Nutritional considerations for a new era: A CF foundation position paper

Area of Science:

• Nutrition Science
• Pulmonology
• Metabolic Health

Background:

• The landscape of Cystic Fibrosis (CF) care is rapidly evolving, particularly with the advent of cystic fibrosis transmembrane regulator highly effective modulator therapy (HEMT).
• pwCF are living longer, necessitating a re-evaluation of traditional high-fat, high-calorie dietary recommendations.
• Emerging concerns include potential negative nutritional and cardiovascular consequences of aging with CF, alongside issues like poor diet quality, food insecurity, and eating disorders.

Approach:

• A multidisciplinary committee convened by the CF Foundation conducted a focused literature review.
• Four specialized workgroups were established: Weight Management, Eating Behavior/Food Insecurity, Salt Homeostasis, and Pancreatic Enzyme use.
• The committee synthesized current knowledge to inform updated nutrition strategies.

Key Points:

• The traditional CF diet may require modification due to potential long-term health impacts.
• Increased prevalence of overweight and obesity in pwCF requires new nutritional management approaches.
• Addressing diet quality, food insecurity, and eating disorders is crucial for comprehensive CF care.

Conclusions:

• pwCF are experiencing improved longevity, shifting nutritional needs and considerations.
• Overnutrition presents new challenges, potentially impacting pulmonary and cardiometabolic health.
• Updated nutrition guidelines are essential to support the evolving health profile of individuals with CF.