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Introduction: Academic achievement is crucial for advancing learning goals. For disease-related and possibly additional reasons, children with sickle cell anaemia (SCA) in Sub-Saharan Africa (SSA) may be at risk of disease-associated school difficulties. Limited data exist on academic achievement of school-age children with SCA in the region. We aimed to assess academic achievement of children with SCA in Uganda. Methods: This cross-sectional study was conducted in Kampala, Uganda. Participants were school-going children with SCA, aged 6-12 years, attending Mulago Hospital SCA Clinic, and age-matched sibling controls without SCA. Academic achievement was assessed using the Wide Range Achievement Test, Fourth Edition (WRAT4) using outcome measures of spelling, mathematical computation, word reading, and sentence comprehension by age-normalized Z-scores. Results: Among 68 SCA and 69 controls tested, mean age was 9.4 (SD2.0) years; with males 55.9% and 46.4%, respectively. Mean haemoglobin was 7.9 (0.)g/dL in the SCA group versus 12.8 (0.)g/dL in the controls, (p<0.001). Children with SCA scored lower in spelling, (mean difference [95% confidence interval] -0.36 [-0.02 to -0.69], p=0.04) and mathematical computation, (mean difference [95% confidence interval] -0.51 [-0.17 to -0.85], p=0.003) than the controls. No differences by group were found in word reading or sentence comprehension. In the SCA group, lower scores in spelling correlated with age, and males performed better than females in mathematical computation. Discussion: School-aged children with SCA were at risk of poor performance in spelling and mathematical computation. Our findings suggest their need for educational evaluation and support, especially in these two areas, in SSA.

Megan Askew

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Background Adolescent and young adult (AYA) women with sickle cell disease (SCD) have increased pregnancy-related health risks and are prescribed potentially teratogenic medications, yet little is known about pediatric SCD provider contraceptive practices. We aimed to assess pediatric hematology providers’ beliefs, practices, motivators, and barriers for providing contraceptive care to female AYA with SCD. Methods Guided by the Health Belief Model (HBM), we developed a 25-question, web-based survey to assess providers’ practices. Survey links were distributed nation-wide to pediatric SCD and/or general hematology providers through their publicly available emails and by request to directors of U.S. accredited Pediatric Hematology-Oncology fellowship programs for distribution to their SCD providers. Data analysis included descriptive statistics, chi-square analysis, logistic regression. Results Of 177 respondents, 160 surveys meeting inclusion criteria were analyzed. Most providers reported counseling (77.5%) and referring female AYA patients for contraception (90.8%), but fewer reported prescribing contraception (41.8%). Counseling practices significantly differed in trainees versus established providers (54% vs. 85%, p<0.001) with a similar trend for prescribing (p=0.05). Prescription practices did not differ significantly by provider beliefs regarding potential teratogenicity of hydroxyurea. Key motivators included patient request and disclosure of sexual activity. Key barriers included inadequate provider training, limited visit time, and perceived patient/parent interest. Conclusion Provider contraceptive practices for female AYA with SCD varied, especially by provider status. Health beliefs regarding teratogenic potential of hydroxyurea did not correlate with contraceptive practices. Clinical guidelines, provider training, and patient/parent decision-making tools may be tested to assess whether provider contraceptive practices could be improved.