Real-time prediction of rapid weight change in children with cystic fibrosis who have initiated modulator therapy.

Authors

Ashlyn Garter, Emrah Gecili, Md Monir Hossain, Aisaku Nakamura, Matthew Wackler, Thomas Boat, Rhonda D Szczesniak

Published in

Clinical nutrition ESPEN. Pages 103448. Jul 02, 2026. Epub Jul 02, 2026.

Abstract

Broad uptake of elexacaftor/tezacaftor/ivacaftor (ETI) modulator therapy has substantially improved nutritional status among children with cystic fibrosis (CF), but post-ETI weight change may be suboptimal without personalized monitoring of body composition and physical function markers. We developed a prediction model including these markers and evaluated clinical thresholds assessing individualized risk of rapid weight change over time.
A retrospective longitudinal cohort study was performed (n=27 children with CF aged 6-11 years starting ETI) to predict age-related body mass index (BMI) percentile over time via random forest and Bayesian elastic net. Model-based clinical thresholds were identified for rapid weight change over time.
Median (Q1, Q3) rate of change in BMI-for-age percentile was 2.06 (-2.42, 5.34). Most (92.6%) met clinical thresholds for rapid weight change; 48.2% experienced weight cycling. Body composition and physical function predictors improved model deviance (-2 log-likelihood: 569.05 vs. 820.42) and forecasting performance (mean absolute percentage error [SD]: 0.12 [0.22] vs. 0.85 [1.81]). Greater hand grip strength corresponded to increased BMI-for-age percentile over time and was a criterion for clinical thresholds. Younger age and higher fat mass index (FMI) and fat-free mass index (FFMI) z-scores at enrollment were associated with lower BMI-for-age percentile improvement during treatment. Baseline body composition and physical function markers yielded similar predictive accuracy to inclusion as time-varying predictors. Model performance using BMI z-scores yielded minor differences.
Monitoring post-ETI body composition and hand grip strength may sufficiently predict rapid weight change in pediatric CF, thereby improving nutritional health and modulator benefit.

PMID:
42392543
Bibliographic data and abstract were imported from PubMed on 03 Jul 2026.

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