A Comprehensive Review of Gastrointestinal Manifestations in Cystic Fibrosis in the Era of Highly Effective Modulator Therapy.

Abstract

Cystic fibrosis (CF) is a multifaceted genetic disorder impacting the respiratory, gastrointestinal, and hepatobiliary systems, necessitating a multidisciplinary approach for management. Its effects are observed throughout all stages of life, from infancy to adulthood, with gastrointestinal manifestations varying at each stage, including conditions such as meconium ileus and gastroparesis. Given its wide range of differential diagnoses and implications, a thorough understanding of its associated conditions is crucial for healthcare professionals. Monitoring CF involves tracking growth and development, as nutritional decline can impede patient progress. Quality of life can differ significantly based on treatment approaches, underscoring the importance of effective therapeutic strategies. The introduction of highly effective modulators has notably improved the clinical course of CF. However, CF providers have begun to identify previously unrecognized nutritional issues such as obesity and eating disorders with the advent of highly effective modulators. With variations in gastrointestinal clinical manifestations and treatment offerings between pediatric and adult providers, it is important to review CF gastrointestinal (GI) diseases in depth encompassing the entire spectrum of the gut health in CF for the larger benefit of pediatric and adult GI providers who may not be primarily focused on CF care. With this article, we aim to empower both pediatric and adult GI providers in dealing with CF GI symptoms effectively in their clinical practice as we see more patients with CF living longer and hope to contribute to their betterment and achieving fulfilling lives.