The guidelines include recommendations on screening, diagnosis, disease management, and directions for future research. An extensive article on the guidelines is published online Feb. 29 in the journal Haemophilia.
In VWD, low or absent levels of a blood protein called von Willebrand factor affect the blood’s ability to clot. Von Willebrand factor also carries with it clotting factor VIII, another important protein that helps blood clot. VWD is typically milder but more common than another bleeding disorder, hemophilia. However, for some people with VWD, the condition can pose serious health risks, especially during surgery, following an injury, or during childbirth. VWD affects 1 out of every 100 to 1,000 people - both males and females. Hemophilia mainly affects males.
“These are the first guidelines on von Willebrand Disease published in the United States and we are pleased to offer clinicians science-based recommendations in the evaluation and treatment of patients,” said NHLBI Director Elizabeth G. Nabel, M.D. “The disease can be difficult to diagnose, especially in women of child-bearing age and in children, and the danger of excessive bleeding is often under-recognized.”
In consultation with the American Society of Hematology, the NHLBI convened an expert panel on VWD whose members were selected for their scientific and clinical knowledge and experience, including specialists in hematology as well as family medicine, obstetrics and gynecology, pediatrics, internal medicine, and laboratory sciences. A draft of the guidelines was posted on the NHLBI Website for a 30-day public comment period in September 2006.
Screening recommendations in the guidelines call for physicians to take a complete medical history and physical examination, and order a sequence of blood tests to evaluate persons whose history suggests VWD or any bleeding disorder.
The symptoms of VWD include frequent large bruises from minor bumps or injuries, frequent or hard-to-stop nosebleeds, extended bleeding from the gums after a dental procedure, heavy bleeding after a cut or after surgery, and heavy or extended menstrual bleeding in women.
“The guidelines provide physicians with recommendations for diagnosing and treating the three major types of VWD,” said William L. Nichols, Jr., M.D., associate professor of medicine at the Mayo Clinic in Rochester, Minn., and chairman of the NHLBI expert panel that developed the guidelines. “While von Willebrand Disease cannot be cured, it can be treated. Proper diagnosis is important, and with the right treatment plan, even people with type 3 VWD, the most serious form, can live active lives.”-NIH/National Heart, Lung and Blood Institute
