CF Care Guidelines - Nutrition/GI

Because nutrition is so important to a long and high-quality life for people with cystic fibrosis, the CF Foundation has created several care guidelines related to nutritional and GI (gastrointestinal) issues.

On this page:

Nutrition in Children and Adults

Good nutrition is very important for people with cystic fibrosis. To help ensure proper nutrition in people with CF, the CF Foundation Subcommittee on Growth and Nutrition combined recent nutritional studies with an analysis of results from the Patient Registry Report to create guidelines on the management of CF nutrition. Recommendations include:

  • High-calorie diet, including supplements when needed
  • Behavioral intervention to encourage good eating habits in children
  • Keeping track of nutritional indicators, such as body mass index
  • Appropriate doses of pancreatic enzymes

Care Guidelines

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Pancreatic Enzyme Replacement

More than 90 percent of people with CF take pancreatic enzymes to improve digestion and growth. Care guidelines on pancreatic enzymes cover:

  • How to find the right dose (by weight or fat in the diet)
  • What to do if pancreatic enzymes do not help digestion and growth
  • How to avoid fibrosing colonopathy from too many pancreatic enzymes

Care Guidelines

The CF Foundation is currently supporting the development of new pancreatic enzyme formulations that may improve and simplify pancreatic enzyme dosing in the near future.

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Cystic Fibrosis-Related Diabetes

More than 35 percent of adults with CF have cystic fibrosis-related diabetes (CFRD). Because proper treatment of diabetes improves nutrition and health, the CF Foundation has care guidelines on diagnosis, screening and managing CFRD. The guidelines cover:

  • The definition of CFRD
  • Screening for CFRD at clinic
  • Managing CFRD
    • Monitoring blood sugars
    • Insulin use
    • Meal planning
    • Nutritional supplements
    • Exercise
  • Maintaining good nutrition and growth
  • Adapting to living with diabetes
  • Screening for complications

Care Guidelines

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Vitamin D 

Vitamin D is a vitamin that helps people with CF have stronger bones and better nutrition. However, getting the right level of Vitamin D in the body of people with CF is hard to do. The care guidelines cover:

  • When vitamin D levels should be measured 
  • How much and what kind of vitamin D should be taken 
  • How to increase vitamin D levels

Care Guidelines

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Bone Health

Bone disease is increasingly common in people with CF. The CF Foundation gathered a group of experts to examine the causes of bone disease in CF and create recommendations to help maintain healthy bones. The practice guidelines describe the science behind the recommendations, which include:

Guidelines

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Liver Disease

More than 10 percent of people with CF have liver disease, a number that may increase as people with CF live longer lives. Care guidelines for CF liver disease covers:

  • Screening
  • Diagnosis
  • Treatment – depending on type of liver disease
  • Nutritional care
  • Managing advancing liver disease

Guidelines

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Pediatric Nutrition

Normal growth is associated with good lung function in children with cystic CF. The Foundation gathered a team of experts to determine the best ways to ensure good nutrition for growth. Guidelines on pediatric nutrition include:

  • Keeping track of growth by measuring:
    • height or length, weight, head circumference (in infants)
    • bone health, stage of puberty
    • pancreatic insufficiency
  • Getting the right amount of:
    • pancreatic enzymes
    • formula or breastfeeding (for infants)
    • necessary calories
    • vitamins, minerals, fatty acids
    • salt
  • Treating nutritional failure through:
    • improving eating habits
    • supplements
    • tube feedings

Guidelines

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Updated 7/19/12