ABSTRACT
Cystic fibrosis (CF) is an inherited disease affecting the respiratory, gastrointestinal, hepatobiliary, and reproductive systems. Nutrition status in persons with CF is often compromised due to increased energy needs, frequent infections, pancreatic insufficiency, lung disease, or CF-related diabetes. Maintaining good nutrition status has been associated with better pulmonary function, reduced hospitalizations, and increased longevity. Nutrition support as oral supplementation (used in >37% of the CF population) or tube feeding (used in >13% of the CF population) is often required for children and adults with CF. The purpose of this update is to describe current consensus and evidence for enteral nutrition support guidelines, reported complications of enteral feeding in the CF population, evidence of expected outcomes, and to discuss related areas requiring further research. A case report is provided to illustrate potential outcomes of aggressive enteral support.