Von Willebrand Disease is not a form of hemophilia. Because it is an inherited bleeding disorder people tend to associate it with hemophilia but in fact hemophilia is due to a deficiency of a clotting factor namely Factor VIII or IX.
Von Willebrand Disease is due to a deficiency of another blood clotting protein called von Willebrand Factor (von Willebrand’s Factor). von Willebrand’s Factor is a glue-like protein that helps blood cells called platelets stick to the site of an injury to form a temporary plug known as a platelet plug. If a person has either a low level of von Willebrand’s Factor or a defect in the protein then they have von Willebrand’s Disease. It is an autosomal dominant disorder meaning it affects males and females equally and you only need inherit one defective gene to have the disorder. It is not uncommon for numerous family members to have von Willebrand’s Disease though their symptoms may vary widely. On the other hand, hemophilia is what is known as a sex-linked recessive disorder meaning it affects mainly males and the females are the carriers.
The main symptoms of von Willebrand’s Disease, all related to the body’s inability to form a platelet plug include: are easy bruising and mucous membrane bleeding such as prolonged and heavy menstrual bleeding, frequent or prolonged nose bleeds, prolonged oozing after even simple cuts, prolonged bleeding after surgery and dental work and occasionally gastrointestinal bleeding. Unlike von Willebrand’s Disease, a person with hemophilia has normal von Willebrand’s Factor. The problem is that once the platelet plug is made, they cannot stabilize it with a fibrin blood clot which requires adequate amounts of the other clotting factors especially Factors VIII and IX. Because of this, the platelet plug is pushed out and bleeding resumes.
In VON WILLEBRAND’S DISEASE there are three main subtypes:
- Type 1 is a simple deficiency of von Willebrand’s Factor but the protein is otherwise normal.
- Type 2 means there is a molecular defect in the structure of the protein.
- Type 3 means there is a complete lack of von Willebrand’s Factor.
As von Willebrand’s disease can affect both males and females with equal chance, the complications of menstruation and child birth are major concerns.
Most women with Type 1 von Willebrand’s Disease, the most common form, do fine with pregnancy and delivery as the hormones that rise during pregnancy stimulate an increase in the level of von Willebrand’s Factor. When a woman breast feeds after delivery this helps the body to keep those same hormone levels high and prevent post-delivery bleeding.
In Type 2 and 3 von Willebrand’s Disease unfortunately, these hormones have no effect and other therapies are needed, However, pregnancy is still very possible and should always be managed in conjunction with an experienced hematologist who specializes in bleeding disorders. Not all hematologists do so. Most are also oncologists and specialize mainly in cancer treatment (ie blood cancers such as leukaemia). It is imperative to seek expert advice from physicians at Hemophilia Centers.