Adults with congenital bleeding disorders

Patients suffering from hemophilia have been affected by two recent major events. First, the advent of lyophilized factor concentrates during the 1960-1970s greatly lessened the morbidity of moderate and severe disease. Hemophilia patients were freed frequent trips to emergency rooms, prolonged hospitalizations and dependence on transfusions of blood products. Their quality of life improved and Hemophilia Treatment Centers were established throughout the US to provide comprehensive medical and psychosocial care. Second, members of the hemophilia community who had now hoped for a more normal life began to experience the opportunistic infections that were ultimately understood as the sentinel signs of AIDS. When scientists identified the human immunodeficiency virus (HIV) and its mode of transmission via plasma-derived clotting factor concentrates became evident. By 1984, 50% of all individuals with hemophilia had become infected with the HIV virus. In 1980, hemophilia life expectancy was almost 68 years of age, but declined to 49 years in the late 1980s. An entire generation of adult hemophilia patients became affected by the HIV epidemic and also the hepatitis C virus. Currently, there is a growing number of young adults with hemophilia, born after 1990, where hemophilia factor replacement therapy has proven to be safe. The risk of acquiring an infection through the use of these factors is extremely low; the last documented concentrate mediated HIV transmission occurred in 1987. The challenge remains to prevent the crippling effects of hemophilia while focusing attention on the health and wellness of these individuals.