Neurocognitive impairment in Ugandan children with sickle cell anemia compared to non-sickle siblings

Paul Bangirana, Makerere University, Uganda
Richard Idro, Makerere University College of Health Sciences, Uganda
Robert Opoka, Aga Khan University
Deogratias Munube, Makerere University, Uganda
Ezekiel Mupere, Makerere University, Uganda
Philip Kasirye, Makerere University, Uganda
Nancy Green, Columbia U. Medical Center,USA
Amelia Boehme, Columbia U. Medical Center,USA

This work was published before the author joined Aga Khan University

Abstract

Purpose: Sickle cell anemia (SCA) increases childhood risk of neurocognitive impairment from severe anemia and vascular brain injury. We hypothesized that impaired neurocognitive function was more prevalent in Ugandan children with SCA than in their non-SCA siblings. Materials and methods: In the BRAIN SAFE 1 cross-sectional study (Green NS, 2019), a sample of children ages 1-12 years were randomly selected from Mulago Hospital SCA clinic in Kampala. Their neurocognitive function was compared to that of unaffected siblings using age-appropriate tests: Mullen Scales of Early Learning (Mullen) for children under age five and Kaufman Assessment Battery for Children, second edition (KABC-II) for ages 5-12. Raw scores were converted into age-adjusted Z-scores compared to established community controls; Z-scores-2 or lower were categorized as impaired neurocognitive function.