von Willebrand Disease

Thanks to Renee Paper, RN of the Hemophilia Foundation of Nevada for this information.

What is von Willebrand Disease?

von Willebrand disease is an inherited deficiency or defect of a blood clotting substance called von Willebrand factor.  Its' symptoms include easy bruising, frequent nose bleeds, bleeding gums, gastrointestinal bleeding, prolonged menstrual bleeding, and excessive bleeding following surgery.  Unlike hemophilia, von Willebrand Disease occurs equally amongst males and females and is much more common than hemophilia, occurring in 1 in every 100 births.

Who gets vWD?

Anyone can get vWD; both males and females.  It affects an estimated 1 out of every 100 people.  That's millions of Americans!

Where Does vWD Come From?

vWD is an inherited disorder meaning it is passed from parent to child.  Either parent can pass the disorder on to their offspring.  Even though a person with vWD is born with the disorder they may have no symptoms until challenged with injury, surgery, dental procedures such as having a tooth pulled or in the case of affected females, menstruation and childbirth.

Symptoms:

  • Easy bruising
  • Heavy or prolonged menstrual bleeding
  • Frequent or prolonged nosebleeds
  • Prolonged bleeding after injury, surgery, childbirth, or invasive dental procedures

How is vWD Diagnosed?

The diagnosis is made via specialized blood tests including:

  • von Willebrand Factor Antigen: measures the amount of von Willebrand Factor in the blood
  • Ristocetin Co-Factor: determines how well your von Willebrand Factor works
  • Factor VII level: Measures the level of a clotting protein called Factor VIII which in some people with vWD may also be reduced
  • von Willebrand Factor Multimers: an examination of the structure of the von Willebrand Factor

How is vWD Treated?

  • Despopressin Acetate (DDAVP®): This medicine helps to raise the level of von Willebrand Factor in the blood leading to effective clotting.  Most people with vWD respond to this medicine.  The injectable form is called DDAVP®.  The high-potency nasal spray form is called Stimate®
  • Clotting Factor Concentrates: People who do not respond to desmopressin may need intravenous infusions of concentrates containing Factor VIII/von Willebrand Factor.  These are made from human plasma and are treated to prevent the transmission of viruses.  The two brands most widely used in the U.S. are Humate-P® and Alphanate SD®
  • Hormones: Estrogen, usually in the form of birth control pills, helps to raise the level on von Willebrand Factor in the body and are very useful for heavy menstrual bleeding.
  • Anti-fibrinolytics: are medicines which help to prevent a clot that has formed from being dissolved.  They are useful in vWD because they keep a clot in place longer allowing healing to occur and preventing re-bleeding.  These are mainly used following nosebleeds, oral bleeding and heavy menstrual bleeding.  The most widely used brand is Amicar® (aminocaproic acid).

Where do I go for treatment?

Hemophilia Treatment Centers (Salt Lake City HTC): A network of specialty treatment centers in the U.S. and worldwide with medical teams that specialize in the diagnosis and treatment of bleeding disorders including vWD.