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von Willebrand Disease

Although less widely known, von Willebrand Disease (VWD) is understood to be the most common inherited bleeding disorder, affecting millions of people worldwide. The prevalence is as high as 1 to 2% in the general population.

What is von Willebrand Disease?

Definition:

VWD is a bleeding disorder caused by missing, non-functioning, or decreased amounts of a factor called von Willebrand Factor (VWF). Although usually inherited, VWD can also be the result of a spontaneous, new genetic mutation. The abnormal gene in VWD is located on a chromosome called an autosomal chromosome or autosome, rather than a sex-linked chromosome (like hemophilia is carried on). Therefore, the inheritance of VWD can occur equally among females and males.

Types:

There are three sub-categories of VWD:

  • Type I, which accounts for 60-80% of cases, results from a decreased production of normal VWF and factor VIII- decreased amounts of VWF and factor VIII.
  • Type II, which accounts for 10-30% of cases, is characterized by qualitative abnormalities of VWF and is further divided into subtypes 2A, 2B, 2M, and 2N- non-functioning VWF.
  • Type III, which accounts for 1-5% of cases, is the most severe form of the disease and is characterized by a very low or undetectable VWF in plasma and a low factor VIII activity. 1 in a million people have type III VWD. Symptoms are more frequent and severe - missing or very low levels of VWF and factor VIII.

What are the symptoms of von Willebrand Disease?

Signs/Symptoms:

Some individuals with VWD do not show any symptoms of the disease while others show mild to severe symptoms.

Typical signs include:

  • Pro-longed nosebleeds
  • Easy bruising
  • Heavy menstrual flow
  • Excessive or unusual bleeding from the gums and mouth
  • Pro-longed bleeding associated with surgery

What does von Willebrand Factor do in the blood?

Effects:

Blood does a lot of different things as it circulates through the veins. The blood is made up of four main components: plasma, which contains blood clotting proteins; red blood cells, which carry oxygen; white blood cells, which defend the body from infection; and platelets, round, sticky cells that help plug or seal off tears in blood vessels. One of the most important substances that makes the platelets stick together is VWF. This substance is essential for platelet-plug formation. VWF works as a "platelet glue" and also helps protect factor VIII and transport it through the body. Individuals with VWD are missing, have non-functioning, or decreased amounts of VWF, so they are either unable to form a platelet-plug or it is a pro-longed process.

How is a person diagnosed with von Willebrand Disease?

Diagnosis:

Bleeding symptoms, family history, test results, and routine blood tests prior to surgery are where diagnosis starts. However, VWD can be hard to diagnose because symptoms can be different in every patient, even those in the same family. Testing can also be complicated. Many kinds of blood tests are needed, so it is important to contact a hematologist at a Hemophilia Treatment Center (HTC) for proper diagnosis.

What treatment options are available?

Treatment:

Treatment is based on the type of VWD and the symptoms. In order to correct clotting problems, VWF and factor VIII levels need raised. Here are some common treatments:

  • DDAVP -synthetic copy of a natural hormone that releases the body's stored VWF into the bloodstream and increases levels of factor VIII, usually taken by infusion or nasal spray, and most effective for type I patients.
  • Replacement therapy -these medicines contain actual VWF proteins and factor VIII concentrates, taken by infusion, and effective for VWD patients who do not respond to DDAVP and for patients with type 2B and type III VWD.
  • Local clotting agents (antifibrinolytics) -they can be used to help the body protect clots and hold them in place, taken as injection or oral (tablet, capsule, elixer, or mouthwash).
  • Oral contraceptives -birth control pills can increase VWF levels and can be useful for women with heavy menstrual bleeding.

Learning to live with a bleeding disorder

Shock and disbelief often accompany learning you or a loved one has a bleeding disorder. It is perfectly normal to experience many upsetting emotions and have a feeling of fear and loss of control. Gaining information and experience will lessen the fear over time and grant you more control over the situation. The more you learn, the more you can incorporate VWD into the routine of your daily life.

Help and support is available

  • To find out more call FactorHealth, toll-free 1-866-322-3461 or click here
  • Connect with your nearest Hemophilia Treatment Center for comprehensive medical services.
  • For a complete list of Hemophilia Treatment Centers, visit the National Hemophilia Foundation web site.
  • Join your local Bleeding Disorders Chapter. For a complete list of local chapters, visit the National Hemophilia Foundation web site

Chapters provide:

  • Newsletter with information, educational articles and updates
  • Parent support groups
  • Organized events such as camps, family days, and educational events

Recommended Web Site Resources:

References:

Aventis Behring. von Willebrand Disease (VWD); A Patient's Guide to Understanding. 2003.
National Hemophilia Foundation. What You Should Know About Bleeding Disorders. 1997.
Paper, Renee RN, & Kelley, Laureen A. (2002). A Guide to Living With von Willebrand Disease. Aventis Behring, LLC.
Zappa, Susan C., RN. Von Willebrand Fact (or) Sheet. 2003.
Zappa, Susan C., RN, et al. Emergency Care for Patients with von Willebrand Disease.

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