Mortality in children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil

Abstract

ObjectiveTo determine the mortality rate of children, adolescents and adults with sickle cell anemia in Rio de Janeiro, Brazil.MethodsThe number of deaths, the mortality rate and the causes of deaths in patients with sickle cell anemia who were treated and followed up at our institution for 15 years were determined and compared to data available for the Brazilian population.ResultsThe overall number of deaths was 281 patients with a mortality rate of 16.77%. Survival probability was significantly higher in females. The number of deaths and the mortality rate were age-specific with a significant increase in the 19- to 29-year-old age group. The remaining life expectancy of the patients with sickle cell anemia was less than that of Brazilians at large. The gap between the two was about 20 years for ages between one and five years with this gap decreasing to ten years after the age of 65 years. The most common causes of death were infection, acute chest syndrome, overt stroke, organ damage and sudden death during painful crises.ConclusionTo the best of our knowledge, this is the first Brazilian study in a single institution in Rio de Janeiro; the mortality rate was 18.87% among adult patients with sickle cell anemia. The mortality rates in children and adults are higher than those reported in developed countries of the northern hemisphere.

Fatores de risco para mortalidade em pacientes com doença falciforme: uma revisão integrativa

“…Regarding gender, the higher mortality rate observed for males resembles data obtained in other studies, 38,39 although several studies have also found a higher mortality rate among women, especially in the adult period, which reinforces the non-relationship between the SCD and the gender of the individuals.…”

Prevalência da doença falciforme em adultos com diagnóstico tardio

“…(12) Relatively lower blood viscosity in women, due to low levels of Hb and hematocrit, may be one of the possible causes. (9) The median age of participants in the present study was 35 (18-70) for those with HbSS, and 31 (18-82) for HbSC patients; there was no association between age and genotype. These results are similar to those found in a study of 132 African-American individuals, whose participants ranged in age from 15 -70 years, with a mean age of 34.2 years.…”

“…(7,24) However, regular treatment, adherence to treatment, family support, and lifestyle are important for reducing morbidity and mortality. (9) In the state of MS, human and technological resources are concentrated in the municipalities of Campo Grande and Dourados, revealing a low resolution for the treatment of hemoglobinopathies in health microregions. The access to the hematologist in the public network depends on the agreement of the municipality of origin of the patient with the capital, ordered by a state central of regulation, which can generate a repressed demand for consultation.…”

“…The mortality in sickle-cell anemia has significant decreased in children in the last decades, while the mortality rate for adults has gradually increased. (8,9) According to a study performed in the National Center for Health Statistics, between 1979 and 2005, there were 16,654 deaths related to SCD. The mortality rate in individuals over 19 years of age increased by 1% (p <0.001) each year, with a mean age at death of 33.4 years for men, and 36.9 years for women.…”

A case study exploring the impact on family life of two genetic conditions – severe haemophilia A and sickle cell disease

“…SCD crises can initially present in a very similar way to a joint bleed and are very painful, but it is not clear how best to help patients distinguish between the two to allow prompt treatment. Monthly blood transfusions have improved quality of life for children with SCD and have increased longevity, so that survival into old age is now the expectation for many, at least in wealthier economies [10,11] . However, this means they look forward to a future marred by organ damage and debility due to complications [1,2] .…”