1972, the major cause of death for individuals with hemophilia was cerebral hemorrhage. The mean life expectancy was approximately 40 years between 1941 and 1960 and approximately 54 years between 1961 and 1970; it increased to 60 years between 1971 and 1980 (Jones and Ratnoff 1991). By the early 1980s, hemorrhage was no longer the major cause of death (Levine interview). To the extent that the pain and the visible signs of a crippling disease were reduced, the stigma of hemophilia was also significantly reduced (Jones and Ratnoff 1991; Resnik 1994).

While the development of the AHF concentrate products revolutionized the treatment of hemophilia, the development of hemophilia treatment centers provided a nationwide system of care and treatment. In 1975, the federal government established funding for 20 regional centers, representing approximately 100 local treatment centers, to provide hemophilia care (Department of Health and Human Services 1994). The treatment centers ensured comprehensive medical care and early application of treatment as well as fostering a sense of community for individuals with hemophilia and their families (Smith and Levine 1984).

In the setting of the treatment center both physicians and patients were able to see the advantages of treating bleeding episodes early. This observation translated into advocating home care therapy; hemophiliac patients and their families quickly became proficient in self-infusion (Smith and Levine 1984). The results were significant—home care therapy nearly eliminated the crippling associated with the disease and led to significant improvements in the health and quality of life for the individual with hemophilia (Eyster, et al. 1980; Brinkhous 1981; Smith and Levine 1984; Rosendaal, et al. 1991; Chorba, et al. 1994).

In this context of progress in the treatment and care of hemophilia, the physicians treating hemophilia and their patients shared in a rather unique and close relationship. In part, this stemmed from the somewhat insular care network for individuals with hemophilia at the hemophilia treatment centers, and in part, it developed from sharing in the significant medical progress in hemophilia treatment. Hemophiliac patients and their families generally held their physicians in high regard and trusted them implicitly (Kasper interview). The third important factor in the treatment of the hemophilia patient in the years before the AIDS epidemic was the recognition that infection with hepatitis B as well as non-A, non-B hepatitis was a frequent risk associated with use of AHF concentrates (Aronson 1979; Johnson et al. 1985; Chorba et al. 1994; Hoyer 1994; Goldfinger, Kasper, Louvrein, Roberts interviews). This observation can be traced to the early 1970s when hepatitis emerged as a frequent complication in patients receiving blood and blood products (Seeff 1988). During this era, it came to be expected that most hemophiliac patients would develop hepatitis (Chorba, et al. 1994; Hoyer interview). Although hepatitis infections were occasionally severe, leading to liver failure and death (Aronson 1979; Johnson, et al. 1985), the benefits of treatment seemed to outweigh the risks from such



The National Academies | 500 Fifth St. N.W. | Washington, D.C. 20001
Copyright © National Academy of Sciences. All rights reserved.
Terms of Use and Privacy Statement