von Willebrand disease (VWD)

Von Willebrand Disease (vWD) is an inherited disease affecting 1% of the population, which in Michigan means there are a possible 50,000 residents with some level and form of  vWD. VWD affects all racial, ethnic and economic groups world wide. Currently thousands of women in Michigan have bleeding symptoms that are not diagnosed or have been misdiagnosed leading to unnecessary hysterectomies. The symptoms for vWD include; heavy and/or long menstrual cycles, excessive nose bleeds, easy bruising and heavy bleeding after dental work, surgery, injury and childbirth. VWD is a treatable disease when the proper diagnosis has been made.

Someone affected by vWD does not have the ability to form the platelet plug, which helps to stop bleeding. A person with vWD either does not have enough of the proteins called von Willebrand Factor (vWF) to help stop bleeding or they have enough vWF but it does not work properly. There are three types of vWD: Type 1, which makes up 70–80% of people with vWD, is a lack of adequate numbers of vWF to form a proper platelet plug. Type 2 affects 15–30% of people with vWD and is when there is enough vWF present but it does not work properly. Type 3 is the rarest form of vWD and is when the person does not produce any vWF.

Some of the most commonly used treatments of vWD include:

Hormone treatment with estrogen and progesterone, the hormones found in oral contraceptives, can raise the levels of Factor VIII and von Willebrand factor. This therapy can be useful for managing heavy menstrual flow and other bleeding. For women with Type 1 vWD, treatment with oral contraceptives might be the only therapy needed.

DDAVP (desmopressin acetate): is a synthetic hormone. It works by causing the body to release von Willebrand factor. DDAVP can be administered intravenously or through a nasal spray. The nasal spray that is used to treat bleeding disorders is called Stimate. People affected by Type 1 and 2a vWD typically use this treatment.

Factor VIII Concentrate: some types of Factor VIII concentrates are rich in von Willebrand factor. Humate- P is a well-known Factor VIII concentrate product licensed to treat vWD. Factor VIII concentrates are infused into the blood stream. Although these products can be used for all types of vWD, individuals affected by Type 2 and 3 vWD are generally the patients prescribed such a treatment.

Patients with severe platelet function defects may need to receive transfusions of normal platelets.

 

Paper, R. & Kelley, L. (2002). A Guide to Living with von Willebrand Disease. Georgetown MA.: Kelley Communications