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Cystic fibrosis (CF) is a potentially life-threatening disease that causes thick, sticky mucus to build up in the lungs and digestive tract. Persons with cystic fibrosis need to eat high calorie and high protein foods throughout the day.

This article discusses the nutritional needs for persons with CF. For specific information about the disease itself, see the article on cystic fibrosis.

Your pancreas is an organ that sits in your belly behind your stomach. An important job of your pancreas is to make enzymes. These enzymes help your body digest and absorb protein and fats. However, the build-up of sticky mucus in the pancreas from cystic fibrosis can lead to serious problems, including:

• Stools that contain mucus, are foul smelling, or float
• Gas, bloating, or distended belly
• Problems getting enough protein, fat, and calories, in your diet

Because of these problems, people with cystic fibrosis may have a hard time maintaining a normal weight. Even if your weight is normal, you may not be getting the correct nutrition. Children with cystic fibrosis may not grow or develop correctly.

The following are methods for adding protein and calories to the diet. In addition to these tips, make sure that you are taking a multivitamin containing vitamins A, D, E and K.

Enzymes, vitamins, and salt:

• Everyone with cystic fibrosis must take pancreatic enzymes. These enzymes help your body absorb fat and protein. Taking them all the time will decrease or get rid of foul-smelling stools, gas, and bloating.
• Take enzymes with all meals and snacks.
• Talk to your doctor about increasing or decreasing your enzymes, depending on your symptoms.
• Ask your doctor about taking vitamins A, D, E, K, and extra calcium.
• People who live in hot climates may need a small amount of extra table salt.

Eating patterns:

• Eat whenever you are hungry. This may mean eating several small meals throughout the day.
• Keep a variety of nutritious snack foods around. Try to snack on something every hour. Try cheese and crackers, muffins, or trail mix.
• Make an effort to eat regularly, even if it's only a few bites; or include a nutritional supplement or milkshake.
• Be flexible. If you aren't hungry at dinner time, make breakfast, mid-morning snacks, and lunch your main meals.

Getting more calories and protein:

• Add grated cheese to soups, sauces, casseroles, vegetables, mashed potatoes, rice, noodles or meat loaf.
• Use whole milk, half and half, cream, or enriched milk in cooking or beverages. Enriched milk has non-fat dry milk powder added to it.
• Spread peanut butter on bread products or use it as a dip for raw vegetables and fruit. Add peanut butter to sauces or use on waffles.
• Skim milk powder adds protein -- try adding 2 tablespoons of dry skim milk powder in addition to the amount of regular milk in recipes.
• Add marshmallows to fruit or hot chocolate. Add raisins, dates, or chopped nuts and brown sugar to hot or cold cereals or for snacks.
• A teaspoon of butter or margarine adds 45 calories to foods. Mix it into hot foods such as soups, vegetables, mashed potatoes, cooked cereal, and rice. Serve it on hot foods -- hot breads, pancakes, or waffles absorb more butter than cool ones.
• Use sour cream or yogurt on vegetables such as potatoes, beans, carrots, or squash. They can also be used as a dressing for fruit.
• Breaded meat, chicken, and fish have more calories than broiled or plain roasted.
• Add extra cheese on top of frozen prepared pizza.
• Add coarsely chopped hard cooked egg and cheese cubes to a tossed salad.
• Serve cottage cheese with canned or fresh fruit.
• Add grated cheeses, tuna, shrimp, crabmeat, ground beef, diced ham or sliced boiled eggs to sauces, rice, casseroles, and noodles.

Stallings VA, Stark LF, Robinson KA, Feranchak AP, Quinton H. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: Results of a systematic review. Journal of the American Dietetic Association. 2008;108:832-839.

Boat TF, Acton JD. Cystic fibrosis. In: Kliegman RM, Behrman RE, Jenson HB, Stanton BF, eds. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa: Saunders Elsevier;2007:chap 400.

Updated by: Jeffrey Heit, MD, Internist with special emphasis on preventive health, fitness and nutrition, Philadelphia VA Medical Center, Philadelphia, PA. Review provided by VeriMed Healthcare Network. Also reviewed by David Zieve, MD, MHA, Medical Director, A.D.A.M., Inc.