Presentation (CTI)

OBJECTIVES: Sickle cell disease (SCD) is associated with high premature mortality. This study describes age at death in SCD-related deaths in Brazil and assesses temporal trends by sex and disease severity.
METHODS: Data were obtained from the Mortality Information System (SIM/DATASUS) and included deaths with SCD (ICD-10 codes D57.0 and D57.1) as the underlying cause from 2013 to 2024. Age at death was described overall and by sex and disease severity (with or without crisis). Temporal trends were assessed by sex and disease severity using linear and joinpoint regression.
RESULTS: A total of 5,355 patients were included. The overall mean age at death was 32.90y (SD: 19.77). Over time, age at death increased by 1.22% according to the Annual Percent Change (APC) (95%CI: 0.22 to 2.20; p=0.02). Females (n=2646) had mean age at death of 35.05y (SD: 20.48), with an APC of 1.28% (95%CI: 0.30 to 2.23; p=0.01). Males (n=2709) had a mean age at death of 30.79y (SD: 18.83), with an APC of 1.12% (95%CI: -0.03 to 2.20; p=0.06). In the linear regression analysis, males had, on average, a 4.29-year lower age at death than females (p<0.01). Individuals with SCD with crisis (n=1,506) had a mean age at death of 29.49y (SD: 18.59), with an APC of 1.31% (95%CI: -0.37 to 3.02; p=0.13). Among those without crises, the mean age at death was 34.23y (SD: 20.06), with an APC of 1.57% (95%CI: 0.73 to 2.39; p<0.01). In the linear regression analysis, patients with crisis had, on average, a 5.19-year lower age at death than those without crisis (p<0.01).
CONCLUSIONS: These findings highlight disparities by sex and disease severity, with lower ages at death among males and patients with crisis. The overall upward pattern may reflect improvements in care, disease management, and public health policies for SCD in Brazil.