National trends in the mortality of children with sickle cell disease, 1968 through 1992.

Abstract

OBJECTIVES: This paper describes national trends in mortality of children with sickle cell disease and the settings in which death occurred. METHODS: United States death certificate data from 1968 through 1992 were used to calculate mortality rates of Black children with sickle cell disease 1 to 14 years old. Deaths from trauma, congenital anomalies, and perinatal conditions were excluded. RESULTS: Between 1968 and 1992, mortality rates of Black children with sickle cell disease decreased 41% for 1- to 4-year-olds, 47% for 5- to 9-year-olds, and 53% for 10- to 14-year-olds. During 1986 through 1992, children who died before hospital admission accounted for 41% of deaths among 1- to 4-year-olds, 27% among 5- to 9-year-olds, and 12% among 10- to 14-year-olds. CONCLUSIONS: Survival of Black children with sickle cell disease has improved markedly since 1968. A substantial proportion of deaths continue to occur prior to hospital admission. Trends in sickle cell mortality can be monitored inexpensively with death-certificate data.