Child mortality from sickle cell disease in Nigeria: a model-estimated, population-level analysis of data from the 2018 Demographic and Health Survey

Obiageli E Nnodu,Assaf P Oron,Alayo Sopekan,Godwin O Akaba,Frédéric B Piel,Dennis L Chao

doi:10.1016/s2352-3026(21)00216-7

Abstract

SummaryBackgroundChild mortality from sickle cell disease in sub-Saharan Africa is presumed to be high but is not well quantified. This uncertainty contributes to the neglect of sickle cell disease and delays the prioritisation of interventions. In this study, we estimated the mortality of children in Nigeria with sickle cell disease, and the proportion of national under-5 mortality attributable to sickle cell disease.MethodsWe did a model-estimated, population-level analysis of data from Nigeria's 2018 Demographic and Health Survey (DHS) to estimate the prevalence and geographical distribution of HbSS and HbSC genotypes assuming Hardy-Weinberg equilibrium near birth. Interviews for the survey were done between Aug 14 and Dec 29, 2018, and the embedded sickle cell disease survey was done in a randomly selected third of the overall survey's households. We developed an approach for estimating child mortality from sickle cell disease by combining information on tested children and their untested siblings. Tested children were aged 6–59 months at the time of the survey. Untested siblings born 0–14 years before the survey were also included in analyses. Testing as part of the DHS was done without regard to disease status. We analysed mortality differences using the inheritance-derived genotypic distribution of untested siblings older than the tested cohort, enabling us to estimate excess mortality from sickle cell disease for the older-sibling cohort (ie, those born between 2003 and 2013).FindingsWe analysed test results for 11 186 children aged 6–59 months from 7411 households in Nigeria. The estimated average birth prevalence of HbSS was 1·21% (95% CI 1·09–1·37) and was 0·24% (0·19–0·31) for HbSC. We obtained data for estimating child mortality from 10 195 tested children (who could be matched to the individual mother survey) and 17 205 of their untested siblings. 15 227 of the siblings were in the older-sibling cohort. The group of children with sickle cell disease born between 2003 and 2013 with at least one younger sibling in the survey had about 370 excess under-5 deaths per 1000 livebirths (95% CI 150–580; p=0·0008) than children with HbAA. The estimated national average under-5 mortality for children with sickle cell disease born between 2003 and 2013 was 490 per 1000 livebirths (95% CI 270–700), 4·0 times higher (95% CI 2·1–6·0) than children with HbAA. About 4·2% (95% CI 1·7–6·9) of national under-5 mortality was attributable to excess mortality from sickle cell disease.InterpretationThe burden of child mortality from sickle cell disease in Nigeria continues to be disproportionately higher than the burden of mortality of children without sickle cell disease. Most of these deaths could be prevented if adequate resources were allocated and available focused interventions were implemented. The methods developed in this study could be used to estimate the burden of sickle cell disease elsewhere in Africa and south Asia.FundingSickle Pan African Research Consortium, and the Bill & Melinda Gates Foundation.

Highlights

Sickle cell disease is caused by a mutation in the β-globin gene (HBB) that leads to the production of an abnormal form of the β-subunit of haemoglobin, HbS.[1]
Without health care targeted towards sickle cell disease, most children born with the more severe forms of the disease, such as sickle cell anaemia, which accounts for most sickle cell disease births, would not survive to adulthood.[2]
We found that child mortality from sickle cell disease is high in Nigeria and contributes to a substantial portion of the country’s under-5 mortality

Summary

Introduction

Sickle cell disease is caused by a mutation in the β-globin gene (HBB) that leads to the production of an abnormal form of the β-subunit of haemoglobin, HbS.[1]. In sub-Saharan Africa, where most births of babies with sickle cell disease take place,[6] progress has been far slower than in high-income and middle-income countries. Mobilisation of resources to address sickle cell disease in sub-Saharan Africa are hampered, in part, by an absence of agreed-upon health burden estimates. Most public statements about mortality from the disease in this region are derived from a review of published evidence,[7] suggesting that under-5 mortality of children with sickle cell disease in sub-Saharan Africa is 50–90%

Methods

Results

Conclusion