Executive functioning in pediatric cystic fibrosis: A preliminary study and conceptual model

Research has shown that broad cognitive functioning in individuals with CF is intact. Specific executive functioning (EF) deficits have been identified, however, and adults with CF report more symptoms of ADHD than the general population. EF skills are critical to the management of a complex disease like CF although studies have not adequately examined EF mechanisms in CF. This manuscript (a) described EF in a small sample of children with CF, (b) summarized relations found between EF and psychosocial variables, and (c) presented a conceptual model by which to understand EF's impact on adherence in CF.

Data for this preliminary study were collected from 19 children with CF and their caregivers (ages, 6-18). Caregivers completed questionnaires assessing their child's physical and mental health, their own functioning, and overall family functioning. EF was measured using a parent-report rating scale. Patient health data were collected from the electronic medical record.

This sample did not demonstrate elevated levels of EF impairment. Worse EF was related to poor family communication/cohesion, as well as higher treatment burden, worse lung function, poorer adherence, and older age. From these findings, a preliminary model was developed describing EF in the context of CF and adherence.

Findings from this preliminary study suggest that the CF regimen and associated symptoms may overload otherwise adequate EF skills. Reducing disease burden and preventing burnout should be a focus of treatment. A better understanding of EF in CF and the impact on adherence would allow for better clinical management and effective design of interventions.