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Cystic fibrosis - nutrition

Cystic fibrosis (CF) is a life-threatening disease that causes thick, sticky mucus to build up in the lungs and digestive tract. People with CF need to eat foods that are high in calories and protein throughout the day. Function The pancreas is an organ in the

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This guide cannot diagnose a condition or recommend a personal treatment plan. Discuss medical questions with a qualified professional.

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

Function

The pancreas is an organ in the abdomen behind the stomach. An important job of the pancreas is to make enzymes . These enzymes help the body digest and absorb protein and fats. A buildup of sticky mucus in the pancreas from CF can lead to serious problems, including:

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

Because of these problems, people with CF may have a hard time staying at a normal weight. Even when their weight is normal, a person may not be getting the right nutrition. Children with CF may not grow or develop correctly.

Recommendations

The following are ways for adding protein and calories to the diet. Be sure to follow other specific instructions from your health care provider.

Enzymes, vitamins, and salt:

  • Most people with CF 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 provider about increasing or decreasing your enzymes, depending on your symptoms.
  • Ask your provider about taking extra vitamins A , D , E , K , and extra calcium. There are special formulas for people with CF.
  • 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, such as cheese and crackers, muffins, or trail mix.
  • Try to eat regularly, even if it is only a few bites. Or, include a nutrition 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 nonfat 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 it on waffles.
  • Skim milk powder adds protein. Try adding 2 tablespoons (tbsp) or 16.6 grams (g) of 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 have them for snacks.
  • A teaspoon (5 g) 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.
  • Use sour cream or yogurt on vegetables such as potatoes, beans, carrots, or squash. It 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.

References

Egan ME, Schechter MS, Voynow JA. Cystic fibrosis. In: Kliegman RM, St. Geme JW, Blum NJ, et al, eds. Nelson Textbook of Pediatrics . 22nd ed. Philadelphia, PA: Elsevier; 2025:chap 454.

Hollander FM, de Roos NM, Heijerman HGM. The optimal approach to nutrition and cystic fibrosis: latest evidence and recommendations. Curr Opin Pulm Med . 2017;23(6):556-561. PMID: 28991007 pubmed.ncbi.nlm.nih.gov/28991007/ .

Ong T, Ramsey BW. Cystic fibrosis: a review. JAMA . 2023;329(21):1859-1871. PMID: 37278811 pubmed.ncbi.nlm.nih.gov/37278811/ .

Solomon GM, Hoover W, Sorscher EJ, Rowe SM. Cystic fibrosis: diagnosis and management. In: Broaddus VC, Ernst JD, King TE, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine . 7th ed. Philadelphia, PA: Elsevier; 2022:chap 68.

Review Date 4/18/2026

Updated by: Charles I. Schwartz, MD, FAAP, Clinical Assistant Professor of Pediatrics, Regional Medical Director of Penn Medicine Primary and Specialty Care, Perelman School of Medicine at the University of Pennsylvania, General Pediatrician at PennCare for Kids, Phoenixville, PA. Also reviewed by David C. Dugdale, MD, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

Related MedlinePlus Health Topics

  • Cystic Fibrosis

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Related questions

01When to see a doctor

If you or your child has symptoms of cystic fibrosis — or if someone in your family has CF — talk with your healthcare professional about testing for the condition. Make an appointment with a doctor who has skills and experience in treating CF. CF requires regular follow-up with your healthcare professional, at least every three months. Call your healthcare professional if you have new or worsening symptoms, such as more mucus than usual or a change in the mucus color, lack of energy, weight loss, or severe constipation. Get medical care right away if you're coughing up blood, have chest pain or trouble breathing, or have severe stomach pain and bloating. Call 911 or your local emergency number or go to the emergency department at a hospital if: You're having a hard time catching your breath or talking. Your lips or fingernails turn blue or gray. Others notice that you're not mentally alert.

Source: www.mayoclinic.org ↗
02What you can do

You might want to take a friend or family member with you to the appointment to help you remember information. Before your appointment, make a list of: Symptoms and when they started. Include anything that makes symptoms worse or better. All medicines, vitamins, herbs and supplements that you or your child take. Include the doses. Family history, such as whether anyone in your family has cystic fibrosis. Treatment you or your child have had for CF, if any. Include what the treatment was and if it helped. Any other medical conditions and their treatments. Questions to ask your healthcare professional. Questions to ask may include: What is likely causing these symptoms? What kinds of tests are needed? What treatment do you recommend? I or my child have other health conditions. How will cystic fibrosis affect them? Are there any limits needed? Feel free to ask other questions during your appointment.

Source: www.mayoclinic.org ↗
03What Is Cystic Fibrosis?

Cystic fibrosis (CF) is a genetic disorder, which means you get it from your parents at birth. It affects your lungs, pancreas, and other organs. CF changes the way chloride (salt) moves through the cells of your body. This causes the mucus (which should be thin and slippery) in various organs to become thick and sticky. Over time, this thick mucus builds up inside your airways, making it hard to breathe. The mucus traps germs and leads to infections and inflammation. It can also cause severe, long-term damage to the lungs and lead to respiratory failure (inability to breathe normally) and death. In the pancreas, the thick mucus caused by CF prevents the release of digestive enzymes when you eat. This leads to malnutrition and poor growth. CF can also cause liver disease, reproductive problems, and cystic fibrosis-related diabetes (CFRD). More than 40,000 people in the U.S. live with CF. Doctors diagnose about 1,000 new cases each year. Today, more than half of the CF population is aged 18 or older, and new treatments have expanded the life expectancy by decades.

Source: www.webmd.com ↗
04How Do I Take Alyftrek for Cystic Fibrosis?

Your health care provider will decide the correct dose of Alyftrek based on your age and weight. Alyftrek is a tablet that is taken by mouth. It should be swallowed whole with food that contains fat, such as eggs, nuts, meats, or dairy products, to help the body absorb the medicine properly. The treatment plan usually follows these steps after receiving the recommended liver evaluations and tests: For people aged 6 to <12 years (<40 kg): Take three tablets (vanzacaftor 4 mg/tezacaftor 20 mg/ deutivacaftor 50 mg) once daily with fat-containing food. For people aged 6 to <12 years (≥40 kg) or ≥12 years: Take two tablets (vanzacaftor 10 mg/tezacaftor 50 mg/deutivacaftor 125 mg) once daily with fat-containing food. Dose adjustments may be needed in people with liver problems. Alyftrek should be taken at the same time each day to maintain steady levels of the medicine in the body. Keep all appointments with your health care provider before and while taking Alyftrek. Your health care provider may monitor how well your liver is working.

Source: www.webmd.com ↗
05What is it used for?

A sweat test is used to diagnose cystic fibrosis (CF).

Source: medlineplus.gov ↗
Research context

Read sources and limitations before applying a claim.

Research and Statistics: Who Has Cystic Fibrosis?

About 40,000 people are living with cystic fibrosis in the United States, and there are approximately 105,000 people with CF worldwide. (3) More than 75 percent of people with the disease are diagnosed by age 2, and more than half of all people living with cystic fibrosis are 18 or older. CF occurs predominantly in white populations, at a rate of 1 in 2,500 births. Between 2 and 5 percent of white people are carriers of the CFTR gene variant but have no overt clinical signs of disease. The disease is less common among African Americans, occurring at the much lower frequency of approximately 1 out of 17,000 births. (15) CF gene variants are most prevalent in persons of northern and central European ancestries or of Ashkenazi Jewish descent. They are rarely found in Native Americans, Asians, or native Africans. (16) CF is equally common among men and women, but women patients fare significantly worse than male patients with the disease. The median survival age for female CF patients is about three years younger than it is for men, but the reasons for the poorer survival rates among women are not completely understood. (17)

Source: everydayhealth.com ↗
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