Defining Sickle Cell Disease Mortality Using a Population-Based Surveillance System, 2004 through 2008. (March 2016)

• Record Type: Journal Article
• Authors: Paulukonis, Susan T.; Eckman, James R.; Snyder, Angela B.; Hagar, Ward; Feuchtbaum, Lisa B.; Zhou, Mei; Grant, Althea M.; Hulihan, Mary M.
• Is Part Of: Public health reports. Volume 131:Number 2(2016)
• Page Start: 367
• Page End: 375
• Publication Date: 2016-03
• Subjects: Public health -- United States -- Periodicals; 614.0973
• DOI: 10.1177/003335491613100221
• Languages: English
• ISSNs: 0033-3549

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 =5615) was more than twice as high as the count identified in compressed mortality files using SCD as the underlying cause of death alone ( n =5297). 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.