Perceptions and Practice of Early Diagnosis of Sickle Cell Disease by Parents and Physicians in a Southwestern State of Nigeria.

BACKGROUND: Early sickle cell disease (SCD) diagnosis has shown promise in combating SCD in many countries. The aim of this study was to assess the practice and perception of early SCD diagnosis among a group of parents and physicians in Nigeria. Patients and Methods. This was a cross-sectional descriptive study conducted to assess the opinions and practice of early diagnosis of SCD among 135 physicians caring for SCD patients and 164 mothers of children with SCD in a southwestern state of Nigeria. RESULTS: Most physicians 132 (97.8%) were aware of prenatal SCD diagnosis, but only 51 (37.8%) would recommend it. Most physicians 129 (95.6%) routinely recommend premarital SCD genetic counseling and testing, and 89 (65.1%) were aware of the national government newborn screening program but lesser proportion 75 (55.6%) were willing to recommend it. Amongst the mothers, 154 (94%) and 158 (96%) had encountered genetic counseling for SCD and were willing to offer newborn screening to their children, respectively. On the contrary, fewer mothers 42 (25%) were aware of prenatal SCD diagnosis, 28 (17%) were willing to partake in it, and 44 (26%) were undecided. There were discrepancies in the willingness by physicians to practice early SCD diagnosis and its uptake by mothers (p < 0.0001). The commonest reason given by both the physicians and mothers for not practicing SCD prenatal diagnosis was the high cost of the procedure. CONCLUSION: The perceptions and practice of early SCD diagnosis was suboptimal in the study locality. Scaling up awareness and universal coverage are required.

Evaluation of a Portable Haemoglobin Metre Performance in Children with Sickle Cell Disease and Implications for Healthcare in Resource-poor Settings.

BACKGROUND: Sub-Saharan Africa has the largest burden and worst outcome of sickle cell disease (SCD). This gloomy outlook has been attributed to the lack of use of simple and cost-effective measures for diagnosis and treatment of the disease. Although haematology analysers are the gold standard for accurate measurement of haemoglobin (Hb) concentration, they are often out of reach of most health facilities in resource-poor settings, thus creating a care gap. We conducted this study to examine the agreement between a point-of-care device and haematology analyser for determining the Hb concentration in children with SCD and its usefulness in resource-poor settings. METHODS: Ethylenediaminetetraacetic acid blood samples collected from participants were processed to estimate their Hb concentration using two devices (Sysmex KX21N haematology analyser and portable mission Hb device). The agreement between the two sets of measurements was assessed by the Bland and Altman method. RESULTS: The intraclass and concordance correlation coefficients were 0.854 and 0.936, respectively. Sensitivity and specificity were 84.2% and 98.6%, respectively. The positive and negative predictive values were 94.1% and 96.0%, respectively. The Bland and Altman's limit of agreement was -2.3 to 1.6 and the mean difference was -0.34 with non-significant variability between the two measurements (p = 0.949). CONCLUSION: Hb concentration determined by the portable testing system is comparable with that determined by the haematology analyser. We recommend its use as a point-of-care device for determining Hb concentration of SCD children in resource-poor settings where haematology analysers are not available.