Lifespan's A - Z Health Information Library

Cystic fibrosis (CF) is a serious, life-threatening disease in children. It affects many body systems by producing abnormally thick secretions that can obstruct the pancreas, intestines, bile ducts, and air passages in the lungs.

With pancreas blockage, the enzymes (molecules that speed up chemical reactions) needed for absorption in the small intestine are not available, leading to malabsorption. Steatorrhea (fatty diarrhea) is frequent as are foul smelling stools, due to undigested fat, protein, and starch.

The major treatment breakthrough for malabsorption caused by CF was the development of enteric coated pancreatic enzymes. The enzymes are taken with meals and snacks. They improve the digestion and absorption of fat, starch, and protein.

The dietary needs are based on the severity of the symptoms. While many children with CF have delayed growth, the goal is to provide adequate nutrients for normal growth and development. From infancy through adulthood nutritious, high-calorie, high-protein foods are essential. Energy needs can be 50 - 100% greater and protein needs 50% greater than the Recommended Daily Allowance.

Historically, low-fat diets were discouraged to lessen fat malabsorption. With the advent of pancreatic enzymes, fat can be 30 - 50% of calories, depending on the person's ability to tolerate it. The body absorbs fat-soluble vitamins (vitamins A, D, E, K) poorly, so these are commonly prescribed. A multivitamin and liberal salt use is also recommended.

A multi-disciplinary team of a doctor, nurse, respiratory therapist, dietitian, and social worker, work with the patient and family to provide for an improved quality of life. Life expectancy continues to improve as new treatments are developed.

The most serious complication of CF is infection in the lungs. Also, the inability of the lungs to clear out infection and mucus may lead to the inability to breathe. Nutrition plays an important role in strengthening the patient's immune system to fight disease.

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:

• 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.
• 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.
• 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 two 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 while it's hot -- hot breads, pancakes or waffles absorb more butter than cool ones.
• Sour cream or yogurt can be used on vegetables such as potatoes, beans, carrots, or squash. Try them in gravies or as a salad dressing for fruit.
• Breaded meat, chicken, and fish have more calories than broiled or plain roasted.
• Add extra mozzarella or jack cheese on top of frozen prepared pizza.
• Coarsely chopped hard cooked egg and cheese cubes are tasty in 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, noodles, butter toast or hot biscuits.