LAUSR

Sickle cell disease (SCD) is a group of hereditary chronic diseases with a substantial impact on quality of life and morbimortality. In Brazil, it is one of the most common hereditary diseases; however, there are sparse epidemiological data for the country. Using data from Death Certificates, we aimed to estimate, the median age at death, years of life lost due to SCD and the median survival. From 2015 to 2019, we identified 3,320 records of deaths of individuals with SCD, from a total of 6,553,132 records. Among individuals with SCD, the median age at death was 37 years younger than the general population (SCD: 32.0 [IQR 19.0 - 46.0]; general population: 69.0 [IQR 53.0 - 81.0]). Results were consistent when stratified by sex or race. Over the five years evaluated, crude death rates varied from 0.30 to 0.34 per 100,000 inhabitants (mean 0.32). We estimate a prevalence of 60,017 individuals living with SCD (29,02 cases per 100,000) and an average incidence of 1,362 cases yearly. The median estimated survival was 40 years among individuals with SCD and 80 years for the general population. SCD was associated with an increased risk of mortality in most age ranges. Between 1 and 9 years and 10 and 39 years, the risk of death was 32 and 13 times higher in individuals with SCD, respectively. The most common causes of death were sepsis and respiratory failure. These results highlight the burden of SCD in Brazil and the necessity of improved care for this population.