Estimated mortality rates of individuals with sickle cell disease in Brazil: real-world evidence

Abstract

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.

Real-world evidence of the burden of sickle cell disease: a 5-year longitudinal study at a Brazilian reference center

“… 2 , 5 , 6 Cancado et al . 19 analyzed 6,553,132 deaths registered in Brazil from 2015 to 2019, of whom 3320 were individuals with SCD. Among them, the median age at death was 32.0 years old, 37 years younger than the general population (median age at death of 69 years old).…”

Insegurança alimentar e nutricional em adultos com doença falciforme: um protocolo de revisão de escopo

“…No Brasil, a mortalidade causada pelo DF ainda é elevada, especialmente em adultos jovens. No estudo de série temporal realizado entre 2015 e 2019 (2) , verificou-se que cerca de 7000 óbitos tiveram como causa básica a anemia falciforme. Entre esses óbitos a maioria (44%) eram mulheres e de cor parda ou negra (44%), predominantemente na região Sudeste e Nordeste e maiores de 34 anos, evidenciando uma menor sobrevida na terceira década de vida.…”

“…Esses fatores reduzem a qualidade de vida entre as pessoas adultas, e quando potencializadas pela baixa adesão ao tratamento, desnutrição e aspectos sociais, tais como desemprego, baixo nível socioeconômico e nível de escolaridade, torna-os mais vulneráveis às outras complicações da doença, inviabilizando a única terapêutica de cura, o Transplante de Medula Óssea (TMO) (2,8,18) e, consequentemente, aumentando o seu risco de mortalidade.…”