What are the dietary restrictions for patients with cystic fibrosis (CF)?

Updated: Oct 30, 2018
  • Author: Girish D Sharma, MD, FCCP, FAAP; Chief Editor: Kenan Haver, MD  more...
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Answer

In general, a normal diet with additional energy and unrestricted fat intake is recommended. A high-energy and high-fat diet, in addition to supplemental vitamins (especially fat soluble) and minerals, is recommended to compensate for malabsorption and the increased energy demand of chronic inflammation.

In children, because of various physical activities and eating habits, assessment and modification of energy requirements is based on growth and weight gain. Special consideration is given to female patients with a potential for delayed puberty because of malnutrition, patients with diabetes mellitus, and patients with liver disease.

Additional salt intake is recommended for patients living in hot climates, especially during exercise or activities that cause excessive sweating.

Nutritional supplements in the form of either high-energy oral preparations (eg, Scandishake) or enteral feeds (eg, elemental formulas, high-fat mixtures) via nasogastric tube or gastrostomy may be indicated in some patients. In one study, gastrostomy tube placement has been shown to significantly improve percentile body mass index and percent-predicted FEV1 in male patients and female pediatric patients. Lung function changes after placement did not depend on the level of lung function at placement. [66] . The Cystic Fibrosis Foundation published evidence-informed guidelines on enteral tube feeding in cystic fibrosis which include indications, evaluation and investigations before the procedure, as well as timing in relation to pulmonary status. [67]


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