Cross-sectional survey reveals varied dietary adjustments regarding weight in pediatric cystic fibrosis on CFTR modulators, highlighting the need for individualized nutrition.
Key Points
To describe parents' and caregivers' perceptions and approaches to weight management in children with cystic fibrosis following the introduction of CFTR modulator therapies.
Cross-sectional survey with content validity established through four expert reviews and two cognitive interviews.
Distributed via social media groups connected to a western US clinic and directly to families at a southeastern US cystic fibrosis clinic.
Evaluated descriptive survey responses from 49 caregivers representing 60 children with cystic fibrosis.
All 60 children were prescribed CFTR modulators, with 46 (76.6%) receiving therapy for over one year.
Caregivers agreed with clinicians on their child's weight classification in 86% of cases (n=49) and reported high comfort discussing weight with clinic teams (mean score 8.31 ± 2.47 out of 10).
Dietary modifications occurred in 52% of children treated for over a year, with strategies spanning calorie increases, calorie reductions, and cessation of supplemental nutrition.