Cystic Fibrosis Treatment

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

Prevent and control lung infections
Loosen and remove thick, sticky mucus from the lungs
Prevent or treat blockages in the intestines
Provide enough nutrition
Prevent dehydration (a condition in which the body doesn't have enough fluids)

Depending on how severe the disease is, 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.

Treatment for Lung Problems

The main treatments for lung problems in people who have CF are chest physical therapy (CPT), exercise, and medicines.

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 breathe out through. It 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 helps 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 before doing this.

Medicines

If you have CF, you doctor may prescribe antibiotics, anti-inflammatory medicines, bronchodilators, or mucus-thinning medicines. These medicines help treat or prevent lung infections, reduce swelling, open up the airways, and thin mucus.

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 a tube inserted into a vein. This type of treatment may require you to stay in the hospital.

Anti-inflammatory medicines can help reduce swelling in your airways that's caused by ongoing infections. These medicines may be inhaled or oral.

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

Your doctor may prescribe mucus thinners to reduce the stickiness of your mucus and to 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 and the level of oxygen in your blood is low, you may need oxygen therapy. Oxygen usually is given through nasal prongs or a mask.

If other treatments haven't worked, 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.

Treatment for Digestive Problems

CF can cause a number of digestive problems, including poor growth and development, bulky stools, intestinal gas, a swollen belly, severe constipation, and pain or discomfort.

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 additional nutrients.
A high-salt diet or salt supplements that you take before doing vigorous exercise.
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, it may be placed directly into your stomach through a surgically made hole. Before you go to bed each night, you will 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 that people who have CF develop often requires different treatment than other types of diabetes.

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

National Heart, Lung, and Blood Institute