The Relationship Between Executive Functioning and Adaptive Functioning in Pediatric Sickle Cell Disease
Authors:   , Jones Kelly Elizabeth , Daly Brian P. 
Published: July 2021

Sickle cell disease (SCD) is a genetic hematological disorder with complications including pain crises, chronic anemia, infections, organ damage, and stroke. The frontal lobes are highly susceptible to stroke in youth with SCD, and subsequently, executive functioning skills are often compromised. Additionally, children with SCD often have atypical adaptive demands, particularly medical-self care activities. Impairments in executive functioning may help explain difficulties with independent self-management and transition to adult hood. In an interactional model described by Tarazi and colleagues (2007), the role of increased medical self-care demands and executive dysfunction in the development of self-care independence in children with chronic illness is considered. The current study aimed to investigate the relationship between executive functioning and adaptive functioning in youth with SCD by identifying which variables are most important in self-management and independence. It also aimed to validate the KIS-SCD as a measure of independence in medical-self care activities. Twenty-seven participants, ages 12-18 completed neuropsychological testing (general intelligence, attention, processing speed, memory, and executive functioning) and a measure of disease knowledge. Caregivers completed questionnaires assessing adherence, executive functioning, adaptive functioning, and medical self-care independence. Participants displayed borderline executive functioning skills despite parent reports of executive functioning within normal limits. Parent reports of executive functioning (BRIEF) were significantly correlated with adaptive functioning (ABAS-II; r = -.67, p < .01) and medical self-care independence (KIS-SCD; r = -.65, p < .01. Differences were found between those with low and high demands on response monitoring (t(24) = 2.32, p = .029) and inhibition/flexibility (t(24) = 2.07, p = .049). Results from hierarchical linear regression analyses revealed significantly greater predictive value of parent reports of executive functioning than other cognitive and medical variables for general adaptive functioning. Cognitive skills (i.e., sustained attention, memory) held the strongest predictive values for medical self-care independence. No variables were significantly predictive of adherence to SCD clinic appointments. Findings suggest that performance-based measures of executive functioning may be more sensitive that parent report measures in identifying executive functioning impairments in SCD. Executive functioning may explain variance in general adaptive functioning, particularly as it relates to parent reports, suggesting that parent reports and performance-based measures assess separate constructs of executive functioning. Cognitive functioning may be more related to disease-specific demands than global adaptive functioning. Continued research is recommended to further investigate the interaction between executive functioning and medical demand levels. Implications for these findings in the context of disease management and transition planning are discussed.