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We used the Reach, Effectiveness, Adoption, Implementation, and Maintenance (RE-AIM) framework to evaluate a Stroke Prevention Team's readiness to prevent strokes in children with sickle cell anemia living in northern Nigeria. The NIH sponsored Stroke Prevention Trial in Nigeria included a goal of a sustainable stroke prevention program. The program's 1-year reach for transcranial Doppler screening was 14.7% (4710/32,000) of which 6.0% (281/4710) had abnormal velocities (≥200 cm/s). All participants with abnormal transcranial Doppler velocities were started on hydroxyurea (effectiveness). The leaders of all 5 hospitals agreed to adopt the program. After 1 year, program-implementation and maintenance rates were 100%, demonstrating the program's feasibility and short-term sustainability., Competing Interests: M.R.D.: had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis. M.R.D.: designed the study. S.U.A. and M.A.T.: provided the TCD training. A.H.D., H.B.-M., H.I., A.T., A.A.S., B.W.J., S.G., Y.K., L.H., S.A., M.A.Z., and N.I.: administered the TCD screening. E.T. and L.J.: verified the neurological examinations. A.I.G., F.M.U., M.A.T., A.I.G., S.U.A., B.C.G., J.G., and D.L.G.: collected the data. D.L.G. and M.R.: performed the analyses. M.R.D., S.A., D.L.G., A.A.B., M.R.D.: interpreted the results. D.L.G., A.A.B., L.C.J., E.T., M.H.A., and M.R.D.: wrote the manuscript. Before submission, all authors reviewed the manuscript.The authors declare no conflict of interest., (Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc.)

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., (© 2021 Ofakunrin AOD et al.)

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., (© The Author(s) [2019]. Published by Oxford University Press.)

Background: To improve the quality of care for children with sickle cell anemia in Kano, Nigeria, we initiated a standard care protocol in 2014 to manage children with strokes at Aminu Kano Teaching Hospital., Methods: The standard care protocol requires that children with acute strokes be treated with hydroxyurea at a fixed dose of 20 mg/kg/day within two months of the stroke., Results: Twenty-nine children with sickle cell anemia and initial stroke were identified based on clinical World Health Organization criteria from 2014 to 2017. Follow-up was a median of 1.04 years (interquartile range 0.43 to 1.83 years) to either July 2017 or a second stroke, corresponding to an initial stroke incidence rate of 0.88 per 100 patient-years. Eight children had a recurrent stroke, six of whom were prescribed hydroxyurea 20 mg/kg/day by two months after initial stroke. Two children died. Six of the recurrent strokes occurred within six months of the initial stroke, two before hydroxyurea prescription. The stroke recurrence rate was 17.4 events per 100 patient-years. Adherence was approximately 60%, partly because families had to pay for hydroxyurea. Stroke incidence is probably underestimated because despite formal training for stroke detection during the quality improvement period, no participant had assessment using a standardized pediatric stroke scale and neuroimaging was not available., Conclusions: In children with sickle cell anemia, a high rate of initial and recurrent strokes exists in a low-resource setting. Ongoing needs include training to detect strokes with an objective stroke assessment and government-supported free access to hydroxyurea for stroke prevention., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Hydroxyurea (HU) is a well-known Hb F-inducing agent with proven clinical and laboratory efficacy for patients with sickle cell disease. However, concerns about its long-term safety and toxicity have limited its prescription by physicians and acceptability by patients. Thus, this study aims to evaluate clinician's barriers to the use of HU in the management of patients with sickle cell disease in Nigeria. An online survey targeted physicians in pediatrics, hematology, medicine, family medicine and general medical practice managing sickle cell disease in Nigeria. The survey was in four sections: demographic, knowledge and experience with HU, and barriers to the use of HU. Ninety-one (73.0%) of 123 contacts completed the survey. Seventy-three percent and 74.0% of the respondents noted that HU reduced transfusion rates and improved overall quality of life (QOL) of patients, respectively. While the majority of the practitioners (55.6%) see between 10-50 patients per month, most (66.7%) write <5 prescriptions for HU per month. Lack of a national guideline for use of HU, especially in children (52.0%), concern for infertility (52.0%), and safety profile of HU in pregnancy and lactation (48.2%), top the factors considered by the respondents as major barriers to the use of HU. Hydroxyurea is grossly under prescribed in Nigeria, despite that the vast majority of physicians who attend patients with sickle cell disease know about its clinical efficacy. Evidence-based clinical practice guidelines could be explored as a way to standardize practices and improve confidence of practitioners to improve physicians' prescription of HU in the management of sickle cell disease.