Mortality from sickle cell disease in Brazil

Area of Science:

• Hematology
• Public Health
• Epidemiology

Background:

• Sickle cell disease (SCD) remains a significant health concern, with mortality rates higher than the general population despite medical advancements.
• In Brazil, understanding SCD mortality patterns is crucial for public health strategies.

Purpose of the Study:

• To evaluate the causes and independent predictors of mortality in a Brazilian SCD cohort.
• To identify factors contributing to premature death in individuals with sickle cell disease.

Main Methods:

• Analysis of the multicenter REDS-III cohort data (2013-2018) including 2,793 SCD patients.
• Comparison of deceased and surviving patients using statistical tests and multivariable Cox regression to identify mortality predictors.
• Confirmation of deaths via chart review and national death certificate linkage.

Main Results:

• A total of 159 deaths (5.7%) occurred, with a median life expectancy of 65.7 years.
• Infection (33.3%), non-infectious pulmonary conditions (25.2%), and neurologic disease (14.5%) were leading causes of death.
• Independent predictors of mortality in adults included older age, iron overload, and prior hospital admissions.

Conclusions:

• Mortality in Brazilian SCD patients occurs approximately 10 years earlier than in the general population.
• The burden of SCD mortality is increasingly affecting adults, particularly in their third and fourth decades.
• Prompt management of infections, acute chest syndrome, stroke, iron overload, and frequent hospitalizations is critical for improving survival in SCD.