Defining Sickle Cell Disease Mortality Using a Population-Based Surveillance System, 2004 through 2008

Public Health Rep. 2016 Mar-Apr;131(2):367-75. doi: 10.1177/003335491613100221.

Abstract

Objective: Population-based surveillance data from California and Georgia for years 2004 through 2008 were linked to state death record files to determine the all-cause death rate among 12,143 patients identified with sickle cell disease (SCD).

Methods: All-cause death rates, by age, among these SCD patients were compared with all-cause death rates among both African Americans and the total population in the two states. All-cause death rates were also compared with death rates for SCD derived from publicly available death records: the compressed mortality files and multiple cause of death files.

Results: Of 12,143 patients identified with SCD, 615 patients died. The 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 through age 4 years, but the rate was higher among those with SCD than both the African American and total population rates from ages 5 through 74 years. The count of deceased patients identified by using 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).

Conclusion: Accurate assessment of all-cause mortality and age at death requires long-term surveillance via population-based registries of patients with accurately diagnosed SCD.

MeSH terms

  • Adolescent
  • Adult
  • Age Distribution
  • Aged
  • Anemia, Sickle Cell / diagnosis
  • Anemia, Sickle Cell / ethnology
  • Anemia, Sickle Cell / mortality*
  • Black or African American / statistics & numerical data*
  • California / epidemiology
  • Cause of Death
  • Child
  • Child, Preschool
  • Death Certificates
  • Female
  • Georgia / epidemiology
  • Humans
  • Infant
  • Infant, Newborn
  • Male
  • Medical Record Linkage
  • Middle Aged
  • Neonatal Screening*
  • Population Surveillance / methods*
  • Sex Distribution
  • Young Adult