Gastrointestinal pathophysiology and nutrition in cystic fibrosis

ABSTRACT

Introduction: Cystic fibrosis (CF) is a severe, progressive, multisystemic disease that is caused by mutations in the cystic fibrosis transmembrane conductance regulator gene. Optimizing nutrition is critical, as higher growth parameters are associated with better pulmonary function and outcomes, but unfortunately patients with this disease are prone to malnutrition, growth failure, and vitamin deficiencies. The purpose of this review is to provide a timely highlight of the physiologic processes and outcome data to support today’s management strategies, as well as review these principles themselves.

Areas covered: This review covers the background of the importance of vigilant attention to nutrition and growth in these patients, the underlying physiology leading to an abnormal gastrointestinal tract and its role in CF malnutrition, and current evaluation and management strategies to address nutrition in CF. Analysis of up-to-date relevant literature was performed using PubMed.

Expert commentary: Advances in research and clinical developments over the years have improved knowledge of this disease as well as patient outcomes. Of particular importance is optimizing nutrition especially in the early stages of life, as well as accounting for the markedly abnormal CF intestinal milieu when addressing the gastrointestinal and nutritional needs of these patients.

KEYWORDS:

• Cystic fibrosis transmembrane conductance regulator (CFTR)
• cystic fibrosis
• enteral nutrition
• failure to thrive
• growth failure
• malabsorption
• nutrition
• Pancreatic enzyme replacement therapy (PERT)
• pancreatic insufficiency
• vitamin deficiency

Declaration on interest

J.H. Teckman is a consultant in the ‘Alpha-1’ patient community, grant reviewer and advocate for the non-profit Alpha-1 Foundation and the Alpha-1 Project. J.H. Teckman is a consultant, site investigator, and/or grant recipient involved with the following companies; Alnylam Inc, Arrowhead Pharmaceuticals Inc, Dicerna Inc, Editas Pharmaceuticals, Gilead Pharmaceuticals, Intellia Corp, Proteostasis Inc, RestorBio, Third Rock Consulting, Triangle Insights, GLG consulting, and Snyder Legal.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.