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VWD Fact Sheet

What It Is Von Willebrand disease (VWD) is the most common hereditary bleeding disorder in the United States1. It is caused by a deficiency or abnormality of the von Willebrand factor in the blood. People with VWD bruise easily2 and experience prolonged bleeding.1

VWD is classified by types, ranging from Type 1 (the most common and mild), to Type 3 (the most severe).3
Causes and Symptoms Some patients with VWD have low or nonexistent levels of the von Willebrand factor (VWF), a protein in the blood that is necessary for normal blood clotting. In other cases, VWD presents with normal levels of VWF but the factor does not work as it should. In either case, platelets do not bind properly at the site of vessel injury3.

Women with VWD are more likely to experience heavy, prolonged menstruation. Other common symptoms of VWD include frequent nosebleeds, easy bruising and prolonged bleeding during dental procedures. More serious symptoms include bleeding into joints and internal organs and prolonged bleeding after surgery or childbirth.
Incidence and Prevalence According to the U.S. Centers for Disease Control (CDC) VWD affects 1 - 2 percent of the general population and is estimated to affect between 3 - 6 million people in the United States, more than half of whom are women1,5.

VWD is typically passed from parent to child. A man or woman with the disease has a 50 percent chance of passing the gene on to his or her child. Parents who carry the disease but do not have bleeding symptoms can pass the gene to their children.

A family history of a bleeding disorder is the primary risk factor6.
Living with VWD Bleeding can be mild or serious and can occur as a result of injury, or without any obvious cause. Individuals with more severe VWD should avoid unnecessary trauma, including contact sports.

VWD can be more complicated in women due to heavy and prolonged menstrual bleeding, which is often misdiagnosed as a gynecological problem.3

Special care may be required for the VWD patient during childbirth, surgery and dental procedures.

VWD patients typically have a normal life span.
Diagnosis and Treatment

Because VWD is usually mild, many people with this disorder either are asymptomatic or fail to report any symptoms; thus, in most cases, the condition is undiagnosed. VWD can be easily diagnosed by blood tests. Establishing a comprehensive family medical history is also essential but not always adequate to identify VWD8.

The National Heart, Lung and Blood Institute (NHLBI) recently issued the first-ever clinical guidelines for the diagnosis and treatment of VWD, providing health professionals with evidenced-based recommendations on screening, diagnosis, disease management, and directions for future research9. Treatment is aimed at raising the levels of VWF circulating in the blood stream.

Desmopressin acetate: For patients with Type 1 and some Type 2a, this medicine is either injected or inhaled to help the body release stored VWF into the bloodstream and increase levels of factor VIII.

Replacement therapy: For patients with Type 1 – 3, factor concentrate medicines are used to replace the missing VWF proteins6. Factor concentrates, such as Humate-P®, are approved by the U.S. Food & Drug Administration for use in treating adult and pediatric patients who experience spontaneous and trauma-induced bleeding episodes in severe VWD, and in mild or moderate VWD where the use of desmopressin is known or suspected to be inadequate.

Oral contraceptives: For patients with mild VWD, oral contraceptives are often used to control heavy menstrual bleeding in women.

1 Centers for Disease Control and Prevention. Hereditary Bleeding Disorders. Available at: www.cdc.gov/ncbddd/hbd/hemophilia.htm . Accessed: Oct 3, 2006.
2 Armenian S, Raffel JL, Nugent DJ, et al. Painless urethral bleeding: an unusual presentation of Von Willebrand disease. Haeomphilia. 2003 May;9(3):332-5.
3 National Heart Lung and Blood Institute. What Is… Available at: www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_WhatIs.html. Accessed: Oct 3, 2006
4 Federici AB, Castaman G, Thompson A, et al. Von Willebrand's disease: clinical management. Haemophilia. 2006 Jul;12(s3):152-8
5 U.S. Census Bureau. U.S. Population Clock Projection. Available at: www.census.gov/cgi-bin/popclock. Accessed: May 5, 2006.
6 National Heart Lung and Blood Institute. Causes. Available at: www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_Causes.html. Accessed: Oct 3, 2006
7 Chuong JC, Brenner, PF. Management Of Bleeding Disorders In Women. American Journal of Obstetrics & Gynecology. 1996 175(3):787-792
8 National Heart Lung and Blood Institute. Diagnosis. Available at: www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_Diagnosis.html. Accessed: Oct 3, 2006
9 National Heart, Lung and Blood Institute. The Diagnosis, Evaluation and Management of von Willebrand Disease. Available at: www.nhlbi.nih.gov/guidelines/vwd. Accessed: March 24, 2008
10 National Heart, Lung and Blood Institute. Treatments. Available at: www.nhlbi.nih.gov/health/dci/Diseases/vWD/vWD_Treatments.html. Accessed: Oct 3, 2006

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