There is no known cure for cystic fibrosis. Treatments focus on improving the patient’s capacity to breathe and eliminating infections.

Chest physical therapy (CPT) is a technique commonly used to loosen mucus. In this therapy, patients are gently but firmly pounded on the chest and back to dislodge mucus from the lungs. The movement involved is sometimes called chest clapping or percussion. The patient then coughs up the mucus. Chest clapping usually is performed two to four times daily. During CPT, patients may either sit or lie on their stomachs with head down. This allows gravity to help drain the mucus from the lungs.

Devices have been developed that can aid CPT. These include an electric chest clapper (also known as a mechanical percussor), inflatable therapy vest (which uses high-frequency airwaves to force mucus from the lungs), a “flutter” device (causes vibrations that dislodge mucus) and positive expiratory pressure (mask that creates vibrations that break mucus loose from airway walls). Children typically require the assistance of a therapist or caregiver in performing CPT. Adult patients or patients in their teens may learn techniques to perform CPT alone or with mechanical aids.

Certain breathing techniques can also help loosen mucus. The forced expiration technique involves forcing out two breaths and then engaging in relaxed breathing. Active cycle breathing uses the same technique, but also focuses on deep breathing exercises to help loosen mucus.

Regular aerobic exercise also can increase a person’s lung capacity, loosen mucus and trigger coughing that helps clear the mucus. Patients who exercise regularly may be able to reduce their need for CPT.

Infections of the airways tend to be ongoing and low-grade in patients with cystic fibrosis. In some cases, these infections are serious enough that they may require hospitalization. Antibiotics are used to treat these infections. Oral antibiotics may be used to treat relatively mild infections. Inhaled (aerosolized) and intravenous (into a vein) antibiotics may be used for more significant infections.

Other medications that may be used to treat cystic fibrosis include:

• Bronchodilators. Inhaled drugs that function by relaxing the muscles around airways, allowing the airways to open. They usually are taken just before CPT to help clear mucus.
  
  
• Corticosteroids. These drugs reduce inflammation around the lungs.
  
  
• Ibuprofen. An over-the-counter or prescription nonsteroidal anti-inflammatory drug (NSAID) that may help reduce the progression of cystic fibrosis in young children.
  
  
• Insulin. This diabetes drug may be prescribed for patients with cystic fibrosis-related diabetes (CFRD).
  
  
• Mucus-thinning drugs. These medications reduce the stickiness of mucus in the airways.
  
  
• Enzymes and nutritional medications. People with cystic fibrosis, particularly children, may require oral pancreatic enzymes to aid in digestion of fats and proteins and absorption of vitamins. A physician also may recommend a high-calorie diet and vitamin supplements.

Feeding tubes may be necessary for patients who are at risk of malnutrition and who do not respond to other nutrition therapies. Some patients may require the implantation of a gastrostomy tube that can be used to deliver nutrients directly into the stomach. These tubes may be used in children at night to provide nourishment while asleep.

Patients who do not receive adequate levels of oxygen may require oxygen therapy. This is usually delivered through oxygen prongs or a mask.

In severe cases, patients may benefit from lung transplantation. Many factors help determine whether a person is a good candidate for transplantation. These include the type of bacteria present in the lungs, patient age and weight, and the nature of the patient's other medical conditions. Both lungs require transplantation in people with cystic fibrosis, which may reduce the availability of donor organs.

Researchers are investigating potential treatments for cystic fibrosis, including gene therapy and new medications.