Contrary to international trends, the mortality rate of sickle cell disease increased in Brazil after the implementation of the neonatal screening program, probably due to improving access to diagnosis. This study aimed to assess differences in the temporal trend of the mortality rate and median age at death from sickle cell disease in Brazil, considering implemented measures to expand diagnosis, and improve health care access in-country and in the international scenario. Time series were extracted from the Brazilian Mortality Information System from 1996 to 2019. Changes in the mortality rate and median age at death were verified via segmented regression models, which were stratified by sex, region of residence, and age. Most deaths occurred in non-white people, young adults, and the Southeast and Northeast population. Sickle cell disease mortality rate increased until 2010 (13.31%; 95%CI: 6.37; 20.70), particularly in individuals aged 30 years or more (12.78%; 95%CI: 2.98; 23.53) and in the Northeast (12.27%; 95%CI: 8.92; 15.72). Most deaths occurred in the second decade of life (3.01 deaths/million), with a 59% increase in the median age of death in Brazil, from 27.6 to 30.3 years, more pronounced in females and the North Region. The observed gain in the survival of sickle cell disease in Brazil is still much lower than in developed countries and presents regional disparities, probably due to the lack of access to health care and recent treatments, such as hydroxyurea, still restricted to hematological referral centers in Brazilian capitals.
Keywords:
Sickle Cell Anemia; Mortality; Interrupted Time Series Analysis; Regression Analysis
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