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Treatments and drugs
By Mayo Clinic staffMany treatments exist for the symptoms and complications of cystic fibrosis. The main goal is to prevent infections, reduce the amount and thickness of secretions in the lungs, improve airflow, and maintain adequate calories and nutrition.
To accomplish these objectives, cystic fibrosis treatment may include:
- Antibiotics. Newer antibiotics may more effectively fight the bacteria that cause lung infections in people with cystic fibrosis. Among these are aerosolized antibiotics that send medication directly into airways. One of the major drawbacks of long-term use of antibiotics is the development of bacteria that are resistant to drug therapy. In addition, using antibiotics over a long period of time can lead to fungal infections of the mouth, throat and respiratory tract.
- Mucus-thinning drugs. When your white blood cells attack bacteria in your airways, DNA in your cells is released, making the mucus in your airways even thicker. The aerosolized drug dornase alfa (Pulmozyme) is an enzyme that fragments DNA, making mucus thinner and easier to cough up. Side effects of the drug may include airway irritation and sore throat.
- Bronchodilators. Use of medications such as albuterol, which can be delivered by an inhaler or a nebulizer, may help keep open the bronchial tubes by clearing thick secretions.
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Bronchial airway drainage. People with cystic fibrosis need a way to physically remove thick mucus from their lungs. This is often done by manually clapping with cupped hands on the front and back of the chest — a procedure that's best performed with the person's head over the edge of the bed so that gravity helps clear the secretions.
In some cases an electric chest clapper, known as a mechanical percussor, is used. An inflatable vest that vibrates at high frequency also can help people with cystic fibrosis cough up secretions. Many adults and children with pulmonary cystic fibrosis need to have bronchial airway drainage at least twice a day for 20 to 30 minutes. Older children and adults can learn to do this themselves, especially if they use mechanical aids, such as vests and percussors. Young children need the aid of parents, grandparents or older siblings.
- Oral enzymes and better nutrition. Cystic fibrosis can cause you to become malnourished because the pancreatic enzymes needed for digestion don't reach your small intestine, preventing food from being absorbed. As a result, you may need many more calories than you otherwise would. Supplemental high-calorie nutrition, special fat-soluble vitamins and enteric-coated oral pancreatic enzymes can help you maintain or even gain weight.
- Lung transplantation. Your doctor may suggest lung transplantation if you have severe breathing problems, life-threatening pulmonary complications or increasing resistance to antibiotics used to treat lung infections. Whether you're a good candidate for the procedure depends on a number of factors, including your overall health, certain lifestyle factors and the availability of donor organs. Because both lungs are affected by cystic fibrosis, both need to be replaced. If your chest isn't large enough to hold two adult donor lungs, your surgeon is likely to use two lower lobes contributed by two living donors. However it's performed, lung transplantation is a major operation and may lead to serious complications, especially post-surgical infections.
- Pain relievers. Ibuprofen (Advil, Motrin, others) may slow lung deterioration in some children with cystic fibrosis.
Future treatments
Major progress in cystic fibrosis research came in 1989, when researchers identified the genetic mutation that causes the disease. Since then scientists have been studying ways to insert copies of the normal gene into cells of the respiratory tract.
The challenge has been to find a reliable way to deliver the normal genetic material to affected cells that line the airways. Several methods have been developed as delivery systems, including using modified viruses, fat capsules (liposomes) and synthetic vectors. Clinical trials are under way to test the effectiveness of these delivery systems.
Other research is focusing on modifying the protein that the cystic fibrosis gene produces. This may help normalize the movement of salt and water in and out of the cells.
