Sickle cell disease: caregiver’s awareness and phenotype distribution among children presenting to children emergency of a tertiary hospital in Lagos, Nigeria

Abstract

Background: Sickle cell disease accounts for significant morbidity and mortality in sub-Saharan Africa and the burden is expected to increase further by 2050. Nigeria is known to bear the highest burden of sickle cell disease in the world with about 2.69–5% of the population affected by the disease.Aim: This study determined awareness of sickle cell disease among caregivers and phenotype distribution of children presenting to children emergency in Lagos University Teaching Hospital (LUTH), Nigeria.Methods: The study was crosssectional and descriptive in design and data was collected using a pretested, structured intervieweradministered questionnaire among 250 caregivers and children. HemoTypeSC™ rapid test kit was used to determine the hemoglobin  phenotype in whole blood of the respondents who were consecutively recruited following the caregiver’s consent. The Statistical Package for Social Sciences version 21 software was used for analysis.Univariate and bivariate analyses were carried out with a level of significance set at p ≤ 0.05.Results: The mean age of the children was 50.27±50.91 months. There were more females 141 (56.4%) than males. Almost all 242 (96.8%) caregivers did not know the children’s Hb phenotype. Most 173 (69.2%) of the children had HbAA;55(22.0%) were HbAS; 6(2.4%) were HbAC; 15 and 1 (6.0% and 0.4%) were HbSS and HbSCphenotypes respectively. Education was statistically significant with awareness of SCD (p=0.002) and awareness of SCD was statistically significant with knowledge of prevention (p<0.001) among the caregivers.Conclusion: Awareness of SCD among the caregivers of children was high, although the majority of them did not know the children’s Hb phenotype. Most of the children had HbAA with a high proportion of HbSS and HbSC phenotypes. A routine neonatal/early infant screening program for SCD is highly recommended in Nigeria for early diagnosis and prevention of SCD complications.