Sujet(s)
Drépanocytose , Antidrépanocytaires , Hydroxy-urée , Humains , Drépanocytose/traitement médicamenteux , Drépanocytose/complications , Hydroxy-urée/usage thérapeutique , Afrique subsaharienne/épidémiologie , Enfant , Antidrépanocytaires/usage thérapeutique , Enfant d'âge préscolaire , Adolescent , Femelle , MâleRÉSUMÉ
Oral iron supplementation in iron deficient children with sickle cell anemia and normal transcranial Doppler ultrasound (TCD) velocities does not reduce arterial flow in the middle cerebral artery.
Sujet(s)
Drépanocytose , Accident vasculaire cérébral , Enfant , Humains , Vitesse du flux sanguin , Drépanocytose/complications , Drépanocytose/imagerie diagnostique , Échographie-doppler transcrânienne , Circulation cérébrovasculaireRÉSUMÉ
In high-income countries, premarital genetic counseling for Sickle Cell Disease (SCD) is a standard practice. However, in Nigeria, there is no formal premarital genetic counseling program available for SCD. We conducted a series of focus group discussions with health care professionals, patients with SCD, and parents of the patients with or without SCD to gain an understanding of their attitudes and beliefs towards SCD/Sickle Cell Trait and premarital genetic counseling for SCD. Data were analyzed using Charmaz's constructivist grounded theory approach. Two themes were highlighted in the analysis as follows: (1) the difference between the perception of premarital sickle cell screening among individuals with SCD versus the general population, and (2) the personal beliefs and physical challenges that could lead to the avoidance of premarital screening within the general community. Lack of disease-related knowledge, testing facilities, transportation, and stigma associated with the disease were the most commonly perceived barriers to premarital testing. Also, a willingness to receive premarital testing for SCD exists within our community to reduce the spread of the disease and advocate for improved health-related quality of life of patients with SCD. The content and structure of a premarital genetic counseling program in Kano, Northern Nigeria, needs to be developed.
Sujet(s)
Drépanocytose , Conseil génétique , Humains , Nigeria/épidémiologie , Qualité de vie , Assistance , Drépanocytose/épidémiologieRÉSUMÉ
Cardiopulmonary complications remain a leading cause of morbidity and mortality in sickle cell disease (SCD). The overall goals of this study were to evaluate the relationship between left ventricular hypertrophy (LVH) and laboratory markers of hemolysis and determine the association between LVH and SCD-specific therapies (hydroxyurea and chronic red cell transfusion). Data from the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study cohort was used. LVH was defined based on the left ventricular mass indexed to the body surface area as left ventricular mass index >103.0 g/m2 for males and >84.2 g/m2 for females. There were 1,409 children included in the analysis and 20.3% had LVH. Results of multivariable analysis of LVH showed baseline hemoglobin levels were associated with the lower odds of having LVH (odds ratio [OR]: 0.71, 95% confidence interval [CI]: 0.60- 0.84). The odds of LVH increases for every 1-year increase in age (OR: 1.07, 95% CI: 1.02-1.13). Similarly, the odds of LVH were lower among males than females (OR: 0.59, 95% CI: 0.38-0.93). The odds of LVH were higher among those on hydroxyurea compared to no therapy (OR: 1.83, 95% CI: 1.41-2.37). Overall results of the study showed that LVH occurs early in children with SCD and the risk increases with increasing age and with lower hemoglobin. Further, we found higher use of hydroxyurea among those with LVH, suggesting that the need for hydroxyurea conveys a risk of cardiovascular remodeling.
Sujet(s)
Drépanocytose , Hypertrophie ventriculaire gauche , Drépanocytose/complications , Drépanocytose/thérapie , Marqueurs biologiques , Enfant , Transfusion d'érythrocytes , Femelle , Humains , Hydroxy-urée/usage thérapeutique , Hypertrophie ventriculaire gauche/complications , Hypertrophie ventriculaire gauche/étiologie , Mâle , Facteurs de risqueRÉSUMÉ
Reduced growth and delayed maturation have been described in children with sickle cell disease (SCD). This study investigated growth and hemolysis in children with SCD in the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) cohort. The database includes 5287 children, of which, 3305 had at least 2 growth measurements over a 5-year period. Body mass index was converted to z-scores (zBMI), and 19.8%, 66.1%, 14.2% of children were classified as underweight, normal, and overweight/obese, respectively. Multivariable analysis of growth was conducted and included variables: age, sex, blood pressure, hemoglobin, reticulocyte count, treatment with chronic red cell transfusion therapy (CRCT), or hydroxyurea therapy. Baseline hemoglobin levels were associated with the lower odds of being underweight (odds ratio [OR]=0.93, 95% confidence interval [CI]: 0.86-0.99), and higher odds of being overweight/obese (OR: 1.26, 95% CI: 1.17-1.36) compared with normal zBMI. CRCT was associated with being overweight/obese at baseline (OR: 1.85, 95% CI: 1.31-2.60). Overall, results showed that children who were underweight improved regardless of therapy over the 2-year time period. However, children on CRCT are at higher risk for being overweight and should be monitored closely.
Sujet(s)
Drépanocytose , Antidrépanocytaires , Drépanocytose/complications , Drépanocytose/thérapie , Indice de masse corporelle , Enfant , Hémoglobines , Humains , Obésité/complications , Surpoids , Maigreur/complications , Maigreur/étiologieRÉSUMÉ
Ischemic stroke is one of the most devastating complications of sickle cell anemia (SCA). Previous studies have shown that intracardiac shunting including patent foramen ovale (PFO) can be a potential risk factor for stroke in children with SCA. This study investigates the association between PFO and overt ischemic stroke in the DISPLACE (Dissemination and Implementation of Stroke Prevention Looking at the Care Environment) study cohort of 5,247 children with SCA of whom 1,414 had at least one clinical non-contrast transthoracic echocardiogram. Presence of PFO was taken from the clinical report. Further, we assessed the association between PFO and other clinical and hemolytic factors in children with SCA such as history of abnormal sickle stroke screen [elevated Transcranial Doppler ultrasound (TCD) velocity] and patient's baseline hemoglobin. In 642 children for whom all data were available, the adjusted odds ratio (OR) for overt stroke was higher in those with PFO but this was not statistically significant (OR: 1.49, 95% CI: 0.20-11.03, p = 0.6994). With an OR of 0.85, the study suggested less PFOs in those with abnormal TCD, but this was not statistically significant (95% CI: 0.17-4.25, p = 0.8463). Overall, the prevalence of PFO in this large sub study of non-contrast echocardiography amongst children with SCA is much lower than previous smaller studies using bubble contrast echocardiography. Overt stroke was non-statistically more common in children with SCA and PFO, but there was no evidence that PFO was more common in those with abnormal TCD, the most important pediatric sickle stroke screen.
RÉSUMÉ
Sickle cell disease is often complicated by retinopathy, which can be proliferative or non proliferative. Proliferative sickle cell retinopathy potentially leads to blindness. There is a paucity of data on sickle cell disease-related retinopathy from Africa, where the disease is most prevalent. We aimed to determine the clinical, ophthalmic, and laboratory predictors of sickle cell retinopathy in an African population. We conducted a cross-sectional study of 262 participants, aged 13 years and above, with sickle cell disease. Demographic and clinical data were collected using a structured questionnaire and standard physical examinations. Vitreo-retinal specialists performed eye examinations on all the participants. Hematological and biochemical assessments were conducted using standard methods. A multivariate stepwise forward logistic regression was performed to determine the predictors of retinopathy. The median age of the participants was 20 years (interquartile range: 17-25 years). Most of the participants had a homozygous Hb S (HBB: c.20A>T) genotype (96.9%), with 3.1% who carried a Hb S/Hb C (HBB: c.19G>A) genotype. The prevalence of non proliferative sickle cell retinopathy was 24.4%. Only 1.9% had proliferative sickle cell retinopathy (PSCR). Elevated systolic blood pressure (BP) [odds ratio (OR): 6.85, 95% confidence interval (95% CI): 1.05-44.45, p = 0.059], moderate visual impairment (OR: 5.2, 95% CI: 1.39-19.63, p = 0.015), and anterior segment changes (OR: 2.21, 95% CI: 1.19-4.13, p = 0.012) were independently predictive of retinopathy. This study provides new insight into predictors of retinopathy in sickle cell disease, with implications on early screening and prevention.
Sujet(s)
Drépanocytose , Rétinopathies , Adolescent , Adulte , Drépanocytose/complications , Drépanocytose/diagnostic , Drépanocytose/génétique , Pression sanguine , Études transversales , Humains , Rétinopathies/diagnostic , Rétinopathies/épidémiologie , Rétinopathies/étiologie , Troubles de la vision/complications , Jeune adulteRÉSUMÉ
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 [StrokePreventioninNigeria (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.
Sujet(s)
Drépanocytose/complications , Accident vasculaire cérébral/prévention et contrôle , Afrique subsaharienne , Femelle , Humains , Mâle , Accident vasculaire cérébral/épidémiologieRÉSUMÉ
Severe anaemia, defined as haemoglobin level < 6·0 g/dl, is an independent risk factor for death in individuals with sickle cell disease living in resource-limited settings. We conducted a cross-sectional study of 941 children with sickle cell anaemia, who had been defined as phenotype HbSS or HbSß0 thalassaemia, aged five to 12 years, and were screened for enrollment into a large primary stroke prevention trial in Nigeria (SPRING; NCT02560935). The main aim of the study was to determine the prevalence and risk factors for severe anaemia. We found severe anaemia to be present in 3·9% (37 of 941) of the SPRING study participants. Severe anaemia was significantly associated with the lower educational level of the head of the household (P = 0·003), as a proxy for poverty, and a greater number of children per room in the household (P = 0·004). Body mass index was not associated with severe anaemia. The etiology of severe anaemia in children living with sickle cell anaemia in Nigeria is likely to be multifactorial with an interplay between an individual's disease severity and other socio-economic factors related to poverty.
Sujet(s)
Drépanocytose/épidémiologie , Niveau d'instruction , Caractéristiques familiales , Pauvreté , Thalassémie/épidémiologie , Adulte , Drépanocytose/complications , Drépanocytose/thérapie , Enfant , Enfant d'âge préscolaire , Études transversales , Femelle , Humains , Mâle , Nigeria/épidémiologie , Accident vasculaire cérébral/épidémiologie , Accident vasculaire cérébral/prévention et contrôle , Thalassémie/thérapieSujet(s)
Drépanocytose/complications , Antidrépanocytaires/usage thérapeutique , Hydroxy-urée/usage thérapeutique , Dépistage de masse/organisation et administration , Accident vasculaire cérébral/prévention et contrôle , Échographie-doppler transcrânienne , Drépanocytose/traitement médicamenteux , Drépanocytose/épidémiologie , Enfant , Enfant d'âge préscolaire , Femelle , Humains , Nourrisson , Nouveau-né , Mâle , Dépistage de masse/méthodes , Nigeria/épidémiologie , Accident vasculaire cérébral/imagerie diagnostique , Accident vasculaire cérébral/épidémiologie , Accident vasculaire cérébral/étiologieRÉSUMÉ
INTRODUCTION: Sickle Cell Disease (SCD) is one of the most common genetic diseases in the world affecting every organ. The major challenge in the medical care of children with SCD is preventing end-organ dysfunction, particularly the brain. Major neurologic complications in children less than five years with SCD include, but are not limited to, Silent cerebral infarct, cerebral sinus thrombosis, epilepsy, reversible encephalopathy syndrome, and ischemic and hemorrhagic stroke. Recurrent headaches and migraine are not rare in children under five years with SCD. This review will focus on the neurologic complications and the description of the modifiable risk factors in children less than 5 years of age with emphasis on differences between high and low resource settings. AREAS COVERED: Neurologic complications of children under 5 years of age and the modifiable risk factors. The PUBMED database was searched using medical subject headings (MeSH) and keywords for articles regarding neurologic complications in children under 5 years of age. CONCLUSION: Neurologic complications in children under five years of age with SCD may be more frequent than currently reported, among which Silent cerebral infarct and cognitive impairment are the most common.
Sujet(s)
Drépanocytose/complications , Épilepsie/étiologie , Thromboses des sinus intracrâniens/étiologie , Accident vasculaire cérébral/étiologie , Enfant d'âge préscolaire , Humains , Nourrisson , Nouveau-né , Facteurs de risqueRÉSUMÉ
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.
Sujet(s)
Drépanocytose/traitement médicamenteux , Drépanocytose/épidémiologie , Prévention secondaire , Accident vasculaire cérébral/épidémiologie , Accident vasculaire cérébral/prévention et contrôle , Adolescent , Drépanocytose/diagnostic , Antidrépanocytaires/administration et posologie , Enfant , Enfant d'âge préscolaire , Femelle , Études de suivi , Humains , Hydroxy-urée/administration et posologie , Nourrisson , Mâle , Nigeria , Études prospectives , Récidive , Accident vasculaire cérébral/diagnosticRÉSUMÉ
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.
Sujet(s)
Drépanocytose/complications , Drépanocytose/imagerie diagnostique , Cognition/physiologie , Dysfonctionnement cognitif/imagerie diagnostique , Dysfonctionnement cognitif/diagnostic , Échographie-doppler transcrânienne/méthodes , Facteurs âges , Vitesse du flux sanguin , Enfant , Enfant d'âge préscolaire , Femelle , Identité de genre , Humains , Mâle , NigeriaRÉSUMÉ
Background: Cardiopulmonary disease is a major cause of morbidity and mortality in persons with sickle cell disease (SCD). Tricuspid regurgitant jet velocity (TRJV) and predicted forced expiratory volume in 1 s (FEV1%) predicted are independently associated with death in SCD. The goal of this study was to determine the prevalence of elevated TRJV and the association, if any, between TRJV and FEV1% predicted among persons with sickle cell anaemia (SCA) in Nigeria. Methods: Using a cross-sectional design, we enrolled 100 adult Nigerians (≥15 y) with SCA. We screened participants using Doppler echocardiogram to determine their TRJV and assessed their lung function with spirometry. Results: The prevalence of elevated TRJV was 6%, with 74% of participants having low FEV1% predicted (<70%). TRJV was negatively correlated with FEV1%, but this finding was not statistically significant (Spearman's ρ=-0.0263, p=0.8058). Conclusions: We found a low prevalence of elevated TRJV and a trend in association between TRJV and FEV1% predicted in Nigerian adults with SCA. Our findings underscore the need to explore further the relationship between SCD and cardiopulmonary disease in adults.
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Drépanocytose/épidémiologie , Hypertension pulmonaire/épidémiologie , Insuffisance tricuspide/épidémiologie , Adolescent , Adulte , Drépanocytose/complications , Comorbidité , Études transversales , Femelle , Humains , Hypertension pulmonaire/étiologie , Mâle , Adulte d'âge moyen , Nigeria , Facteurs de risque , Systole , Insuffisance tricuspide/étiologie , Capacité vitale , Jeune adulteRÉSUMÉ
BACKGROUND: Multidrug resistant tuberculosis (MDR-TB), is an emerging public health problem in sub-Saharan Africa (SSA). This study aims to determine the trends in prevalence of MDR-TB among new TB cases in sub-Saharan Africa over two decades. METHODS: We searched electronic data bases and accessed all prevalence studies of MDR-TB within SSA between 2007 and 2017. We determined pooled prevalence estimates using random effects models and determined trends using meta-regression. RESULTS: Results: We identified 915 studies satisfying inclusion criteria. Cumulatively, studies reported on MDR-TB culture of 34,652 persons. The pooled prevalence of MDR-TB in new cases was 2.1% (95% CI; 1.7-2.5%). There was a non-significant decline in prevalence by 0.12% per year. CONCLUSION: We found a low prevalence estimate of MDR-TB, and a slight temporal decline over the study period. There is a need for continuous MDR-TB surveillance among patients with TB.
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Tuberculose multirésistante/épidémiologie , Afrique subsaharienne/épidémiologie , Surveillance épidémiologique , Humains , Prévalence , Tuberculose pulmonaire/épidémiologieRÉSUMÉ
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 (â¼20 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.
Sujet(s)
Drépanocytose/complications , Drépanocytose/traitement médicamenteux , Accident vasculaire cérébral/étiologie , Accident vasculaire cérébral/prévention et contrôle , Drépanocytose/épidémiologie , Antidrépanocytaires/administration et posologie , Antidrépanocytaires/effets indésirables , Antidrépanocytaires/usage thérapeutique , Enfant , Enfant d'âge préscolaire , Femelle , Études de suivi , Hospitalisation , Humains , Hydroxy-urée/administration et posologie , Hydroxy-urée/effets indésirables , Hydroxy-urée/usage thérapeutique , Incidence , Mâle , Adhésion au traitement médicamenteux , Nigeria , Reproductibilité des résultats , Sensibilité et spécificité , Accident vasculaire cérébral/diagnostic , Accident vasculaire cérébral/épidémiologie , Résultat thérapeutique , Échographie-doppler transcrânienneRÉSUMÉ
BACKGROUND AND OBJECTIVE: Respiratory symptoms including wheezing are common in adults with sickle cell anaemia (SCA), even in the absence of asthma. However, the prevalence of spirometry changes and respiratory symptoms in adults with SCA is unknown. METHODS: Using a cross-sectional study design, we tested the hypothesis that adults with SCA (cases) would have higher rates of lower airway obstruction and wheezing than those without SCA (controls) using the American Thoracic Society Division of Lung Diseases' questionnaire. Patients were adults with SCA aged between 18 and 65 years. Controls were consecutive unselected individuals without SCA who presented to an outpatient general medicine clinic. RESULTS: We enrolled 150 adults with SCA and 287 consecutive controls without SCA. The median age was 23.0 and 27.0 years for adults with and without SCA, respectively. Cases were more likely to report cough without a cold (35.0% vs 18.6%, P < 0.001), lower forced expiratory volume in 1 s (FEV1 ) % predicted (70.1% vs 82.1%, P = 0.001) and lower forced vital capacity (FVC) % predicted (67.4% vs 74.9%, P = 0.001) than controls. In the multivariable model, wheezing was significantly associated with SCA status (OR = 1.69, 95% CI = 1.08-2.65, P = 0.024). Similarly, FEV1 % predicted was significantly associated with SCA status and wheezing (P = 0.001 for both). CONCLUSION: Adults with SCA experience a higher rate of wheezing and impaired respiratory functions compared with controls from the same region.
Sujet(s)
Obstruction des voies aériennes/épidémiologie , Drépanocytose/complications , Drépanocytose/physiopathologie , Maladies pulmonaires/épidémiologie , Bruits respiratoires/étiologie , Adulte , Obstruction des voies aériennes/complications , Études cas-témoins , Toux/étiologie , Études transversales , Femelle , Volume expiratoire maximal par seconde , Humains , Maladies pulmonaires/complications , Mâle , Prévalence , Spirométrie , Enquêtes et questionnaires , Capacité vitale , Jeune adulteRÉSUMÉ
In the United States, primary stroke prevention in children with sickle cell anemia (SCA) is now the standard of care and includes annual transcranial Doppler ultrasound evaluation to detect elevated intracranial velocities; and for those at risk, monthly blood transfusion therapy for at least a year followed by the option of hydroxyurea therapy. This strategy has decreased stroke prevalence in children with SCA from approximately 11% to 1%. In Africa, where 80% of all children with SCA are born, no systematic approach exists for primary stroke prevention. The two main challenges for primary stroke prevention in children with SCA in Africa include: 1) identifying an alternative to blood transfusion therapy, because safe monthly blood transfusion therapy is not feasible; and 2) assembling a health care team to implement and expand this effort. We will emphasize early triumphs and challenges to decreasing the incidence of strokes in African children with SCA.