National Hemophilia Foundation Applauds First von Willebrand Disease Clinical Practice Guidelines Issued by NHLBI

Common But Widely Undiagnosed Bleeding Disorder
Can Lead to Serious Health Complications

NEW YORK, NY—May 6, 2008

The National Hemophilia Foundation (NHF) welcomes and applauds the issuance of the first clinical guidelines in the United States for the diagnosis and management of von Willebrand disease (VWD), the most common hereditary bleeding disorder, by the National Heart, Lung and Blood Institute (NHLBI), part of the National Institutes of Health (NIH) and the U.S. Department of Health and Human Services. The guidelines, developed in consultation with the American Society of Hematology by a panel of multi-disciplinary medical experts and released on February 29, include recommendations on screening, diagnosis, disease management and opportunities for future research.

“Because it is under-recognized and difficult to diagnose, many people with VWD may not realize they have it, which is why the National Hemophilia Foundation has long focused efforts on educating the public and health professionals about this serious and potentially life-threatening disease,” said Howard A. Balsam, interim chief executive officer of the NHF. “We are pleased that the NHLBI guidelines will help to elevate public awareness of this important health issue and provide the medical community with evidence-based direction on how to detect and manage VWD.”

NHF provides resources on bleeding and clotting disorders to the general public through HANDI, their resource center and library of over 13,000 materials. Information specialists are available through telephone, e-mail and fax to answer questions, refer individuals to a hemophilia treatment center in their local area and provide articles, brochures and news releases regarding bleeding and clotting disorders. The library contains an extensive amount of resources particularly on von Willebrand disease and the effects of this disease and other bleeding disorders on women. Contact HANDI at 800-42-HANDI, handi@hemophilia.org or through fax at 212-328-3799.

About von Willebrand Disease
Von Willebrand disease is caused by a deficiency or abnormality of von Willebrand factor (VWF), a protein in the blood that is necessary for normal blood clotting. VWD is classified by types, ranging from Type 1 (the most common and usually mild) to Type 3 (the most severe). Patients with Type 2 VWD have VWF that does not work properly, while patients with Type 1 or Type 3 do not have enough VWF to produce a normal clot. Women with VWD are more likely to experience heavy, prolonged menstruation. Other common symptoms of VWD include frequent nosebleeds and easy bruising. Bleeding can be mild or serious and can occur as a result of injury, or without any obvious cause. More serious symptoms include bleeding into joints and internal organs. The VWD patient may require special care during dental procedures, surgery and childbirth.

NHF, in partnership with the Centers for Disease Control and Prevention (CDC) and with an educational grant from CSL Behring, has developed Project Red Flag, a national public awareness campaign to educate women and their healthcare providers about the symptoms of bleeding disorders, especially VWD, and encourage proper diagnosis and treatment. Not only was CSL Behring part of the efforts to develop the guidelines, but has been working with state bleeding disorder organizations to advocate for legislation encouraging greater screening of VWD in cases of menorrhagia. Already introduced in the Pennsylvania and Minnesota state legislatures, efforts for similar legislation are currently underway in California.

According to available statistics from the Centers for Disease Control and Prevention (CDC), VWD affects 1 to 2 percent of Americans—as many as 2.8 million people, more than half of whom are women. Women may suffer severe health consequences if their condition is not correctly diagnosed and are at greater risk for miscarriage, for life-threatening bleeding following surgery or childbirth and for undergoing unnecessary hysterectomies. Studies conducted by the CDC show that every year 30,000 women undergo hysterectomy for the diagnosis of abnormal uterine bleeding. Many of these women have an undiagnosed bleeding disorder that could be managed medically rather than surgically. According to the CDC it takes a woman, on average, 16 years to get a diagnosis for VWD.

“The NHF has long collaborated with other organizations and industry to help local chapters, partner associations, hemophilia treatment centers and consumers raise awareness of VWD in their local communities,” said Barbara A. Konkle, MD, Professor of Medicine, University of Pennsylvania, Director, Penn Comprehensive Hemophilia and Thrombosis Program, and Chair, NHF Women with Bleeding Disorders Task Force. “We’re pleased to see the Institute affirm the importance of VWD, and the need for professional guidance and consumer education around this disorder.”

Screening and Treatment
The NHLBI guidelines recommend that physicians take a complete medical history and physical examination, and order a sequence of blood tests to evaluate individuals whose history suggests VWD or any bleeding disorder.

According to the guidelines, treatment for VWD depends on its type and severity. Medicines may be prescribed to increase the release of von Willebrand factor into the bloodstream (desmopressin, administered either intravenously or through a nasal spray like Stimate^®), replace von Willebrand factor (Humate-P^® or Alphanate SD-HT^®), prevent the breakdown of clots (antifibrinolytics) or to control heavy menstrual bleeding in women (oral contraceptives).

For more information on the NHLBI guidelines go to http://www.nhlbi.nih.gov/guidelines/vwd/index.htm. For more information on VWD, you can visit the Project Red Flag Web site at www.projectredflag.org or call the National Hemophilia Foundation’s Information Resource Center at 1-800-42-HANDI (email to handi@hemophilia.org). Trained staff members are available Monday through Friday, 9 a.m. to 5:00 p.m. EST to answer your requests.

About the National Hemophilia Foundation
The National Hemophilia Foundation is dedicated to finding better treatments and cures for bleeding and clotting disorders and to preventing the complications of these disorders through education, advocacy and research.

Established in 1948, the National Hemophilia Foundation is a nonprofit 501(c)3 organization with chapters throughout the country. Its programs and initiatives are made possible through the generosity of individuals, corporations and foundations as well as through a cooperative agreement with the Centers for Disease Control and Prevention (CDC).

For more information about the National Hemophilia Foundation visit www.hemophilia.org.