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How Is Cystic Fibrosis Treated?

Cystic fibrosis (CF) has no cure. However, treatments have greatly improved in recent years. The goals of CF treatment include:

  • Preventing and controlling lung infections
  • Loosening and removing thick, sticky mucus from the lungs
  • Preventing or treating blockages in the intestines
  • Providing enough nutrition
  • Preventing dehydration (a lack of fluid in the body)

Depending on the severity of CF, you or your child may be treated in a hospital.

Specialists Involved

If you or your child has CF, you may be treated by a CF specialist. This is a doctor who is familiar with the complex nature of CF.

Often, a CF specialist works with a medical team of nurses, physical therapists, dietitians, and social workers. CF specialists often are located at major medical centers.

The United States also has more than 100 CF Care Centers. These centers have teams of doctors, nurses, dietitians, respiratory therapists, physical therapists, and social workers who have special training related to CF care. Most CF Care Centers have pediatric and adult programs or clinics.

For more information about CF Care Centers, go to the Cystic Fibrosis Foundation's Care Center Network Web page.

Treatment for Lung Problems

The main treatments for lung problems in people who have CF are chest physical therapy (CPT), exercise, and medicines. Your doctor also may recommend a pulmonary rehabilitation (PR) program.

Chest Physical Therapy

CPT also is called chest clapping or percussion. It involves pounding your chest and back over and over with your hands or a device to loosen the mucus from your lungs so that you can cough it up.

You might sit down or lie on your stomach with your head down while you do CPT. Gravity and force help drain the mucus from your lungs.

Some people find CPT hard or uncomfortable to do. Several devices have been developed that may help with CPT, such as:

  • An electric chest clapper, known as a mechanical percussor.
  • An inflatable therapy vest that uses high-frequency airwaves to force the mucus that's deep in your lungs toward your upper airways so you can cough it up.
  • A small, handheld device that you exhale through. The device causes vibrations that dislodge the mucus.
  • A mask that creates vibrations that help break the mucus loose from your airway walls.

Breathing techniques also may help dislodge mucus so you can cough it up. These techniques include forcing out a couple of short breaths or deeper breaths and then doing relaxed breathing. This may help loosen the mucus in your lungs and open your airways.

Exercise

Aerobic exercise that makes you breathe harder can help loosen the mucus in your airways so you can cough it up. Exercise also helps improve your overall physical condition.

However, CF causes your sweat to become very salty. As a result, your body loses large amounts of salt when you sweat. Thus, your doctor may recommend a high-salt diet or salt supplements to maintain the balance of minerals in your blood.

If you exercise regularly, you may be able to cut back on your CPT. However, you should check with your doctor first.

Medicines

If you have CF, your doctor may prescribe antibiotics, anti-inflammatory medicines, bronchodilators, or medicines to help clear the mucus. These medicines help treat or prevent lung infections, reduce swelling and open up the airways, and thin mucus. If you have mutations in a gene called G551D, which occurs in about 5 percent of people who have CF, your doctor may prescribe the oral medicine ivacaftor (approved for people with CF who are 6 years of age and older).

Antibiotics are the main treatment to prevent or treat lung infections. Your doctor may prescribe oral, inhaled, or intravenous (IV) antibiotics.

Oral antibiotics often are used to treat mild lung infections. Inhaled antibiotics may be used to prevent or control infections caused by the bacteria mucoid Pseudomonas. For severe or hard-to-treat infections, you may be given antibiotics through an IV tube (a tube inserted into a vein). This type of treatment may require you to stay in a hospital.

Anti-inflammatory medicines can help reduce swelling in your airways due to ongoing infections. These medicines may be inhaled or oral.

Bronchodilators help open the airways by relaxing the muscles around them. These medicines are inhaled. They're often taken just before CPT to help clear mucus out of your airways. You also may take bronchodilators before inhaling other medicines into your lungs.

Your doctor may prescribe medicines to reduce the stickiness of your mucus and loosen it up. These medicines can help clear out mucus, improve lung function, and prevent worsening lung symptoms.

Treatments for Advanced Lung Disease

If you have advanced lung disease, you may need oxygen therapy. Oxygen usually is given through nasal prongs or a mask.

If other treatments haven't worked, a lung transplant may be an option if you have severe lung disease. A lung transplant is surgery to remove a person's diseased lung and replace it with a healthy lung from a deceased donor.

Pulmonary Rehabilitation

Your doctor may recommend PR as part of your treatment plan. PR is a broad program that helps improve the well-being of people who have chronic (ongoing) breathing problems.

PR doesn't replace medical therapy. Instead, it's used with medical therapy and may include:

  • Exercise training
  • Nutritional counseling
  • Education on your lung disease or condition and how to manage it
  • Energy-conserving techniques
  • Breathing strategies
  • Psychological counseling and/or group support

PR has many benefits. It can improve your ability to function and your quality of life. The program also may help relieve your breathing problems. Even if you have advanced lung disease, you can still benefit from PR.

For more information, go to the Health Topics Pulmonary Rehabilitation article.

Treatment for Digestive Problems

CF can cause many digestive problems, such as bulky stools, intestinal gas, a swollen belly, severe constipation, and pain or discomfort. Digestive problems also can lead to poor growth and development in children.

Nutritional therapy can improve your strength and ability to stay active. It also can improve growth and development in children. Nutritional therapy also may make you strong enough to resist some lung infections. A nutritionist can help you create a nutritional plan that meets your needs.

In addition to having a well-balanced diet that's rich in calories, fat, and protein, your nutritional therapy may include:

  • Oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins.
  • Supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that your intestines can't absorb.
  • High-calorie shakes to provide you with extra nutrients.
  • A high-salt diet or salt supplements that you take before exercising.
  • A feeding tube to give you more calories at night while you're sleeping. The tube may be threaded through your nose and throat and into your stomach. Or, the tube may be placed directly into your stomach through a surgically made hole. Before you go to bed each night, you'll attach a bag with a nutritional solution to the entrance of the tube. It will feed you while you sleep.

Other treatments for digestive problems may include enemas and mucus-thinning medicines to treat intestinal blockages. Sometimes surgery is needed to remove an intestinal blockage.

Your doctor also may prescribe medicines to reduce your stomach acid and help oral pancreatic enzymes work better.

Treatments for Cystic Fibrosis Complications

A common complication of CF is diabetes. The type of diabetes associated with CF often requires different treatment than other types of diabetes.

Another common CF complication is the bone-thinning disorder osteoporosis. Your doctor may prescribe medicines that prevent your bones from losing their density.

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Cystic Fibrosis Clinical Trials

Clinical trials are research studies that explore whether a medical strategy, treatment, or device is safe and effective for humans. To find clinical trials that are currently underway for Cystic Fibrosis, visit www.clinicaltrials.gov.

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Visit Children and Clinical Studies to hear experts, parents, and children talk about their experiences with clinical research.


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