organs.
What is von Willebrand Factor?
Certain
proteins in your blood help the blood to clot.
Von Willebrand factor is one of those proteins. It is made by the lining of cells in the wall of blood vessels (
veins and arteries). When blood vessels are damaged, platelets (small blood cells that collect and stick together to plug cuts and breaks in blood vessels) clump together at the site of the injury. Von Willebrand factor acts like glue that helps the platelets stick together. Von Willebrand factor is also a carrier of clotting factor VIII, another protein that helps your blood to clot.
Types of von Willebrand Disease
There are three major types of
von Willebrand disease:
- Type 1: A low level of the von Willebrand factor. Levels of factor VIII may also be lower than normal. This is the mildest and most common form of the disease. About 3 out of 4 of all people with von Willebrand disease have Type 1.
- Type 2: Due to a defect, the von Willebrand factor does not work as it should. Type 2 is divided into 2A and 2B. Each is treated differently so it is important to know the exact type.
- Type 3: People with this type usually have no von Willebrand factor and low levels of factor VIII. Type 3 is severe and very rare.
