Mortality rates and age at death from sickle cell disease: U.S., 1979-2005

Improvements in survival for children with sickle cell disease (SCD) during the last 30 years have been well established. Whether similar improvements for adults with the disease have occurred is unknown. We investigated mortality rates for children and adults with SCD.

We used the National Center for Health Statistics multiple-cause-of-death files to examine age at death and calculate mortality rates from 1979 to 2005. We examined trends in mortality rates using negative binomial regression, and we examined age at death using t-tests and linear regression.

We identified 16,654 sickle cell-related deaths. Mean age at death was significantly different for males (33.4 years, 95% confidence interval [CI] 33.0, 33.7) than for females (36.9 years, 95% CI 36.5, 37.4). In a regression model controlling for gender, the mean age at death increased by 0.36 years for each year of the study. The median age at death in 2005 was 42 years for females and 38 years for males. The overall mortality rate increased 0.7% (p<0.001) each year during the time period studied. The adult (>19 years of age) mortality rate increased by 1% (p<0.001) each year during the time period studied. The pediatric mortality rate decreased by 3% (p<0.001) each year during the time period studied.

These data confirm prior findings of a significant decrease in mortality for children with SCD. The mortality rate for adults appears to have increased during the same time period. It seems unlikely that this increase is due merely to an influx of younger patients surviving to adulthood and may reflect a lack of access to high-quality care for adults with SCD.