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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 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 distended belly
  • Problems getting enough protein, fat, and calories in the diet

Because of these problems, people with CF may have a hard time staying at a normal weight. Even when 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 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 on waffles.
  • Skim milk powder adds protein. Try adding 2 tablespoons (8.5 grams) 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 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, Green DM, Voynow JA. Cystic fibrosis. In: Kliegman RM, Stanton BF, St. Geme JW, Schor NF, eds. Nelson Textbook of Pediatrics. 20th ed. Philadelphia, PA: Elsevier; 2016:chap 403.

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 www.ncbi.nlm.nih.gov/pubmed/28991007.

Rowe SM, Hoover W, Solomon GM, Sorscher EJ. Cystic fibrosis. In: Broaddus VC, Mason RJ, Ernst JD, et al, eds. Murray and Nadel's Textbook of Respiratory Medicine. 6th ed. Philadelphia, PA: Elsevier Saunders; 2016:chap 47.

    • Cystic fibrosis

      Animation

    •  

      Cystic fibrosis - Animation

      Parents can pass all kinds of different traits to their children, from blue eyes to blonde hair. Sometimes, parents can also pass the genes for certain diseases to their kids. Cystic fibrosis is one very serious inherited disease that makes it hard for children to breathe and digest food. Let's talk about cystic fibrosis. Genes are the coded instructions that tell our bodies how to operate. Usually, the code is correct and everything runs smoothly. But sometimes, the code is incorrect because of a defective gene or genes. In the case of cystic fibrosis, a faulty gene causes the body to produce an abnormally thick, sticky fluid called mucus. This mucus clogs the lungs, making it hard to breathe. It also gets stuck in the pancreas, making it harder for the body to break down and digest food. Millions of Americans carry the cystic fibrosis, or CF gene. Fortunately, most of them don't have cystic fibrosis. That's because you need to inherit one faulty gene from each parent to actually get the disease. Kids who are born with cystic fibrosis start showing signs very early. Newborns don't grow or gain weight as quickly as they should, and they don't make bowel movements because their bodies aren't digesting food properly. As these children get older, they may have symptoms like coughing and fatigue from the mucus in their lungs, and nausea and stomach pain from the mucus in their pancreas. They'll also get pneumonia and other lung infections more often than normal. So, you may now be asking, how do you know if your child has cystic fibrosis? Doctors diagnose cystic fibrosis using a blood test that looks for the CF gene. There is also a sweat test, which looks for saltier-than-normal sweat, another symptom of cystic fibrosis. Doctors may use other tests, such as a chest x-ray or upper GI series, to check for lung and bowel problems caused by CF. It's a good idea to get treated at a center that specializes in cystic fibrosis because they're up on all the latest therapies. For lung problems, treatments include inhaled medicines to open the airways, medicine to thin mucus and make it easier to cough up, and antibiotics to prevent lung infections. Some people may eventually need a lung transplant. For bowel problems, you'll need to eat a special diet that's higher in protein and calories to make up for the nutrients you're losing. You may also take vitamin supplements. The outlook for people with cystic fibrosis is better today than ever before. Thanks to new treatments, people with this disease can live well into adulthood. They can go to school, play sports, and get a job like everyone else. But because the symptoms of cystic fibrosis are so serious, it's very important to stay on top of treatment, and to call the doctor right away if symptoms get worse. Anyone with a family history of the disease may want to get screened for the CF gene before they decide to have children.

    • Cystic fibrosis - nutritional considerations

      Animation

    •  

      Cystic fibrosis - nutritional considerations - Animation

      If you have cystic fibrosis, it's important to eat specific foods and take the right supplements. Let's talk about cystic fibrosis diet. So, why is your diet important? Cystic fibrosis is a disease that causes thick, sticky mucus to build up in your lungs and digestive tract. You need to eat high-calorie, high-protein foods throughout the day to maintain a normal weight because your disease makes it hard to get enough calories from the foods you eat. How do you get the right diet? First of all, you'll need to take a good multivitamin. Make sure it contains vitamins A, D, E, and K. You'll probably need to take pancreatic enzymes, too. These enzymes help your body absorb fat and protein. A good practice is to eat whenever you are hungry. This probably means you'll need to eat several small meals a day. Keep a variety of nutritious snacks around. Try to snack on something every hour, like cheese and crackers, muffins, or trail mix. Make an effort to eat regularly, even if it's only a few bites. Nutritional supplement milkshakes are good to keep on hand. Here's a recommended diet for someone with cystic fibrosis, Add grated cheese to your meals. Drink whole milk. Use enriched milk in cooking. Eat peanut butter. Put it on bread, use it for a dip, and cook with it. Put a teaspoon of butter on hot foods. Dab sour cream or yogurt on vegetables. Eat breaded meat, chicken, and fish. If you get the right balance of extra protein, fat, and calories, and take the right supplements, you can lead a much healthier life.

    • Cystic fibrosis

      Animation

    •  

      Cystic fibrosis - Animation

      Parents can pass all kinds of different traits to their children, from blue eyes to blonde hair. Sometimes, parents can also pass the genes for certain diseases to their kids. Cystic fibrosis is one very serious inherited disease that makes it hard for children to breathe and digest food. Let's talk about cystic fibrosis. Genes are the coded instructions that tell our bodies how to operate. Usually, the code is correct and everything runs smoothly. But sometimes, the code is incorrect because of a defective gene or genes. In the case of cystic fibrosis, a faulty gene causes the body to produce an abnormally thick, sticky fluid called mucus. This mucus clogs the lungs, making it hard to breathe. It also gets stuck in the pancreas, making it harder for the body to break down and digest food. Millions of Americans carry the cystic fibrosis, or CF gene. Fortunately, most of them don't have cystic fibrosis. That's because you need to inherit one faulty gene from each parent to actually get the disease. Kids who are born with cystic fibrosis start showing signs very early. Newborns don't grow or gain weight as quickly as they should, and they don't make bowel movements because their bodies aren't digesting food properly. As these children get older, they may have symptoms like coughing and fatigue from the mucus in their lungs, and nausea and stomach pain from the mucus in their pancreas. They'll also get pneumonia and other lung infections more often than normal. So, you may now be asking, how do you know if your child has cystic fibrosis? Doctors diagnose cystic fibrosis using a blood test that looks for the CF gene. There is also a sweat test, which looks for saltier-than-normal sweat, another symptom of cystic fibrosis. Doctors may use other tests, such as a chest x-ray or upper GI series, to check for lung and bowel problems caused by CF. It's a good idea to get treated at a center that specializes in cystic fibrosis because they're up on all the latest therapies. For lung problems, treatments include inhaled medicines to open the airways, medicine to thin mucus and make it easier to cough up, and antibiotics to prevent lung infections. Some people may eventually need a lung transplant. For bowel problems, you'll need to eat a special diet that's higher in protein and calories to make up for the nutrients you're losing. You may also take vitamin supplements. The outlook for people with cystic fibrosis is better today than ever before. Thanks to new treatments, people with this disease can live well into adulthood. They can go to school, play sports, and get a job like everyone else. But because the symptoms of cystic fibrosis are so serious, it's very important to stay on top of treatment, and to call the doctor right away if symptoms get worse. Anyone with a family history of the disease may want to get screened for the CF gene before they decide to have children.

    • Cystic fibrosis - nutritional considerations

      Animation

    •  

      Cystic fibrosis - nutritional considerations - Animation

      If you have cystic fibrosis, it's important to eat specific foods and take the right supplements. Let's talk about cystic fibrosis diet. So, why is your diet important? Cystic fibrosis is a disease that causes thick, sticky mucus to build up in your lungs and digestive tract. You need to eat high-calorie, high-protein foods throughout the day to maintain a normal weight because your disease makes it hard to get enough calories from the foods you eat. How do you get the right diet? First of all, you'll need to take a good multivitamin. Make sure it contains vitamins A, D, E, and K. You'll probably need to take pancreatic enzymes, too. These enzymes help your body absorb fat and protein. A good practice is to eat whenever you are hungry. This probably means you'll need to eat several small meals a day. Keep a variety of nutritious snacks around. Try to snack on something every hour, like cheese and crackers, muffins, or trail mix. Make an effort to eat regularly, even if it's only a few bites. Nutritional supplement milkshakes are good to keep on hand. Here's a recommended diet for someone with cystic fibrosis, Add grated cheese to your meals. Drink whole milk. Use enriched milk in cooking. Eat peanut butter. Put it on bread, use it for a dip, and cook with it. Put a teaspoon of butter on hot foods. Dab sour cream or yogurt on vegetables. Eat breaded meat, chicken, and fish. If you get the right balance of extra protein, fat, and calories, and take the right supplements, you can lead a much healthier life.

      Tests for Cystic fibrosis - nutrition

       

      Review Date: 5/20/2018

      Reviewed By: Neil K. Kaneshiro, MD, MHA, Clinical Professor of Pediatrics, University of Washington School of Medicine, Seattle, WA. Also reviewed by David Zieve, MD, MHA, Medical Director, Brenda Conaway, Editorial Director, and the A.D.A.M. Editorial team.

      The information provided herein should not be used during any medical emergency or for the diagnosis or treatment of any medical condition. A licensed medical professional should be consulted for diagnosis and treatment of any and all medical conditions. Links to other sites are provided for information only -- they do not constitute endorsements of those other sites. © 1997- A.D.A.M., a business unit of Ebix, Inc. Any duplication or distribution of the information contained herein is strictly prohibited.
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