The National Heart, Lung, and Blood Institute (NHLBI) of the National Institutes of Health has issued the first clinical guidelines in the United States for the diagnosis and management of von Willebrand Disease (VWD), the most common inherited bleeding disorder. 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.
"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." 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 guidelines address the three types of von Willebrand disease. Since heavy menstrual bleeding is the most common symptom of VWD in women, the guidelines suggest that a full gynecological exam be performed before diagnosis of VWD is made. In addition, the guidelines address the challenges that pregnancy and childbirth present for women with VWD. "Even before conception, VWD patients should consult with a hematologist and an obstetrician who specializes in high-risk pregnancies," said Andra James, M.D., assistant professor of obstetrics and gynecology in the Division of Maternal-Fetal Medicine at Duke University, and a member of the NHLBI expert panel. "Once pregnant, women with VWD should consider using a center that specializes in high-risk pregnancies." Treatment for VWD depends on its type and severity, and this makes knowing the exact type of VWD a patient has very important. Most cases of VWD are mild, and may require treatment only for surgery, tooth extraction, or injury. Medicines may be prescribed to replace von Willebrand factor, or increase the release of it into the bloodstream (desmopressin), to prevent the breakdown of clots (antifibrinolytics) or to control heavy menstrual bleeding in women (oral contraceptives).
According to the guidelines, it is important for people with VWD to avoid over-the-counter medicines that can affect blood clotting, including aspirin, ibuprofen, and other nonsteroidal anti-inflammatory drugs (NSAIDs). Those with VWD should talk to their dentist to determine if medicine is needed before dental work to reduce bleeding. Anyone over two years of age with VWD should be immunized against hepatitis A and B to decrease risks of complications from blood transfusions. It is important to exercise regularly and maintain a healthy weight. Safe exercises include swimming, biking, and walking. Football, hockey, wrestling and lifting heavy weights are not recommended for people with VWD.