Accurate assessment of all-cause mortality and age at death requires long-term surveillance via population-based registries of patients with accurately diagnosed sickle cell disease (SCD). This according to a study comparing death rates in population-based registries with publicly available death records. Researchers found:

• 615 of 12,143 patients identified with SCD died.

• All-cause mortality rate for the SCD population was lower than the all-cause mortality rate among African Americans and similar to the total population all-cause mortality rates from birth to age 4 years.

• But, the rate was higher among those with SCD than both the African American and total population rates from ages 5 to 74 years.

• The count of deceased patients identified through population-based surveillance data (n=615) was more than twice as high as the count identified in compressed mortality files using SCD as the underlying cause of death alone (n=297).

Citation: Paulukonis ST, Eckman JR, Snyder AB, et al. Defining sickle cell disease mortality using a population-based surveillance system, 2004 through 2008. Public Health Rep. 2016 Mar-Apr;131(2):367-375.