Mortality attributed to sickle cell disease in children and adolescents in Brazil, 2000–2019

OBJECTIVE: Estimate rates and describe mortality trends attributed to sickle cell disease in children and adolescents in Brazil from 2000 to 2019. METHODS: This is an ecological study of the time-trend of mortality rates that used the autoregressive method, proposed by Prais-Winsten, to evaluate trends in the estimated rates of sickle cell disease deaths in children and adolescents in Brazil. Deaths with code D57 were obtained from the Mortality Information System, considering age groups (0–4, 5–9, 10–14, 15–19 years) and were used to estimate age-specific and standardized rates by gender and age. RESULTS: From 2000 to 2019, Brazil had 2,422 deaths from sickle cell disease in people under 20 years of age, with higher frequency in the Northeast (40.46%), followed by the Southeast (39.02%), Midwest (9.58%), North (7.84%), and South (3.10%). The main victims were people of Black skin/race (78.73%). In Brazil, the global standardized average rate was 0.20/100,000 people-year, with an elevation trend (annual percentage change – APC = 5.44%; confidence interval – 95%CI: 2.57–8.39). The pattern was repeated in males (APC = 4.38%; 95%CI: 2.17–6.64) and females (APC = 6.96%; 95%CI: 3.05–11.01). Elaborating age-specific rates showed that the range up to four years experienced the highest rates, without distinction by region. The age group of 15 and 19 years was the second most affected in Brazil and in the Northeast, Southeast, and Midwest regions. CONCLUSION: Deaths due to sickle cell disorders showed an elevation trend in children and adolescents. Considering that the magnitude of deaths was more evident in the first years (0–4) and late adolescence (15–19), the study suggests that age-specific approaches may impact the control of fatal outcomes caused by sickle cell disease in Brazil.