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

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

Susan T Paulukonis  1 , James R Eckman  2 , Angela B Snyder  3 , Ward Hagar  4 , Lisa B Feuchtbaum  5 , Mei Zhou  6 , Althea M Grant  7 , Mary M Hulihan  7
Affiliations

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

Susan T Paulukonis et al. Public Health Rep. 2016 Mar-Apr.

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.

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Figures

Figure 1
Figure 1
All-cause mortality rates for the sickle cell disease population identified through population-based surveillance (2004 through 2008), the African American population (2008),a and all populations (2008),a California and Georgia
Figure 2
Figure 2
Number of deaths for sickle cell disease patients identified using NCHS's compressed mortality reports (2004–2008),a NCHS's multiple cause of death reports (2004–2008),b and population-based surveillance data (2004–2008), California and Georgia
Figure 3
Figure 3
Number of deaths for sickle cell disease patients identified using population-based surveillance data (2004–2008), state-based multiple cause of death (MCOD) data files (2004–2008), NCHS's multiple cause of death reports (2004–2008),a and NCHS's compressed mortality reports (2004–2008),b California and Georgia
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