Impact of Medical Approach in a Limited-resources Centre on the Pattern of Sickle Cell Disease Phenotypes and its Complication in a Nigerian Hospital: A Single-center Retrospective Study

Abstract

Background: Sickle cell disease (SCD) is the commonest inherited qualitative blood disorder worldwide. Aims: To guide haematologists and other clinicians in making appropriate clinical judgement in the management of SCD patients in resource poor countries. Objective: To evaluate the demographic characteristics, clinical phenotype, laboratory parameters and complications of the enrolled patients during the period of the study. Study Design: A retrospective study. Place and Duration of the Study: Haematology and Immunology department, UNTH over 8 years period. Methodology: We enrolled 135 patients grouped into mild or severe SCD. The objective parameters were extracted from their case notes. Data analyzed using GraphPad Prism version 8. Results: Of the135 subjects, 39 (29%) had mild disease while 96 (71%) had severe disease. The mean ages in the groups were 27 and 26 years respectively (age ranged from 10 to 55years). Mean weight and frequency of crises per year between the groups were 59 and 58kg, 1.0 and 5.0 crises, respectively. Significant difference existed in the mean blood counts, F = 323.3. P < 0.0001. Renal and liver functions showed no significant derangement. Vaso-occlusive crisis (VOC) was predominant crisis. Leg ulcer (20%) was commonest complication. Significant relationship existed between frequency of crisis and number of complications, r = 0.17, P = .04, [CI = 0.003 – 0.332]. No relationship was observed between the number of blood units and frequency of crisis, r = 0.05, P = .61, [CI = -0.12 - 0.21]. Our patients were counselled at every clinic visit with proper guidance. Significant difference existed between blood pressure and proteinuria, P < .0001, [CI = 4.73 – 23.2]. Conclusion: Multidisciplinary approach improved their clinical outcome. Component blood transfusion practice is strongly advocated. We recommend further studies on the psycho-socio-economic, and environmental factors that impact on clinical phenotypes of this medical condition.