Nutrition is very important for people with CF as malnourished people do not feel or grow well. Malnutrition damages the immune system, which fights off infections. Studies show a direct link between pulmonary function and nutrition. The better your nutrition and weight, the better your lung function and the longer your life expectancy. It is recommended that people with CF eat a high calorie, high fat, high salt (to replace what is lost through sweat) diet. Cholesterol is not an issue in patients with CF. It is still important to maintain a healthy diet with fruits, vegetables, proteins, and fats to get essential vitamins and minerals.

Pancreatic Enzymes Replacement Therapy

Most people with CF need to take enzymes to replace the pancreatic secretions that help digest food. These enzymes are taken with every meal. Depending on the size of the meal and the dose of the tablets, as many as 20 tablets may be needed a day. Your physician and nutritionist will assist in figuring your dosage of enzyme replacement therapy. If a person forgets to take their enzymes, they can have side effects from malabsorption. These include gas, bloating, stomach cramps, and frequent, greasy, large, yellow or brown stools. If you notice these symptoms or an increase in your appetite and your are taking enzymes, a dose adjustment may be needed. There can still be incomplete absorption of food even when taking pancreatic enzymes. A person with CF needs to take in about 30% more food than a person without CF as they have higher caloric demands in their bodies.

In young infants, predigested milk formulas such as Progestemil, are usually well tolerated. These are sometimes used in young infants when they fail to gain sufficient weight on other formulas. Soybean formulas are not recommended for CF patients and should be avoided. Solid foods are lower in calories than formula and are often added later in infants with CF.

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Calories and ProteinCalories and protein must be increased by 2-3 times the amount for good growth in patients with CF. This goal can only be met with good planning and consistent meal and snacks. The following amounts of nutrients are recommends for children with CF:

  1. Calories:
    Infants (0-1 year) – 70-100 calories per pound per day
    Children (1-9) – 60-85 calories per pound per day
    Males (10-18) – 50-60 calories per pound per day
    Females (10-18) – 40-60 calories per pound per day
  2. Protein:
    Infants – 2 grams per pound per day
    Older children – 1 ½ grams per pound per day
    Young Adults – 1 ½ grams per pound per day

Animal foods such as milk, meats, cheese and eggs provide high quality protein and calories. Vegetables that are high in protein and calories are peanut butter, dry beans and peas. Starches such as potatoes, corn, pasta, rice, and breads are higher in calories. Offer a good variety of foods emphasizing high calorie and protein daily. Desserts and sweets should be offered at the end of a meal and only after child has eaten other foods well. Excessive sweets will interfere with eating the foods a CF patients needs for good health and growth.

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Fats

Fat intake will vary with age and total caloric intake. Fats are high in calories and a certain amount of fat is essential for growth. Additional enzymes will often be recommended when foods high in fat are eaten.

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Salt

The CF patient loses large amounts of salt in his sweat which should be replaced to avoid possible dehydration especially during excessive exercise, fever, or hot weather. Extra amounts of salt, in addition to the amount usually cooked in food, should be given during these times.

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Vitamin A, D, E, and K

People with CF also have difficulty absorbing some vitamins, especially those that are fat-soluble, and often need to take vitamin A, E ,D , and K supplements. Vitamin A helps fight infections, prevents night blindness, and general growth and development of the body. Vitamin A is found in animal products such as liver, egg yolks, and fortified milk. Carotene is found in dark green, yellow, and orange fruits and vegetables and is converted to active Vitamin A. Vitamin E plays an important role in several parts of the body, especially the nerves. Vegetable oil, wheat germ, and dried peas and beans are good sources for Vitamin E. A deficiency in Vitamin E may result in unsteadiness while walking or abnormal knee-jerk reflex. Vitamin D is required for strong teeth and bones. It helps with the absorption of calcium and phosphorous. A deficiency in Vitamin D can result in abnormally soft bones. Vitamin D can be found in fortified milk, dairy products, and activated through the skin by sunlight. Vitamin K is used by the liver to make clotting factors to help stop bleeding. Good food sources for Vitamin K include green leafy vegetables, cauliflower, and some dairy products.

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Nutrition Supplements

High caloric nutritional supplements in a variety of flavors and forms such as milkshakes, snack bars, cookies, and even sprinkle powder can be prescribed if needed to assist weight gain or weight maintenance. Sometimes people with CF who can’t eat enough calories can add these supplements which fit in nicely with a busy lifestyle for a snack or if traveling away from home. Talk with your nutritionist and physician about the various products and programs offered.

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Tube Feeding

Some people with CF can not absorb enough nutrients from food they eat by mouth to maintain or gain weight. For these situations, calories in a liquid form can be given through a tube while they sleep. The tube reaches directly into the stomach or intestine. There are several different forms of tube feeding to choose from. Some patients use a nasogastric tube (NG tube) that is passed through the nose, down the throat, and into the stomach. Some patients find this idea rather unpleasant at first, but have learned to insert this themselves each night before bed. Another option is a gastrostomy tube (G-Tube) which requires a minor surgical procedure for placement of the tube through skin of the upper abdomen and into the stomach. For a short time, the patient’s oral intake is reduced to small amounts of food while the small surgical wound heals. Then they can eat normally by mouth during the day and additional calories are added through the G-tube at night. It doesn’t involve the discomfort of the nightly insertion of the NG tube, nor does it interfere with coughing or breathing. It is invisible under clothes during the day, can be hooked up at night, and can be removed when if no longer needed without an operation. The last option is the jejunostomy (J tube). Feeding is similar to G-tube except tube goes further down into the intestine instead of the stomach. It is less common, but has the advantages of protecting from reflux of the tube feeding. No one likes the idea of tube feeding when they first hear about it, but the results in growth and weight gain are very dramatic and convert the skeptics. Many patients or families feel a huge relief as the burden of force-feedings to consume calories is taken away. It is common for patients who receive tube feedings overnight to feel full in the mornings and not want breakfast. Talk with your physician about the pro’s and con’s of each system to decide what is best for you.

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Ways to Increase Calories

Add margarine to bread of grilled sandwiches, vegetables, waffles, and potatoes. Add grated Parmesan cheese to spaghetti, casseroles, popcorn, and salads. Melt cheese on scrambled eggs, casseroles, and sandwiches. Order extra cheese on pizza. Use cheese sauce on vegetables, potatoes, pretzels, nachos, and french fries. Chopped nuts add lots of calories to cookie dough, bread, and pancakes. Buy breakfast cereals with nuts and dried fruit for higher calories. Drink whole milk and use whole milk cheeses. Add powdered nonfat dry milk to whole milk to increase calories (1/4 cup powder milk to 8 ounces whole milk). Add gravy to meat, potatoes, rice, noodles, and fries. Use extra eggs in waffles, pancakes, or french toast. For infants, talk with the nutritionist about things you can add to formula to make higher in calories.

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Behavioral Tips to Improve Nutrition

Feeding problems are common in all children and mealtime can often be a battle. Try to avoid letting toddlers and children “graze” all day as it makes it harder to time enzymes. Try to have meals at the same time each day. Plan an eating schedule of 3 meals and 2 snacks daily. Offer a wide variety of food from the 4 basic food groups when planning meals. Try new foods, but don’t fuss over a rejection. Offer milk or other high calorie drinks with meals and snacks. Make mealtime enjoyable, maybe have a picnic lunch outside, eat with a friend, or in another room of the house for a change. Meal should last around 20-30 minutes. Avoid force-feeding children. If a child refuses to eat for more than 10 minutes, remove from table and do not offer anything else until next schedule meal or snack time. Avoid distractions such as the TV. Be sure the child doesn’t fill up on liquids. Water, juice, cola, tea, or kool-aide will reduce total intake. Do not give drinks 30 minutes prior to a meal. Reward good behavior with praise. Set up a sticker chart system with reward for good eating habits. Most importantly, set an example by eating nutritious meals with your children.