The care available for SCD in Nigeria is still suboptimal and there is an urgent need for concerted effort to tackle the problem, but to make a significant impact on the burden of the disease would require more focus at the primary care level. Some steps to achieving this are outlined.

Adolescents living with SCD in Nigeria have lower health related quality of life compared to their healthy peers. They also experience stigma that impacts their HRQoL. Complications of SCD and hospital admissions contribute significantly to this impairment.

Background Sickle cell disease (SCD) is a neglected burden of growing importance. >312,000 births are affected annually by sickle cell anaemia (SCA). Early interventions such as newborn screening, penicillin prophylaxis and hydroxyurea can substantially reduce the mortality and morbidity associated with SCD. Nevertheless, their implementation in African countries has been mostly limited to pilot projects. Recent development of low-cost point-of-care testing (POCT) devices for sickle haemoglobin (HbS) could greatly facilitate the diagnosis of those affected. Methods We conducted the first multi-centre, real-world assessment of a low-cost POCT device, HemoTypeSC, in a low-income country. Between September and November 2017, we screened 1121 babies using both HemoTypeSC and HPLC and confirmed discordant samples by molecular diagnosis. Findings We found that, in optimal field conditions, the sensitivity and specificity of the test for SCA were 93.4% and 99.9%, respectively. All 14 carriers of haemoglobin C were successfully identified. Our study reveals an overall accuracy of 99.1%, but also highlights the importance of rigorous data collection, staff training and accurate confirmatory testing. It suggests that HPLC results might not be as reliable in a resource-poor setting as usually considered. Interpretation The use of such a POCT device can be scaled up and routinely used across multiple healthcare centres in sub-Saharan Africa, which would offer great potential for the identification and management of vast numbers of individuals affected by SCD who are currently undiagnosed. Funding US Imperial College London's Wellcome Trust Centre for Global Health Research (grant #WMNP P43370).

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BackgroundSickle cell anaemia is the most common genetic disorder worldwide as well as in Nigeria. Delay in the diagnosis of the condition constitutes an important cause of concern for caretakers of affected children.ObjectiveTo determine the age at diagnosis in a population of children with sickle cell anaemia in Lagos, Nigeria.MethodologyThe study was conducted between October and December 2009 at the sickle cell clinic of the Department of Paediatrics of Lagos State University Teaching Hospital, Ikeja, Lagos in South west Nigeria. By convenience sampling, a total of 192 children with sickle cell anaemia aged six months to 15 years were interviewed with the aid of a structured questionnaire.ResultsOverall, the mean age at confirmation of haemoglobin genotype was 27.33 months (± 26. 36 months). The mean age at diagnosis was significantly lower among males than females (25.59 ± 27.74 Vs. 29.14 ± 24.85, p = 0.04). A quarter of the children were diagnosed before infancy and three-quarters before three years of age. Upper social stratum and small family size were significantly associated with earlier diagnosis of sickle cell anaemia.ConclusionToo few subjects are diagnosed in infancy. Routine screening should ideally be done at birth and neonatal period or at the latest, between six and nine months.