Feasibility trial for primary stroke prevention in children with sickle cell anemia in Nigeria (SPIN trial)

The vast majority of children with sickle cell anemia (SCA) live in Africa, where evidence-based guidelines for primary stroke prevention are lacking. In Kano, Nigeria, we conducted a feasibility trial to determine the acceptability of hydroxyurea therapy for primary stroke prevention in children with abnormal transcranial Doppler (TCD) measurements. Children with SCA and abnormal non-imaging TCD measurements (≥200 cm/s) received moderate fixed-dose hydroxyurea therapy (∼20 mg/kg/day). A comparison group of children with TCD measurements <200 cm/s was followed prospectively. Approximately 88% (330 of 375) of families agreed to be screened, while 87% (29 of 33) of those with abnormal TCD measurements, enrolled in the trial. No participant elected to withdraw from the trial. The average mean corpuscular volume increased from 85.7 fl at baseline to 95.5 fl at 24 months (not all of the children who crossed over had a 24 month visit), demonstrating adherence to hydroxyurea. The comparison group consisted of initially 210 children, of which four developed abnormal TCD measurements, and were started on hydroxyurea. None of the monthly research visits were missed (n = total 603 visits). Two and 10 deaths occurred in the treatment and comparison groups, with mortality rates of 2.69 and 1.81 per 100 patient-years, respectively (P = .67). Our results provide strong evidence, for high family recruitment, retention, and adherence rates, to undertake the first randomized controlled trial with hydroxyurea therapy for primary stroke prevention in children with SCA living in Africa

Stroke prevalence amongst sickle cell disease patients in Nigeria: a multi-centre study

Background: Stroke is a life-changing, debilitating complication of sickle cell disease (SCD). Previous studies had recorded high stroke prevalence amongst this group of patients. Nigeria has a large population of people affected by this condition and this study aims to assess the stroke prevalence in this large population.Methodology: Stroke prevalence data from 14 physicians working in 11 tertiary health centres across the country was collated by doctors using the sickle cell registers and patient case notes. This data was then collated and used to obtain the overall stroke prevalence in adult and children.Results: The stroke prevalence in sickle cell disease patients in Nigeria was observed to be 12.4 per 1000 patients. Prevalence in the adult patients was 17.7 per 1000 patients and 7.4 per 1000 patients in children. Twenty three percent of the affected patients had more than stroke episode.Conclusion: The stroke prevalence in Nigeria is lower than previously recorded rates and further studies will be required to investigate other factors which may play a role.Keywords: sickle cell, stroke, Nigeria, prevalenc

Current knowledge and practice of exclusive breastfeeding among mothers in Gwale local government area of Kano State

This survey was conducted to determine knowledge and practice of exclusive breastfeeding, the initiation and duration of breastfeeding and weaning practices among mothers. A community-based survey was conducted on a cross section of 250 mothers with children under the age of five years in Gwale Local government Area, Kano State. They were interviewed using a pre-tested, structured and closed-ended questionnaire. Information was obtained about their socio-demographic characteristics, knowledge and practice of exclusive breastfeeding, pre-lacteal feeding, initiation and duration of breastfeeding and weaning practices. Of the 250 mothers interviewed, 77 (31%), 105 (42%) and 68 (27%) had good, fair and poor knowledge of exclusive breastfeeding respectively. The proportion of mothers who exclusively breastfed their babies after their last delivery were 26.0%, 24.8% and 22.0% and 1 month, 3 months and 6 months of infant's age respectively. Almost all mothers (96.4%) commenced breastfeeding within 24 hours of delivery. However, only 26% started within 30 minutes. Up to 23.6% of the mothers gave pre-lacteal feeds ranging from water 10.8%), glucose drinks (6.4%), honey (3.2%), cow milk (2.4%) and herbal concoctions (0.8%). Most of the respondents (75.6%) continued to breastfeed till 18-24 months of age. Twenty two percents of mothers breastfed for between 6 and 18 months and the remaining 2.4% stopped before the child was 6 months. Educated respondents were more than three times likely to practice exclusive breastfeeding compared to their uneducated counterparts. [O.R=3.7, 95% CI=1.87 to 7.54,

Associations of transcranial doppler velocity, age, and gender with cognitive function in children with sickle cell anemia in Nigeria

Children with sickle cell anemia (SCA) have elevated cerebral blood velocity relative to healthy peers. The primary aim of this study was to evaluate the association between cerebral blood velocity, measured by transcranial Doppler (TCD) ultrasound, age, and gender with cognitive function in children with SCA in Nigeria. Eighty-three children (Mage = 9.10, SD = 1.90 years; 55% female) with SCA in Nigeria completed cognitive assessments and a TCD ultrasound. The association between TCD velocity and measures of perceptual reasoning (Raven's Progressive Matrices), working memory (WISC-IV Digit Span), and executive planning (Tower of London, TOL) were assessed. Results showed that elevated TCD velocity significantly predicted lower scores on TOL Time Violations and Total Problem-Solving Time when controlling for BMI, hemoglobin level, and parent education, suggesting that TCD velocity is related to the efficiency of executive function. Further, age was negatively related to children's performance on the Ravens Matrices and TOL Total Correct, and boys showed greater deficits on the TOL Total Correct relative to girls. Moderation analyses for gender showed that there was a conditional negative association between TCD velocity and Digit Span for boys, but not for girls. Findings suggest that children with SCA in Nigeria with elevated TCD velocity are at risk for deficits in efficiency of executive planning, and boys with elevated TCD velocity are particularly at increased risk for deficits in auditory working memory. Implications of this study are important for interventions to reduce cerebral blood velocity and the use of TCD in this population

Primary prevention of stroke in children with sickle cell anemia in sub-Saharan Africa: rationale and design of phase III randomized clinical trial

Strokes in children with sickle cell anemia (SCA) are associated with significant morbidity and premature death. Primary stroke prevention in children with SCA involves screening for abnormal transcranial Doppler (TCD) velocity coupled with regular blood transfusion therapy for children with abnormal velocities, for at least one year. However, in Africa, where the majority of children with SCA live, regular blood transfusions are not feasible due to inadequate supply of safe blood, cost, and the reluctance of caregivers to accept transfusion therapy for their children. We describe the Primary Prevention of Stroke in Children with Sickle Cell Disease in Nigeria Trial [ S troke Pr evention i n N i g eria (SPRING) trial, NCT02560935], a three-center double-blinded randomized controlled Phase III clinical trial to 1) determine the efficacy of moderate fixed-dose (20 mg/kg/day) versus low fixed-dose (10 mg/kg/day) hydroxyurea therapy for primary stroke prevention; 2) determine the efficacy of moderate fixed-dose hydroxyurea for decreasing the incidence of all cause-hospitalization (pain, acute chest syndrome, infection, other) compared to low fixed-dose hydroxyurea. We will test the primary hypothesis that there will be a 66% relative risk reduction of strokes in children with SCA and abnormal TCD measurements, randomly allocated, for a minimum of three years to receive moderate fixed-dose versus low fixed-dose hydroxyurea (total n = 220). The results of this trial will advance the care of children with SCA in sub-Saharan Africa, while improving research capacity for future studies to prevent strokes in children with SCA

Sickle cell disease genomics of Africa (SickleGenAfrica) network: ethical framework and initial qualitative findings from community engagement in Ghana, Nigeria, and Tanzania

Objectives To provide lay information about genetics and sickle cell disease (SCD), and to identify and address ethical issues concerning SickleGenAfrica covering autonomy and research decision-making, risk of SCD complications and organ damage, returning of genomic findings, biorepository, data sharing, and healthcare provision for patients with SCD. Design Focus groups utilising qualitative methods. Setting Six cities in Ghana, Nigeria, and Tanzania within communities and secondary care. Participants Patients, parents/caregivers, healthcare professionals, community leaders, and government healthcare representatives. Results Results from 112 participants revealed similar sensitivities and aspirations around genomic research, an inclination towards autonomous decision-making for research, concerns about bio-banking, anonymity in data sharing, and a preference for receiving individual genomic results. Furthermore, inadequate healthcare for patients with SCD was emphasised. Conclusions Our findings revealed the eagerness of patients and parents/caregivers to participate in genomics research in Africa, with advice from community leaders and reassurance from health professionals and policy-makers, despite their apprehensions regarding healthcare systems