Nutrition is integral to the care of individuals with cystic fibrosis (CF). Better nutritional status is associated with improved pulmonary function. In some individuals with CF, enteral tube feeding can be useful in achieving optimal nutritional status. Current nutrition guidelines do not include detailed recommendations for enteral tube feeding. The Cystic Fibrosis Foundation convened an expert panel to develop enteral tube feeding recommendations based on a systematic review of the evidence and expert opinion. These guidelines address when to consider enteral tube feeding, assessment of confounding causes of poor nutrition in CF, preparation of the patient for placement of the enteral feeding tube, management of the tube after placement and education about enteral feeding. These recommendations are intended to guide the CF care team, individuals with CF, and their families through the enteral tube feeding process.

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Nutritional management is an integral part of multidisciplinary care for persons with cystic fibrosis. This review will look at how nutrition care has evolved over time. In addition, we will look at how some newer therapies impact nutrition care.

This tool for screening nutrition risk for CF is reliable and valid, with consistent, reproducible results, free from subject or observer bias.

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HGS z scores and FEV significantly increased at follow-up. HGS z scores were lower than the standard even though mean BMI z scores classified participants as normal nutrition status.

Optimal growth and nutrition status predict better lung function and longevity for children with cystic fibrosis (CF). Daily nutrition therapy for children with CF requires adequate food resources, parental knowledge of nutrition and behavior management, and confidence in one’s ability to apply the skills. The Mountain West Cystic Fibrosis Consortium Questionnaire (MWCFC-Q) was designed to identify educational intervention targets to improve the growth and nutrition of children with CF. Parents of children with CF returned 305 anonymous MWCFC-Qs. Data analyzed included household food security, knowledge of nutrition and general CF therapies, and self-confidence in one’s ability to manage components of CF care. Factors associated with food insecurity were reported by 26.3% of respondents. The median accuracy for questions regarding nutrient content of commonly used foods was 71.4% and 57.9% for CF nutrition therapy. Parents’ self-confidence in overall CF management was relatively high at a mean value 8.28 ± 1.22 of 10 possible. However, mean self-confidence in the CF nutrition domain was significantly less than mean self-confidence for the CF-related tasks domain, which included chest physiotherapy and medication administration (7.75 ± 1.56, 8.62 ± 1.24, P < .001, respectively). Parental knowledge of nutrition for CF and confidence in the application of this knowledge can improve the growth and nutrition status of children with CF. Identification of food security issues may enable health care professionals to adjust nutrition interventions and direct families to appropriate food resources. The MWCFC-Q could be useful for designing and testing educational interventions for nutrition management of CF.