Consensus-based guidance for the nutritional management of children with cystic fibrosis on ELEXACAFTOR/TEZACAFTOR/IVACAFTOR.

INTRODUCTION

The introduction of CFTR modulators such as elexacaftor/tezacaftor/ivacaftor (ETI) has significantly altered the nutritional trajectory of children with cystic fibrosis (cwCF). While most eligible children in high-resource countries have commenced ETI, the rollout has outpaced the development of nutritional guidance. This study aimed to: (1) systematically review the literature on ETI and nutrition (2) develop expert consensus-based statements and (3) highlight existing research gaps and priorities.

METHODS

A systematic review and guideline development process was registered on PROSPERO (ID: CRD42024587618). An expert panel of 22 professionals, including dietitians, gastroenterologists, respirologists, endocrinologists, and a parent representative was convened with attention to global representation. Research questions were developed using the Population, Intervention, Comparison, Outcome (PICO) framework. A comprehensive literature search was conducted across Medline, Embase, CINAHL, and Cochrane (January 2010 to April 2025). Studies were independently screened, assessed for quality using Scottish Intercollegiate Guidelines Network (SIGN) criteria, and extracted into evidence tables. Draft statements were developed and refined through iterative feedback and online discussion. Final consensus was achieved using real-time Delphi, with ≥80% agreement required for endorsement. Reporting followed Accurate Consensus Reporting Document (ACCORD) guidelines.

RESULTS

67 consensus-based statements were developed. The top research priorities were identified based on the greatest proportion of "strongly agree" responses.

CONCLUSION

ETI has altered nutritional requirements in cwCF, yet evidence remains limited. This study presents the first consensus-based guidance for the nutritional care of cwCF receiving ETI, providing interim, expert informed recommendations and highlighting research priorities, particularly the need to re-evaluate traditional BMI targets.