Bleeding Blood Disorder Library Resources

von Willebrand disease (VWD) is the single most common bleeding disorder, affecting up to 1 to 2 percent of the US population (CDC UDC, 2002) and accounting for 80 to 85 percent of all bleeding disorders (DiPaola J, et al, 2001). While it affects both men and women, the consequences of VWD are more dire for women because of menstruation, pregnancy and childbirth (AH James and Jamison, MG, 2007). Yet the majority of VWD cases are undiagnosed in women and girls due to the lack of awareness among providers and patients. Often bleeding symptoms manifest in the reproductive tract and the condition is misdiagnosed as a gynecologic problem rather than an underlying bleeding disorder. Menorrhagia, acute or chronic, severe periods, is the most common symptom that women with bleeding disorders experience. A significant portion of women with menorrhagia have VWD. Of women with menorrhagia, the prevalence of VWD has been reported to be 5-20% and of adolescents with menorrhagia, the prevalence of VWD has been reported to be 5-36% (James AH, 2009).

The "hidden" nature of VWD and women's bleeding disorders is evidenced by the fact that so few women and girls who are affected have been identified and diagnosed. Even in the US, only about 9,000 women and girls with bleeding disorders have been diagnosed and connected to the network of 127 federally funded treatment centers across the country (CDC HDS, 2008). The Centers for Disease Control and Prevention (CDC) studied women already diagnosed and found that the average interval between a woman's first seeking medical help for VWD symptoms and receiving an accurate diagnosis is 16 years (A Kirtava, et al, 2004). In addition, the National Hemophilia Foundation-Harris interactive survey on women with bleeding disorders (2005) showed that 91 percent of women had never heard of VWD, yet 31 percent documented experiencing menorrhagia, "heavy" periods, a major symptom of VWD.

Women and girls with VWD and with rare bleeding disorders experience excessive bleeding related to menstruation, childbirth or other gynecologic issues. If these issues are ignored, the patient can encounter life-threatening complications such as postpartum hemorrhage, a 5-fold increased risk of transfusion and maternal mortality rates 10 times higher than those for other women (James AH and Jamison MG, 2007). 

With proper treatment, women and girls with bleeding disorders can lead normal lives. In 2008, the National Heart, Lung and Blood Institute (NHLBI) published practice guidelines for diagnosis and management of VWD for primary care providers and specialists. There is a need to raise awareness and educate healthcare providers about these guidelines to minimize the complications of VWD. Also, it is important to build upon these guidelines, improve the quality and availability of laboratory testing for VWD and enhance therapies necessary to control bleeding.