Abstract

The nutritional landscape in cystic fibrosis has shifted dramatically in the era of CFTR modulator therapy. In this review, we will critically examine the literature on overweight and obesity in CF, current nutritional care unknowns and opportunities for further investigation or adaptation in clinical care. Results of clinical trial and real-world data reflect marked improvement in nutritional status and quality of life. Clinical outcomes including CF related diabetes and CF related liver disease appear positively impacted. Secondary impacts on cardiometabolic disease have been noted, especially in association with excessive weight gain. The prior approaches to optimizing nutrition in cystic fibrosis with caloric excess can likely be safely retired for many. As modulator access expands across the lifespan, a longitudinal focus on health maintenance should be considered.

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