Researchers discovered that the estimated mortality rate of children with sickle cell disease (SCD) living in sub-Saharan Africa was lower than previous estimates and published their results in The Lancet Haematology.

A model estimated that 230,000 infants with SCD are born in sub-Saharan Africa out of an estimated total of 312,000 worldwide, which accounts for around 80% of the world’s SCD population. Assumptions remain that children with SCD in sub-Saharan Africa are more likely to die from the disease during early childhood.

However, childhood deaths due to SCD are difficult to estimate because of the lack of systematic screening at birth and registry upon death. The authors of the study hence set out to estimate early childhood mortality due to SCD in 5 sub-Saharan countries.

The 5 countries investigated were: 

  • Burkina Faso 
  • Democratic Republic of the Congo 
  • Côte d’Ivoire
  • Mali 
  • Senegal 

Read more about SCD epidemiology

The researchers recruited women with SCD from these countries who had at least 1 child with the sickle-cell hemoglobin phenotype. Children who were sickle cell carriers or had sickle beta-thalassemia were excluded. There were 1315 affected families recruited for this study, with an additional 1243 recruited for the control group.

The results demonstrated that the mortality rate of children with SCD under the age of 1 year was 15.3% and that of children with SCD under the age of 5 years was 43.3%. The mortality rate observed in this study is in stark contrast with the mortality rate of children with SCD in Europe or North America, where more than 90% of patients reach the age of 18 years. In London, for example, the 20-year survival rate in a cohort of patients with SCD was 99.0%.

However, the results of this study still defied expectations—some scientists estimate that most children with SCD in sub-Saharan Africa will die before their fifth birthday. This discrepancy may be in part due to this study’s focus on city populations, where better healthcare programs exist compared to rural areas.

“Because this study provides an estimate of mortality in a specific population, there is a need to fund prospective studies including children diagnosed with sickle cell disease at birth, especially for patients living in rural areas,” the authors concluded.

Reference

Ranque B, Kitenge R, Ndiaye DD, et al. Estimating the risk of child mortality attributable to sickle cell anaemia in sub-Saharan Africa: a retrospective, multicentre, case-control studyLancet Haematol. 2022;9(3):e208-e216. doi:10.1016/S2352-3026(22)00004-7