Executive Abilities and Academic Achievement in Children with Sickle Cell Disease

Academic achievement is crucial to a child’s psychosocial and occupational success (Davaoudzadeh et al., 2015; Margari et al., 2013). In children with sickle cell disease (SCD), a genetic disorder resulting in abnormal hemoglobin and significant neurologic sequelae, poor academic achievement is common (e.g. Wang et al., 2001). Studies of typically-developing children have revealed links between academic achievement and neuropsychological abilities, particularly higher-order executive abilities that are mediated primarily by frontal brain regions (Altemeier et al., 2006; Bull & Scerri, 2001). In children with SCD, there is a wealth of evidence that executive abilities are impaired (Berkelhammer et al., 2007), but very little research has been conducted in which academic achievement has been examined within the context of specific cognitive domains, including executive abilities. The present study was designed to investigate relationships among executive performance (assessed using a composite of performance-based measures), executive behavior in daily life (assessed using parent ratings), and academic achievement (assessed using performance-based measures) in children with SCD. Existing data collected during a longitudinal study (baseline and 2-year follow-up) of 38 children with SCD were examined. Results indicate a significant, positive relationship between baseline executive performance and executive behavior, and these two measures were simultaneously associated with concurrent academic achievement. Of note, executive performance did not explain variance in academic achievement above and beyond executive behavior, but executive behavior did predict unique variance. However, neither baseline executive performance nor baseline executive behavior predicted follow-up academic achievement. Our findings suggest that measures of executive behavior should not be overlooked in the context of pediatric SCD, and that additional investigation of potential screening tools for academic outcomes in this population is warranted