How is cystic fibrosis (CF) treated?

In most cases, the patient visits a CF care center four times a year. The caregivers at the center:

  • Show the patient techniques for clearing mucus from the airways, which will aid in keeping his or her lungs healthier.
  • Detect complications of CF and treat these in a timely manner.
  • Offer medications and information about nutrition therapy and physical fitness.

In the last few years, a number of medications called CFTR modulators have been developed to improve functioning of the CFTR protein. These medications are now widely used. Their use depends on which CFTR genes people have, but many individuals who have CF can use these new medications.

Why is a high calorie, high fat diet needed for people with cystic fibrosis (CF)?

Children and adults with cystic fibrosis (CF) have nutritional needs that are different than what would normally be recommended for children and adults without cystic fibrosis. Children and adults may need one and one-half to two times the number of calories as people without CF. More calories are needed because it takes more energy for a person with CF to breathe, fight lung infections and maintain a strong body than a person without CF.

More calories and fats are also needed because the CF prevents the pancreas’s digestive enzymes from fully working as they should, resulting in nutrients and fats from foods not being fully absorbed by the intestines. Although the enzyme capsules that are taken before all meals and snacks helps digest fats, proteins, and starches, a certain amount of nutrients and fats don’t get absorbed. If not enough fats are being absorbed, then fat-soluable vitamins aren’t being fully absorbed and these vitamins are needed to protect the lungs.

It’s also important to emphasize that maintaining a higher than normal weight is very important from early childhood onward. Researchers have shown that young people with CF who maintain a higher weight grow faster and taller up to puberty and again grow taller when they hit their growth spurt at puberty. Young people with CF who started life at a lower weight did not grow as many inches, started puberty at a later age and never got that same puberty growth spurt. Reaching your full genetic potential – getting as tall as possible with lungs as large as possible – is another reason why higher than normal weight in young people with CF is so important.

Salt (sodium) intake. Another common misbelief is that salt (sodium) is unhealthy for all people. This is not the case for children and adults with CF. People with CF lose a lot of salt in their sweat. Although there is not a set standard, doctors generally advise people with CF to eat salty foods especially during hot, humid weather and when they exercise. Also, to add salt to meals and snacks as desired. Ask your doctor or dietician about the amount of salt you need each day.

What are the complications of cystic fibrosis (CF)?

The complications of CF include the following:

  • Adults who have CF can have problems with breathing, digestion, and the reproductive organs.
  • The thick mucus present in people who have CF can hold bacteria, which can cause infection to occur more often.
  • People who have CF have a higher risk of developing diabetes or the bone-thinning conditions osteopenia and osteoporosis.
  • Men who have CF are not able to father children without the aid of alternative reproductive technology. Women who have CF can have a decrease in fertility (the ability to have children) and complications in pregnancy.

Last reviewed by a Cleveland Clinic medical professional on 06/13/2019.

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