The Impact of Hydroxyurea Use on Cognitive Functioning of Children with Sickle Cell Disease Living in Ghana

Abstract

Background: In sickle cell disease (SCD), cognitive impairment is a manifestation of end organ damage with devastating functional effects. Sub-Saharan Africa accounts for the highest annual SCD burden in the world, yet little is known about cognitive impairment in children from Africa with SCD. Additionally, it is unknown if hydroxyurea, the first FDA-approved drug for SCD, preserves cognitive functioning. The primary objective of this study was to assess the potential cognitive benefits of hydroxyurea administered for ≥ 1 year in children with SCD from Ghana. Methods: We conducted a cross-sectional study at Korle Bu Teaching Hospital in Ghana. The cognitive functioning (psychomotor function, attention, working memory and executive function) of children with SCD (aged 5-13 years) on hydroxyurea (cases) was compared to that of an age-matched group not on hydroxyurea (controls). Results: Children taking hydroxyurea (n=30) had higher hemoglobin (mean 9.34 vs 8.32 g/dL, P=0.012) and red blood cell mean corpuscular volume (mean 94 vs 77 fL, P<0.001) when compared to the non-hydroxyurea group (n=28). Children in the hydroxyurea group performed significantly better in the area of working memory (adjusted difference -0.06, p=0.03), while there was no significant difference in the other domains. Conclusion: To our knowledge, this is the first study conducted in Africa exploring the impact of hydroxyurea on cognitive functioning. While causality cannot be inferred in this observational study, our results suggest that hydroxyurea may improve cognitive functioning. Future longitudinal and interventional studies are needed to further assess potential cognitive benefits of hydroxyurea, particularly in sub-Saharan Africa.