VON WILLEBRAND DISEASE

Von Willebrand Disease is the most common inherited bleeding disorder.  Von Willebrand factor is one of the clotting proteins that are needed to help the body make a blood clot to stop bleeding.  People who have von Willebrand Disease do not have enough von Willebrand clotting factor and/or the von Willebrand clotting factor does not work like it should.  Not having enough of any of these clotting proteins can delay the time it takes to make a blood clot and for this blood clot to be weak, causing bleeding to last longer or to stop and then start back up again.

von Willebrand Disease is diagnosed through a series of blood tests and history bleeding symptoms.  People who need to be tested for a bleeding disorder include:

Types of von Willebrand Disease

Unlike hemophilia that usually occurs in males, von Willebrand’s Disease can happen in both males and females equally.  There are three different types of von Willebrand’s disease.  Each type can be mild, moderate or severe.

Type 1 von Willebrand Disease

In type 1, there is not enough von Willebrand factor in the body for clotting to happen.  Type 1 is the most common von Willebrand’s disease and is also the mildest.

Type 2 von Willebrand Disease

In type 2, the von Willebrand factor in the body has a defect and does not work correctly.  There are different types of Type 2 von Willebrand defects, and bleeding is mild to moderate.

Type 3 von Willebrand Disease

This is the most serious form of von Willebrand Disease and is very rare.  In this type, the amount of von Willebrand factor is very low or completely absent, AND factor VIII (8) levels are very low.  Bleeding is severe.

How do you get von Willebrand Disease?

Von Willebrand Disease is mostly an inherited bleeding disorder; however, there is always one person in the family that is the first to have it.  People can also get von Willebrand Disease due to other medical conditions that cause the disease.  This is rare, and is called acquired von Willebrand Disease. 

TREATMENT 

Clotting Factor Replacement

The treatment for von Willebrand Disease is to replace the von Willebrand clotting factor that the body is lacking and/or that doesn’t work well.  Factor replacement is given through an intravenous (in the vein) infusion.  Frequency of treatment is dependent upon disease severity and bleeding history.  Treatments are either provided when a bleeding episode occurs (episodic) or to prevent a bleeding episode from occurring (prophylaxis).  Mostly, people diagnosed with von Willebrand Disease only need treatment when injury occurs, before more invasive dental work and before surgeries.  Those who experience frequent bleeding and/or are diagnosed with a more severe von Willebrand Disease will need to have treatments to prevent bleeding (prophylaxis treatments).  If prophylaxis treatments are needed, patients and caregivers will be taught how to give the factor replacement through the vein so treatments can be given at home.

DDAVP (Stimate)

DDAVP is a medication that pulls more von Willebrand factor into the blood stream from the cells lining blood vessels.  Testing will need to be done to make sure the medicine works to increase von Willebrand levels.  DDAVP does not work for someone diagnosed with Type 2B von Willebrand Disease. It is important to control drinking after getting DDAVP/Stimate because it can cause fluid retention.

Amicar and Lysteda (Aminocaproic Acid/Tranexamic Acid)

Amicar and Lysteda are oral medications that work very well for bleeding of mucous membranes such as nose and mouth bleeding.  Lysteda is also used for heavy menstrual bleeding.  When bleeding occurs, the body tries to heal the area by making a clot that will plug the hole to stop bleeding. This clot will stay there for about four days.  After these four days, chemicals in the body are triggered to breakdown the clot. This is called fibrinolysis. Amicar and Lysteda are called anti-fibrinolytic medications because it stops fibrinolysis. A person with a bleeding disorder needs more time for healing because they cannot make a strong clot to heal the area of bleeding.  Amicar stops the body from breaking the clot down.  This gives the blood vessels more time to completely heal and prevents prolonged bleeding and rebleeding.