Cystic Fibrosis and Nutrition

Cystic fibrosis (CF) is a chronic and inherited disease caused by a defect in the genes and that affects the normal function of organs like the lungs, pancreas, liver, intestines, and sinuses, as well as the reproductive system. Patients experience an abnormal production of thick and sticky mucus, which accumulates in the lungs and airways, making it difficult for patients to breathe and more predisposed to the growth of bacteria.

According to the Cystic Fibrosis Foundation (CFF), “staying healthy with cystic fibrosis means maintaining a healthy lifestyle, which includes taking care of your digestive (gastrointestinal, or GI) tract. Good nutrition is key, so in addition to eating a balanced, high-calorie diet, there are many things you can do — taking medications, managing your time and working with your CF care team — to help keep your body healthy and help your lungs fight off infection.”

Importance of Nutrition for Cystic Fibrosis Patients

Patients who suffer from cystic fibrosis have a deficiency in their pancreas, an organ located in the abdomen behind the stomach, that is responsible for the production of enzymes. Due to the disease and the accumulation of sticky mucus in the pancreas, the production of enzymes — which help in the digestion and absorption of proteins and fats — is compromised. As a result, patients may experience a series of symptoms, including stools filled with mucus that float or are foul smelling; gas; bloating or a distended belly; and lack of enough protein, fat, and calories.

Due to these limitations in CF patients, maintaining a healthy weight may be a difficult task, and even in the case of a normal weight, patients may not be getting the right nutrition. In the case of children, it is particularly problematic since the lack of proper nutrition may compromise normal growth and development. This is why it is important for patients to discuss with their medical team the recommendations for a healthy diet and the need for supplements of vitamins, minerals, or enzymes.

Nutrition Guidelines for Patients with Cystic Fibrosis

Given the importance of nutrition for CF patients, the National Institutes of Health (NIH) have issued a series of guidelines about the topic. Regarding enzymes, vitamins, and salt, the NIH acknowledges that most patients need to take pancreatic enzymes with all meals and snacks to help the body better absorb fat and protein. Taking vitamins A, D, E, K, and extra calcium is also helpful, and there are special formulas specifically for CF patients. In the case of patients who live in hot climates, a small amount of extra table salt may be needed as well.

As for patients’ eating patterns, the NIH recommends that patients eat whenever they feel hungry, possibly eating several small meals a day and keeping a variety of nutritious snack foods around. In addition, the NIH advises patients to consume more calories and proteins, which could include adding grated cheese to soups, sauces, casseroles, vegetables, mashed potatoes, rice, noodles, or meat loaf, as well as using whole milk, half and half, cream, or enriched milk in cooking or beverages.

Additional tips to increase the amount of protein and calories include spreading peanut butter on bread products; adding skim milk powder to normal milk; adding marshmallows to fruit or hot chocolate; adding raisins, dates, or chopped nuts and brown sugar to hot or cold cereals, or have them for snacks; adding butter or margarine to food; using sour cream or yogurt on vegetables like potatoes, beans, carrots, or squash; adding extra cheese on top of frozen prepared pizza; adding coarsely chopped hard-cooked egg and cheese cubes to a tossed salad; serving cottage cheese with canned or fresh fruit; adding grated cheeses, tuna, shrimp, crab meat, ground beef, diced ham or sliced boiled eggs to sauces, rice, casseroles, and noodles; and increasing the consumption of breaded meat, chicken, and fish instead of broiled or plain roasted.

Note: Cystic Fibrosis News Today is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.


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