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

PAULUKONIS, SUSAN T.; ECKMAN, JAMES R.; SNYDER, ANGELA B.; HAGAR, WARD; FEUCHTBAUM, LISA B.; MEI ZHOU; GRANT, ALTHEA M.; HULIHAN, MARY M.

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 (n5615) was more than twice as high as the count identified in compressed mortality files using SCD as the underlying cause of death alone (n5297). 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.

CALIFORNIA; GEORGIA; CAUSES of death; REPORTING of diseases; RESEARCH methodology; MORTALITY; NOSOLOGY; PUBLIC health surveillance; RESEARCH funding; SICKLE cell anemia; DEATH certificates; ACQUISITION of data; RETROSPECTIVE studies; DESCRIPTIVE statistics

Public Health Reports, 2016, Vol 131, Issue 2, p367

0033-3549

Academic Journal

10.1177/003335491613100221