Effectiveness and Safety of Hydroxyurea in the Treatment of Sickle Cell Anaemia Children in Jos, North Central Nigeria.

BACKGROUND: Hydroxyurea has been shown to positively modify sickle cell disease pathogenesis, but its use is low among Nigerian sickle cell anaemia (SCA) patients because of effectiveness and safety concerns. METHODS: We conducted a quasi-experimental study to evaluate the effectiveness and safety of hydroxyurea in 54 SCA children aged 4-17 years. Clinical and haematological parameters were compared at baseline and 12 months after hydroxyurea therapy. The participants were monitored for adverse events. The parameters were compared using relative risk and Wilcoxon Signed-Rank Test. RESULTS: The number of subjects who had more than two episodes of painful crises reduced from 27 (50%) to 2 (2.7%) (p < 0.001), while those who had acute chest syndrome reduced from 6 (11.1%) to 0 (0.0%; p < 0.001). The risk of being transfused more than once was 0.11 times the risk in the 12 months period preceding therapy (95% CI = 0.02-0.85; p = 0.016). Similarly, the risk of hospital stay >7 days was 0.08 times the risk at the baseline (95% CI = 0.02-0.24; p < 0.0001). The median haematocrit and percentage foetal haemoglobin increased from 26 to 28% and 7.8 to 14%, respectively (p < 0.0001). A dose-dependent but reversible leucopenia was observed among six children (11.1%), otherwise, hydroxyurea was safe in the study population. CONCLUSION: Hydroxyurea is effective and safe in SCA children in Jos, Nigeria. The findings could strengthen educational programme aimed at improving the utilization of hydroxyurea among SCA children.

Level of utilization and provider-related barriers to the use of hydroxyurea in the treatment of sickle cell disease patients in Jos, North-Central Nigeria.

BACKGROUND: Hydroxyurea is underutilized by sickle cell health-care providers in Nigeria despite available evidence of its effectiveness in reducing the manifestations and complications of sickle cell disease (SCD). OBJECTIVES: To assess the level of utilization and provider-related barriers to the use of hydroxyurea in SCD therapy in Jos, Nigeria. METHODS: A cross-sectional study conducted among 132 medical doctors providing care for SCD patients. Data on sociodemographics, utilization and barriers to hydroxyurea use were obtained. The barriers were fed cumulatively into the logistic regression model as predictors of utilization. RESULTS: Of the 132 care providers, 88 (67%) had been in medical practice for ≥6years. The level of utilization of hydroxyurea was 24.2%. The significant barriers that predicted the non-utilization of hydroxyurea included lack of expertise (OR=5.1; 95% CI=2.65-9.05), lack of clinical guidelines (OR=3.84; 95% CI=2.37-14.33), fear of side-effects (OR=0.50; 95% CI=0.22-0.68) and doubt about its effectiveness (OR=0.30; 95% CI=0.20-0.90). CONCLUSION: The level of utilization of hydroxyurea in the treatment of SCD among the care providers is sub-optimal with the lack of expertise in its use identified as the most prominent barrier. There is an urgent need for the training of sickle cell care-providers and the development of clinical guidelines on hydroxyurea use.

Serum zinc status in sickle cell anaemia children at the Jos University Teaching Hospital, Jos, Nigeria

Background: Several clinical manifestations of sickle cell anaemia (SCA) have been associated with zinc deficiency. Determining the zinc status of children with SCA in Nigeria, a country that accounts for the highest burden of the disease worldwide, will provide a template that could assist in critically appraising the need or otherwise for zinc supplementation or fortification programmes in these children. Methods: This was a cross-sectional comparative study conducted at the Jos University Teaching Hospital, Jos, Nigeria among 700 children (350 SCA patients and 350 age and sex matched hemoglobin AA controls). Serum zinc was analysed using the atomic absorption spectrophotometry. Results: The median serum zinc concentration of children with SCA was 6(3-7) µmol/l and it was significantly lower than that of the controls 8(4-9) µmol/l, p = 0.04. The prevalence of zinc deficiency in this study was 67% in children with SCA compared with 34% in the control group, (p<0.0001). The proportion of zinc deficient patients was more among children from lower socio economic class (68.5%, 35.5%) than in the upper socio economic class (38.5%, 16.3%) in both cases and controls groups respectively. Conclusion: There is a high prevalence of zinc deficiency in the study population especially among those with sickle cell anaemia. Zinc supplementation or fortification should be considered as part of intervention strategies to improve the zinc status of these children particularly those with sickle cell anaemia