von Willebrand Disease
is the most common of all inherited
bleeding disorders. It is caused
by two problems: a deficiency in
Factor VIII and von Willebrand factor,
another clotting protein.
What is von Willebrand
von Willebrand Factor is a glue-like
"adhesive" protein that
carries and protects Factor VIII
in the bloodstream. It also helps
platelets stick to the blood vessel
walls at the injury site. When there
is not enough von Willebrand factor
or when von Willebrand factor does
not work correctly, a person has
von Willebrand Disease.
This disease is characterized by
prolonged bleeding after surgery
or trauma. In severe cases, the
prolonged bleeding may occur without
a known injury. Von Willebrand Disease
is diagnosed with a detailed patient
and family history and blood tests.
These tests are sensitive to monthly
hormonal changes, stress, medications
and exercise; therefore, sometimes
the tests must be repeated for accuracy.
Treatment of von Willebrand disease
There are effective treatments for bleeding related to von Willebrand Disease. The type of treatment depends on the individual’s type and severity of von Willebrand disease and the specific bleeding problems. A variety of medications can be given to stop an active bleed or to prevent bleeding from happening during surgery or other invasive procedures. For more severe von Willebrand’s Disease, there are preparations of von Willebrand factor that are given intravenously. Milder forms of the disease often respond to DDAVP (desmopressin acetate) which can be given either intravenously or as a nose spray. Birth control pills are usually the best treatment of choice for bleeding related to heavy menstrual periods. An oral medication called aminocaproic acid is often used in conjunction with the above medications to help stop or prevent bleeding. This medication is especially helpful in controlling bleeding in the mouth, often associated with dental work.