Introduction: There is evidence for increased risk of eating disorders in individuals with diet-treated chronic illnesses, however, data in patients with Cystic Fibrosis (CF) is less clear. No studies have evaluated avoidant/restrictive food intake disorder (ARFID) in the CF population. We investigated the prevalence of disordered eating, including ARFID, in adolescents and young adults with CF. Methods: Patients with CF aged 14-35 years were recruited to complete three validated surveys: (1)Eating Disorder Examination Questionnaire (EDE-Q), (2)Nine-Item Avoidant/Restrictive Food Intake Disorder Scale (NIAS), and (3)Cystic Fibrosis Questionnaire-Revised (CFQ-R). Univariate linear regression analysis identified baseline risk factors associated with these survey scores. Variables with univariate p<0.20 were considered for inclusion in a multivariable linear regression model. Backwards stepwise linear regression was used to identify the final model. Results: A total of 52 patients enrolled. The prevalence of eating disorder measured on the EDE-Q was 9.6%, and on the NIAS was 13.5%. The CFQ-R eating and weight subscales were associated with scores on the EDE-Q, and CFQ-R eating subscale and being dF508 homozygous were correlated with the NIAS total score. Discussion: A clinically significant number of participants screened positive for disordered eating on the EDE-Q and NIAS. Scores on the eating and weight scales of the CFQ-R were associated with the scores on these surveys. Further work is needed to better understand the optimal way to use such tools to screen and treat for eating disorders in individuals with CF.

Background: Medical care has shifted from a paternalistic model towards one centered around patient autonomy and shared decision-making (SDM), yet the role of the pediatric patient in decision-making is unclear. Studies suggest that many children with chronic disease are capable of participating in and even making medical decisions at a young age, and yet we do not standardly involve them. Methods: This is a single center survey study investigating physician attitudes towards involvement of children in decisions regarding lung transplantation, utilizing a hypothetical case scenario with systematic manipulation of age and maturity level. We evaluated physician belief regarding ultimate decision-making authority, attempts at reconciliation of parent-child discordance, and views towards utilizing ethics and psychiatry consultation services. Results: The majority of pediatric pulmonologists believe decision-making authority rests with the parents. The effects of age and maturity are unclear. In instances of parent-child disagreement, physician are more likely to try to convince parents to defer to the child if the child is both older and more mature. Physicians are divided on the utility of ethics and psychiatry consultations. Conclusion: Involvement of children in shared decision-making is broadly supported but poorly implemented. Despite evidence that children with chronic disease may have decisional capacity starting at a young age, the majority of physicians still grant decisional authority to parents. There are numerous barriers to involving children in decisions, including legal considerations. The role of age and maturity level in influencing these decisions appears small and warrants further investigation.