How Is Cystic Fibrosis Treated?

There still is no cure for cystic fibrosis (CF), but treatments have improved greatly in recent years. The goals of CF treatment are to:

• Prevent and control infections in your lungs.
• Loosen and remove the thick, sticky mucus from your lungs.
• Prevent blockages in your intestines.
• Provide adequate nutrition.

Treatment for Lung Problems

The main treatments for lung problems in people with CF are:

• Antibiotics for infections of the airways
• Chest physical therapy
• Exercise
• Other medications

Antibiotics

Most people with CF have ongoing, low-grade lung infections. Sometimes, these infections become so serious that you may need to be hospitalized. Antibiotics are the primary treatment.

You may be given several different types of antibiotics. The choice of antibiotics depends on:

• The strains of bacteria involved
• How serious your condition is
• Your previous history of antibiotic use

The different types of antibiotics include:

• Oral antibiotics for relatively mild airway infections.
• Inhaled antibiotics, such as tobramycin (to-bra-MI-sin). They may be used alone or with oral antibiotics.
• Intravenous antibiotics for severe infections or when none of the oral antibiotics work.
• Antibiotics, such as azithromycin (az-ith-roe-MYE-sin), that also reduce inflammation.

Chest physical therapy

Chest physical therapy (CPT) is also called chest clapping or percussion. It involves pounding your chest and back over and over again to dislodge the mucus from your lungs so that you can cough up the mucus. CPT for cystic fibrosis should be done three to four times each day.

CPT is also often referred to as postural drainage. This involves your sitting or lying on your stomach with your head down while you do CPT. This allows gravity to help drain the mucus from your lungs.

Because CPT is hard or uncomfortable for some people, several devices have been developed recently that may help with CPT. The devices include:

• An electric chest clapper, known as a mechanical percussor.
• An inflatable therapy vest that uses high-frequency air waves to force the mucus out of your lungs.
• A "flutter" device, a small hand-held device that you breathe out through. It causes vibrations that dislodge the mucus.
• A positive expiratory pressure (PEP) mask that creates vibrations that help break the mucus loose from the airway walls.

Several breathing techniques may also help dislodge the mucus. These techniques include:

• Forced expiration technique (FET)—forcing out a couple of breaths or huffs and then doing relaxed breathing
• Active cycle breathing (ACB)—FET with deep breathing exercises that can loosen the mucus in your lungs and help open your airways

Exercise

Aerobic exercise helps:

• Loosen the mucus
• Encourage coughing to clear the mucus
• Improve your overall physical condition

If you exercise regularly, you may be able to cut back on your chest therapy. Check with your doctor before doing this.

Other medications

Anti-inflammatory medications may help reduce the inflammation in your lungs that is caused by ongoing infections. These medications include:

• Inhaled or, sometimes, oral steroids. Steroids are the most effective anti-inflammatory medicines.
• Ibuprofen, a type of nonsteroidal, anti-inflammatory medicine. It may slow the progress of CF in young children with mild symptoms.
• Bronchodilators, which are inhaled drugs that relax the muscles around the airways so that the airways can open up. They should be taken just before CPT to help clear mucus.

Mucus-thinning drugs reduce the stickiness of mucus in your airways. They include:

• Human DNase (Dornase Alfa), a drug that loosens the mucus in your lungs. It may lead to shorter hospital stays.
• Acetylcysteine and saline.
• Hypertonic saline, a solution of very salty sterile water taken by nebulizer two times a day, can help clear mucus and improve lung function. Some doctors are now giving it to select patients over 6 years old.

Oxygen Therapy

If the level of oxygen in your blood is too low, you may need oxygen therapy. Oxygen is usually given through nasal prongs or a mask.

Lung Transplantation

Surgery to replace one or both of your lungs with healthy lungs from a human donor may help you. Some of the factors that determine whether you can undergo lung transplantation include:

• The type of bacteria in your lungs
• Your age and weight
• The medications you are taking
• Whether you have other medical conditions, including osteoporosis
• How well your lungs are functioning

Management of Digestive Problems

Nutritional therapy can improve your growth and development, strength, and exercise tolerance. It may also make you strong enough to resist some lung infections. Nutritional therapy includes a well-balanced diet that is rich in calories, fat, and protein.

As part of your nutritional therapy, your doctor may:

• Prescribe oral pancreatic enzymes to help you digest fats and proteins and absorb more vitamins. The enzymes should be taken in capsule form before every meal, including snacks.
• Recommend supplements of vitamins A, D, E, and K to replace the fat-soluble vitamins that your intestines cannot absorb.
• Recommend that you use a feeding tube, called a gastrostomy (gas-TROS-to-me) tube or T-tube, to add more calories at night while you are sleeping. The tube is placed in your stomach. Before you go to bed each night, you attach a bottle with a nutritional solution to the entrance of the tube. It feeds you while you sleep.

Other treatments for the digestive problems caused by CF may include:

• Enemas and mucus-thinning medications to treat intestinal blockages
• Medicines that reduce stomach acid and help the oral pancreatic enzymes work better