Mortality trends and sociodemographic factors associated with early death in sickle cell disease patients in the state of São Paulo.

OBJECTIVE: To estimate trends in mortality rate and average age of death, and identify sociodemographic factors associated with early death in patients with sickle cell disease (SCD). METHODS: An ecological and cross-sectional study was conducted using data from the Mortality Information System. All deaths of patients residing in the state of São Paulo from 1996 to 2015 with at least one International Disease Code for SCD in any field of the death certificate were included. Simple linear regression was used to estimate trends. The Log-rank test and multiple Cox regression were used to identify factors associated with early death. RESULTS: The age-standardized mortality rate per million inhabitants increased by 0.080 per year (R2=0.761; p<0.001). When the events were stratified by age at death, the increase was 0.108 per year for those occurring at age 20 years or older, (R2=0.789; p<0.001) and 0.023 per year for those occurring before age 20 years old (R2=0.188; p=0.056). The average age at death increased by 0.617 years (7.4 months) per year (R2=0.835; p<0.001). Sociodemographic factors associated with early death identified were male gender (hazard ratio - HR=1.30), white race (HR=1.16), death occurring in the hospital (HR=1.29), and living in the Greater São Paulo (HR=1.13). CONCLUSIONS: The mortality rate and the average age of death in patients with SCD have increased over the last two decades. Sociodemographic factors such as gender, race, place of occurrence, and residence were found to be associated with early death.

Mortality trends and sociodemographic factors associated with early death in sickle cell disease patients in the state of São Paulo / Tendência da mortalidade e fatores sociodemográficos associados à morte precoce em pacientes com doença falciforme no estado de São Paulo

ABSTRACT Objective: To estimate trends in mortality rate and average age of death, and identify sociodemographic factors associated with early death in patients with sickle cell disease (SCD). Methods: An ecological and cross-sectional study was conducted using data from the Mortality Information System. All deaths of patients residing in the state of São Paulo from 1996 to 2015 with at least one International Disease Code for SCD in any field of the death certificate were included. Simple linear regression was used to estimate trends. The Log-rank test and multiple Cox regression were used to identify factors associated with early death. Results: The age-standardized mortality rate per million inhabitants increased by 0.080 per year (R2=0.761; p<0.001). When the events were stratified by age at death, the increase was 0.108 per year for those occurring at age 20 years or older, (R2=0.789; p<0.001) and 0.023 per year for those occurring before age 20 years old (R2=0.188; p=0.056). The average age at death increased by 0.617 years (7.4 months) per year (R2=0.835; p<0.001). Sociodemographic factors associated with early death identified were male gender (hazard ratio — HR=1.30), white race (HR=1.16), death occurring in the hospital (HR=1.29), and living in the Greater São Paulo (HR=1.13). Conclusions: The mortality rate and the average age of death in patients with SCD have increased over the last two decades. Sociodemographic factors such as gender, race, place of occurrence, and residence were found to be associated with early death.

RESUMO Objetivo: Estimar as tendências da taxa de mortalidade e da idade média de morte e identificar os fatores sociodemográficos associados ao óbito precoce em pacientes com doença falciforme (DF). Métodos: Estudo ecológico e transversal realizado com dados do Sistema de Informações sobre Mortalidade. Foram incluídos todos os eventos de óbitos de pacientes residentes no estado de São Paulo de 1996 a 2015, que continham pelo menos um Código Internacional de Doenças para DF, em qualquer campo do atestado de óbito. As tendências foram estimadas por meio da regressão linear simples. Para a identificação dos fatores associados ao óbito precoce, foram realizadas análises de sobrevida, por meio da regressão de Cox simples e múltipla. Resultados: A taxa de mortalidade, padronizada pela idade, por milhão de habitantes, aumentou 0,080 ao ano (R²=0,761; p<0,001). Quando os eventos foram estratificados por idade do óbito, naqueles que ocorreram com 20 anos ou mais, o aumento foi de 0,108 ao ano (R²=0,789; p<0,001) e, nos que ocorreram antes de 20 anos, foi de 0,023 ao ano (R²=0,188; p=0,056). A idade média ao morrer aumentou 0,617 ano por ano (R²=0,835; p<0,001). Os fatores associados ao óbito precoce identificados no modelo múltiplo foram: sexo masculino (hazard ratio — HR=1,30), raça branca (HR=1,16), morte dentro do hospital (HR=1,29) e moradia na Grande São Paulo (HR=1,13). Conclusões: Houve aumento da taxa de mortalidade e da idade média de óbito com DF nas duas últimas décadas estudadas. Os fatores sociodemográficos sexo, raça, local de ocorrência e município de residência estiveram associados com a faixa etária do óbito.

Chronic transfusion therapy effectiveness as primary stroke prophylaxis in sickle cell disease patients

Abstract Introduction About 10% of sickle cell anemia patients will have ischemic stroke. Adams showed stroke incidence reduction in children receiving monthly erythrocyte transfusions by reducing transcranial Doppler (TCD) velocities. Since then, chronic transfusion is recommended as primary stroke prophylaxis. This study aims to assess the effectiveness of chronic transfusions as stroke prophylaxis. Method Retrospective study, reviewing medical records from 15 sickle cell anemia patients undergoing chronic transfusion. Data collected were age, sex, adverse reactions, stroke, hemoglobin, reticulocytes, ferritin, HbS and TCD values (baseline, after 12 and 24 months of treatment). Results The mean age was 118.67 ± 41.40 months; six patients experienced allergic reactions. No stroke was recorded. One patient had alloimmunization. There was a decrease in the HbS rate and an increase in hemoglobin values in the first 12 months. Values were maintained after 24 months, but with no improvement of data. Before treatment, the mean HbS rate was 75.18%±11.69; after 12 months, 41.63 ± 14.99 and after 24 months, 43.78 ± 10.6. Thirteen patients initiated chelation after 12 months from the beginning of chronic transfusions and ferritin decline after 24 months. Pre-transfusional TCD velocities were 204.28 ± 9.41 cm/s (right) and 198.85 ± 33.37 cm/s (left). After a 12-month treatment, these values were 158.5 ± 28.89 cm/s and 157.62 ± 34.43 cm/s, respectively, and this reduction was statistically significant (p = 0.002 right and p = 0.02 left). After 24 months, these values were 149.63 ± 26.95 cm/s (right) and 143.7 ± 32.27 cm/s (left). Conclusion Significant reduction of TCD velocity occurred after treatment with chronic transfusion in sickle cell anemia patients, leading to a normal or conditional test and reducing stroke risk in all but one patient.

Chronic transfusion therapy effectiveness as primary stroke prophylaxis in sickle cell disease patients.

INTRODUCTION: About 10% of sickle cell anemia patients will have ischemic stroke. Adams showed stroke incidence reduction in children receiving monthly erythrocyte transfusions by reducing transcranial Doppler (TCD) velocities. Since then, chronic transfusion is recommended as primary stroke prophylaxis. This study aims to assess the effectiveness of chronic transfusions as stroke prophylaxis. METHOD: Retrospective study, reviewing medical records from 15 sickle cell anemia patients undergoing chronic transfusion. Data collected were age, sex, adverse reactions, stroke, hemoglobin, reticulocytes, ferritin, HbS and TCD values (baseline, after 12 and 24months of treatment). RESULTS: The mean age was 118.67±41.40 months; six patients experienced allergic reactions. No stroke was recorded. One patient had alloimmunization. There was a decrease in the HbS rate and an increase in hemoglobin values in the first 12months. Values were maintained after 24months, but with no improvement of data. Before treatment, the mean HbS rate was 75.18%±11.69; after 12months, 41.63±14.99 and after 24months, 43.78±10.6. Thirteen patients initiated chelation after 12months from the beginning of chronic transfusions and ferritin decline after 24months. Pre-transfusional TCD velocities were 204.28±9.41cm/s (right) and 198.85±33.37cm/s (left). After a 12-month treatment, these values were 158.5±28.89cm/s and 157.62±34.43cm/s, respectively, and this reduction was statistically significant (p=0.002 right and p=0.02 left). After 24months, these values were 149.63±26.95cm/s (right) and 143.7±32.27cm/s (left). CONCLUSION: Significant reduction of TCD velocity occurred after treatment with chronic transfusion in sickle cell anemia patients, leading to a normal or conditional test and reducing stroke risk in all but one patient.