Abstract
BACKGROUND: The burden of Sickle cell anaemia (SCA) is huge in Sub Sahara Africa as it affects 1-2% of the population. HbSS impacts negatively on the quality of life of the sufferers. The clinical manifestations start between 3 and 5 months of life as a result of reduction in foetal hemoglobin. OBJECTIVES: This study describes the clinical and laboratory characteristics of HbSS patients at presentation in steady state, vaso-occlusive and hemolytic crises states. MATERIAL AND METHOD: This was a cross sectional, analytical study. Ninety HbSS participants were divided into three groups; steady state, hemolytic and vaso-occlusive crises with 30 individuals in each group. The survey contained sections on bio-data and past medical history obtained from the patients' notes and results of laboratory tests. Data were analysed using SPSS version 23.0. Results were considered statistically significant if p < 0.05. RESULTS: Ninety participants were analysed in this study. The mean age of the participants was 29.4 ± 8.9 years. Only one-third of the participants were diagnosed within the first year of age. Forty-seven (52.2%) participants have steady state haematocrit in the range of 21-25%. All the participants experienced bone pain in a year, about 25% of these participants had more than three episodes of pain per year. There was a statistically significant difference in the mean values of PCV (p < .001), WBC (p < .001), platelet (p = .008), ANC (p < .001), ALC (p < .001), AMC (p < .001), reticulocyte count and ISC % among the different categories. CONCLUSION: This study established the fact that only a minority of the SCD patients are diagnosed in the first year of life and vaso-occlusive crisis is the most frequent reason for hospital presentation. We therefore recommend the institutionalisation by government policy, neonatal screening programme in Nigeria.KEY MESSAGESThe study highlight delay in early diagnosis of SCA due to unavailability of neonatal diagnosis program in our setting.Bone pain remains the major cause of presentation for SCA and most patients presented after a day of onset of pain to the hospital.