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

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 (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.

Osteonecrosis of the femoral head in sickle cell disease: prevalence, comorbidities, and surgical outcomes in California

“…In our novel approach, we built a large SCD cohort from the socioeconomically diverse state of California, using an iterative process based on methods described in other SCD epidemiologic studies. [26][27][28][29][44][45][46] We then assessed the clinical burden of ONFH and hip postsurgical outcomes in the entire cohort, using regression techniques to identify potential predictors for ONFH diagnosis and need for hip arthroplasty.…”

“…[21][22][23][24] Many health services and outcomes research in SCD have focused on disease prevalence, incidence, and mortality, [25][26][27][28][29] but few have addressed chronic SCD complications in general, or ONFH in particular. 16,30 In our present study, we estimated the cumulative incidence and clinical burden of ONFH in patients with SCD in California, highlighted differences between SCD patients with and without ONFH, and determined the association between ONFH and other common SCD complications.…”

Long-Term Engraftment and Fetal Globin Induction upon BCL11A Gene Editing in Bone-Marrow-Derived CD34 + Hematopoietic Stem and Progenitor Cells

“…However, concerns about its potential carcinogenic properties have contributed to its underuse 9 . Current life expectancy of patients with sickle cell anemia in the United States is roughly three decades shorter than that of the general population 10, 11…”

Factors that Predict Outpatient Department Utilization amongst Sickle Cell Disease Patients in the USA

“…The average age of death for SCD patients in California and Georgia between the years 2004-2008 was 43 years old, and the SCD population aged 5-74 years had substantially higher all-cause mortality rates than African Americans and other people in the general population of similar ages [8]. Public health statistics may underestimate SCDassociated mortality, as some patients with SCD do not have SCD as the cause of death listed on their death certificates [9].…”