Abstract
OBJECTIVE: Sickle cell disease (SCD) is a lifelong condition characterized by pain, which is associated with reduced health-related quality of life (HRQL). Data suggest that patients with SCD vary in how they cope and their neurocognitive abilities. This study aimed to characterize executive functioning and pain coping styles in children with SCD experiencing a range of pain frequency (i.e., chronic, episodic, and asymptomatic) and to examine whether executive functioning mediates the relationship between pain coping and HRQL. METHOD: Participants included 100 children and adolescents with SCD between the ages of 8 and 18 years (M = 13.53, SD = 2.8) and their parents who were recruited during outpatient SCD clinic visits in a children's hospital. Children completed questionnaires related to pain experience and pain coping. Parents completed questionnaires about demographic information, their child's executive functioning, and HRQL. RESULTS: Pain intensity, executive dysfunction, and engagement in emotion-focused coping (i.e., internalizing/catastrophizing and externalizing) predicted poor HRQL. In addition, engagement in emotion-focused coping predicted executive dysfunction. Multivariate analysis of covariance revealed executive functioning did not differ based on pain frequency; however, executive functioning was a significant mediator that helped explain the relationships between distraction and emotion-focused coping techniques on HRQL. CONCLUSION: Findings support that executive functioning is an important factor in understanding the relationship between pain coping and HRQL in youth with SCD. Future research is warranted to examine the potential impact of executive functioning on the utility of interventions targeting adaptive pain coping in youth with SCD.