CHANGES IN LONGEVITY AND CAUSES OF DEATH AMONG PERSONS WITH HEMOPHILIA-A

To examine recent changes in longevity and the causes of death among p ersons with hemophilia A, we evaluated death certificate data for pers ons who died in the United States from 1968 through 1989 and had hemop hilia A or congenital Factor VIII disorder (ICD code 286.0) listed on the death certificate as one of the multiple causes of death. Multiple -cause-of-death mortality data for the United States from 1968 to 1989 were examined to compare death rates by year, focusing on death rates and causes of death for 1979-1981, 1983-1985, and 1987-1989. Gender, age group, race, geographic region, and median age at death of persons with hemophilia A and human immunodeficiency virus (HIV)-related dise ase listed as a cause of death were compared with those with hemophili a A without HIV-related disease. From 1968 through 1989, 2,792 hemophi lia A deaths were reported. The death rate increased from 0.5 to 1.3 p er 1,000,000 persons. From 1979-1981 through 1987-1989, mortality incr eased in all age groups above 9 years of age and age at death shifted markedly to lower ages. Median age at death decreased from 57 years in 1979-1981 to 40 years in 1987-1989. The percentage of deaths due to h emorrhage or diseases of the circulatory system decreased markedly as the result of the increase in deaths associated with HIV infection or infections other than HIV infection. Spread of HIV-1 infection in pers ons with hemophilia A has disrupted the reduction in mortality seen wi th factor replacement therapy, implementation of home care, and use of comprehensive hemophilia treatment centers. It is hoped that advances in the care of HIV-infected persons will improve survival in the hemo philia community. (C) 1994 Wiley-Liss, Inc.