Hemophilia: A Story of Success—Disaster and the Perseverance of the Human Spirit. Part 1: Background and Overview

Abstract

Until the 1980s, persons with hemophilia were a small, insular, and relatively unknown population within the United States. They generally lived quiet lives, attempted to blend in with the general population, and went about their lives while coping with a physically devastating illness in relative anonymity. Through the first eight decades of this century, hemophilia was an obscure, misunderstood, and basically unknown disease to the society at large. This situation remained true until the outbreak of the AIDS epidemic and of the mass infection of persons with hemophilia through the infusion of HIV-tainted blood products. It is estimated that 50% of persons with hemophilia are currently infected with HIV, and among those with the most severe form of hemophilia, more than 90% are HIV infected. Also, as a result of infusion of tainted blood products, more than 90% of all persons with hemophilia are infected with hepatitis C. This makes the hemophilia community the largest HIV/HCV coinfected group in the United States. There are two types of hemophilia. Of those with hemophilia, 80% have classic hemophilia (type A), in which the eighth factor in the clotting cascade is deficient or virtually nonexistent. The remaining 20% of persons with hemophilia have type B, in which the ninth factor in the clotting cascade is deficient. Within these two types of hemophilia, persons are classified as having severe hemophilia (60% of cases), moderate hemophilia (15% of cases), or mild hemophilia (25% of cases). These classifications are based on the severity of the factor VIII or factor IX deficiency that exists. The latest data from the Centers for Disease Control and Prevention report the incidence of hemophilia at 1 per 5,032 male births in the United States. Worldwide, the incidence of hemophilia is estimated to be 15 to 20 per 100,000 male births. Statistics from the hemophilia treatment centers indicate that there are currently 13,321 persons with hemophilia A and 3,638 persons with hemophilia B in the United States.