Parent executive functioning, adherence, and health outcomes in infants and toddlers with sickle cell disease

Sickle cell disease (SCD) is an inherited disorder affecting 1 in 365 individuals of African descent in the US. Diagnosis and lifelong management of SCD begin at birth, with behavioral precautions and daily antibiotic prophylaxis required to prevent life-threatening infection. Caregivers’ ability to adhere to the antibiotic regimen is an important protective factor in maintaining health and quality of life. The current study sought to elucidate the self- and pharmacy-reported antibiotic adherence patterns of infants and toddlers with SCD. Primary aims of this work were to determine relations between caregiver executive function (EF), medication adherence, and health outcomes to identify areas for future interventions. Forty-four children aged 0 to 3 years and their caregivers were recruited from a large Mid-Atlantic pediatric hospital. Caregivers completed self-reports of EF (BRIEF), antibiotic adherence (SCAALE), stress (PECI and CHAOS), and health-related quality of life (HRQOL; PedsQL). Caregivers additionally completed performance-based assessment of EF utilizing the NIH Toolbox and signed releases to share pharmacy data used to calculate antibiotic medication possession ratios (MPR). Correlational analyses did not find self-report and performance measures of EF (BRIEF and NIH Toolbox, respectively) to significantly correlate. Similarly, self-report and objective adherence measures (SCAALE and MPR) were not significantly correlated. Importantly, self-reported EF was found to correlate with HRQOL such that lower caregiver-reported EF was associated with poorer HRQOL, highlighting a potential risk factor for negative health outcomes. Both household and illness-related stress (CHAOS and PECI) were positively correlated with self-reported EF (BRIEF) such that more stress was associated with higher executive dysfunction. Contrary to expectations, better caregiver-reported adherence was associated with higher executive dysfunction on the BRIEF self-report. Performance-based EF (NIH Toolbox) was not found to relate to the primary outcome variables (MPR, HRQOL, or household or illness-related stress) in correlations or subsequent regressions. Adherence was investigated as a potential mediator between EF and health outcomes; however, this relation was not observed for either self- or pharmacy-reported adherence. This work permitted an in-depth characterization of an understudied population of infants and toddlers with SCD. The study also elucidated the differing perspectives provided by caregiver-reported and objective measures of adherence and EF. Results indicated that only 51.2% of caregivers had enough medication in their possession to cover their child’s needs during the 3-month study period. The findings about insufficient medication possession notably occur within a sample of caregivers who are invested in the care of their children’s complex health needs, as evidenced by their attendance in this clinic. These results illustrate an opportunity for future evaluation and intervention related to logistical barriers to adherence and management of household and illness-related stressors.