Sickle Cell Disease Related Mortality in the United States (1999-2009)

Background: Little is known about the national outcome of children and adults with sickle cell disease (SCD) given contemporary care.

Procedure: We investigated the number of deaths, standardized crude and age-adjusted mortality rates, and causes of death among individuals with SCD across the United States during 1999-2009 according to death certificates by using a publicly available website (http://wonder.cdc.gov/). Data were compared to mortality during 1979-1998.

Results: When compared to 1979-1998, mortality significantly decreased by 61% in infants <1 year of age, by 67% in children aged 1-4 years, and by 22-35% in children aged 5-19 years. After 19 years of age, mortality rates increased from 0.6 in the 15-19 year group to 1.4/100,000 in the 20-24 year group, corresponding to the transition period from pediatric to adult medical care, and this increase was similar during 1979-1998. Although the age groups with the highest mortality were 35-44 years for males and 45-54 years for females, there was a tendency for longer survival because there were more deaths among those individuals 55-74 years of age compared to previous years. For all individuals, the causes of deaths were cardiac disease (31.6%), respiratory (28.1%), renal (16.4%), infectious (14.4%), neurologic (11.9%), and gastrointestinal and hepatobiliary (9.2%) in nature. Cancer was the cause of death in <1%.

Conclusion: Mortality during childhood has decreased significantly. However, the transition period from pediatric to adult care is critical. Risk-reduction, monitoring, and early treatment intervention of cardiovascular disease in adults is warranted.