SICKLE CELL DISEASE MORTALITY IN BRAZIL: A SINGLE-CENTER EXPERIENCE

Abstract

Introduction: Sickle cell disease (SCD) is one of the most frequent genetic disorders in the world, making it a public health problem in several countries. Nevertheless, there are still few data regarding mortality of this population in Brazil. Methodology: This is a retrospective, epidemiological and descriptive study of mortality from sickle cell disease at the regional level. The sample consisted of living and deceased patients followed at the hemoglobinopathies outpatient clinic at the Reginal Blood Center of Ribeirão Preto between the years 2005 and 2020. The data were obtained from physical or electronic medical records linked to the Medical School of Ribeirão Preto Clinical Hospital. Results: 54 deaths occurred among 641 patients (37 in SS, 6 in SB0, 10 in SC and 1 in SD). The overall death rate was 8.4% and the annual general death rate was 0.56%. Surprisingly, the mortality rate among children below 5 years old was zero, and 1.8% in children below 18 years old. Patients with HbSS/SB had a higher death rate (9.4%, 0.62%/year) compared to SC patients (5.7%, 0.38%/year, p = 0.005). The mean age at death was 38.6/36.6 years for the SS/SB group and 44 years for the SC group. Prior to death, patients had a mean of 2.4 complications, 3.5 in the SS/SB group and 1.7 in the HbSC group. The most common cause of death was acute chest syndrome (50%), followed by pulmonary thromboembolism and sepsis (9.20% each). Discussion: Despite advances in the understanding of pathophysiology, causes of death and treatment, morbidity and mortality in SCD still remain a clinical challenge, especially in middle-income countries such as Brazil. Nevertheless, our results are more similar to high-income countries than published data from other regions in Brazil. Neonatal screening, antibiotic prophylaxis, close follow up since diagnosis and early treatment may have contributed to the reduction of mortality in our sickle cell population. Conclusions: In our centre, young patients with SCD have a low mortality rate, comparable to what is observed in high-income countries. SS/SB patients, as expected, present a higher mortality rate than SC patients. Herein, we believe to have shown that in Brazil it is possible to improve survival rates in patients with SCD.