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Background: Primary stroke prevention programmes for children with sickle cell disease (SCD) have been shown to be feasible interventions in resource-poor countries. Different hydroxyurea (HU) regimens have been utilised in ameliorating the severity of SCD., Objective: To determine the long-term outcomes of the stroke prevention programme for children with SCD in Ibadan (SPPIBA), Nigeria., Methods: A longitudinal study of 396 children with haemoglobin SS disease who had been on the stroke prevention programme for a minimum period of 5 years. All enrollees had nonimaging TCD performed at baseline and thereafter 3-monthly or annually. Children with TCD velocities ≥170 cm/s were treated with HU by dose-escalation regimen., Results: The mean age at first TCD examination was 102 ± 46.7 months and the period of follow-up ranged from 5 to 10 years (mean = 7.2 ± 1.7). Time to significant decline in TCD velocities ranged from 5 to 35 months, (median = 10.0 months). The minimum dose of HU required to achieve significant decline in TCD velocities ranged from 15 to 31 mg/kg/day, mean 23.7 (±3.9). HU dose escalation beyond 20 mg/kg/day was required to attain significant reductions in the time-averaged mean of maximal velocities (TAMMV) in 69.1% of the cases. Two stroke events occurred giving a stroke incidence of 0.08 per 100 patient-years., Conclusion: The majority of Nigerian children with SCD and elevated TCD velocities achieved significant decline in TAMMV within the first year of HU therapy but on higher doses of HU. It might be important to individualise HU doses for optimal outcomes in primary stroke prevention., (© 2021 Wiley Periodicals LLC.)

Background: Chronic blood transfusion (CBT) diminishes the risk of primary and secondary stroke in sickle cell disease (SCD). We appraised CBT and assessed its feasibility as an option for stroke prevention in a setting of limited resources., Methods: All new cases of SCD seen in the Paediatric Hematology/Neurology units of the University College Hospital, Ibadan, Nigeria over a 5-year period were screened and followed up to identify those who had an indication for CBT for stroke prevention. Caregivers were counseled and offered CBT when indicated. Children of caregivers who accepted chronic transfusion were carefully followed up and outcomes documented., Results: Five (10%) of the caregivers of the 50 children who had an indication for CBT for stroke prevention consented to the treatment. They all had homozygous sickle cell anemia and had suffered a stroke. None of the children with abnormal TCD velocities consented to CBT. Two children experienced transfusion reactions, fatal in one. The mean annual cost of chronic transfusion (without chelation) was $3,276 (SD = 1,168). Major reasons given for declining CBT were high costs of blood transfusion, unavailability of blood, the need to regularly seek for blood donors, and the indefinite duration of blood transfusions., Conclusion: High economic costs, unavailability of blood, need to regularly seek for blood donors, cultural beliefs, and high frequency of transfusion reactions are major challenges to a successful CBT program in Nigeria. There is a need for government subsidy on blood transfusions and improved efforts towards provision of safe and affordable blood., (© 2013 Wiley Periodicals, Inc.)

Aims and Objectives: To compare the outcome after a first clinical stroke, following treatment with and without hydroxyurea (HU)., Subjects and Methods: A retrospective review of a cohort of Nigerian children with SCD, who had suffered a first stroke, was carried out. Outcomes in the group of children who received and did not receive HU were compared., Results: Thirty two children presented with stroke and one died of haemorrhagic stroke at presentation. All the children had haemoglobin SS phenotype, and ischaemic stroke was the predominant form seen. Mean age at first clinical stroke was 7 years, 7 months (SD=2 years, 4 months). Thirteen children received HU while 18 declined HU therapy. Maximum dose of HU ranged from 20-25 mg/kg/ day. The secondary stroke incidence of 7/100 person years in the HU group was significantly lower than the 28/100 person years in the non-HU group (P=0.001, OR 3.808, 95% CI 1.556, 9.317). Children who did not receive HU were more likely to drop out of school and to have moderate-severe motor disabilities requiring caregiver assistance for daily living., Conclusion: In settings where facilities for chronic blood transfusion are not accessible or feasible, HU therapy should be considered for secondary stroke prevention in children with SCD.

Sickle cell disease (SCD) is reported to be the most common genetic disorder affecting Nigerians. Children with SCD are at a high risk of neurological morbidity. The main objective of this study was to determine the pattern of adverse neurological outcomes among a cohort of Nigerian children with SCD. All children with SCD seen in the Department of Paediatrics, University College Hospital, Ibadan, Nigeria, over a period of 2 years were carefully evaluated for symptoms and signs of neurological complications, defined as clinical outcomes referable to the central nervous system. Of the 214 children evaluated, 187 were diagnosed with Hb SS disease and 27 with Hb SC disease. Neurological complications were identified in 78 (36.4 %) of the cases. The most common complications were headache (17.8 %), seizure (9.3 %) and stroke (8.4 %). Other less frequent complications included bacterial meningitis (2.8 %), spontaneous visual loss (1.4 %), paraplegia (0.9 %) and transient ischaemic attacks (0.9 %). Neurological complications occurred more frequently in children with sickle cell anaemia than in those with Hb SC disease (P = 0.002, 95 % CI 1.450-82.870). Adverse neurological events are common in Nigerian children with SCD, with a significantly higher risk in Hb SS than Hb SC disease. Stroke represents a major underlying cause of symptomatic epilepsy in SCD. Institution of primary preventive measures for stroke in SCD will significantly reduce the burden of stroke and epilepsy associated with SCD in Nigeria.

Sickle cell anaemia in children is characterised by recurrent crises that frequently involve intensive medical care which may impact on the health and well-being of their carers. The psychosocial impact of sickle cell disease on 67 carers of children with sickle cell disease attending the Paediatric Haematology/Oncology clinic of the University College Hospital, Ibadan, Nigeria, was determined between February and May 2007 using a structured questionnaire adapted from an instrument earlier validated for the study of carer burden in sickle cell disease and relevant to the Nigerian culture. Data analysis was performed using the Statistical Package for Social Sciences (SPSS) version 15.0. Demographic factors as well as frequency of hospitalisations and blood transfusions were each categorised into groups and the Mann-Whitney U-test was used to test for differences in stress scores between any two groups while the Kruskal-Wallis test was used to test for differences in more than two groups. Level of statistical significance was set at P < 0.05. Family finances were adversely affected in 39 (58.2%) families. Financial stress was frequently associated with a history of two or more hospitalisations in the previous year and more so in families with more than three children. Majority (80.6%) of the carers said they had minimal or no difficulty coping with their children. There was also a significant correlation between financial stress and difficulty in parental coping. Caring for the illnesses in the children often caused disruptions in family interactions; worst in the first year after diagnosis and improved over the years. Regular assessment of psychosocial areas of need is necessary to guide provision of necessary support.