There is no cure for cystic fibrosis, but treatment can ease symptoms and reduce complications. Close monitoring and early, aggressive intervention is recommended. Managing cystic fibrosis is complex, so consider obtaining treatment at a center that specializes in cystic fibrosis.
The goals of treatment include:
- Preventing and controlling lung infections
- Loosening and removing mucus from the lungs
- Preventing and treating intestinal blockage
- Providing adequate nutrition
The options include:
- Antibiotics to treat and prevent lung infections
- Mucus-thinning drugs to help you cough up the mucus, which improves lung function
- Bronchodilators to help keep your airways open by relaxing the muscles around your bronchial tubes
- Oral pancreatic enzymes to help your digestive tract absorb nutrients
Chest physical therapy
Loosening the thick mucus in the lungs makes it easier to cough up. Chest physical therapy helps loosen mucus and is usually done from one to four times a day. A common technique is clapping with cupped hands on the front and back of the chest.
Mechanical devices also can help loosen lung mucus. The options include:
- Chest clapper, a hand-held device that mimics the effect of cupped hands clapping over the ribs
- Inflatable vest, a device worn around the chest that vibrates at high frequency
- Breathing devices, usually a tube or mask through which you exhale while performing breathing exercises
Your doctor may recommend a long-term program to improve your lung function and overall well-being. Pulmonary rehabilitation is usually done on an outpatient basis and may include:
- Exercise training
- Nutritional counseling
- Energy-conserving techniques
- Breathing strategies
- Psychological counseling or group support or both
Surgical and other procedures
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- Nasal polyp removal. Your doctor may recommend surgery to remove nasal polyps that obstruct breathing.
- Oxygen therapy. If your blood-oxygen level declines, your doctor may recommend you sometimes breathe pure oxygen to prevent high blood pressure in the lungs (pulmonary hypertension).
- Endoscopy and lavage. Mucus may be suctioned from obstructed airways through an endoscope.
- Lung transplant. If you have severe breathing problems, life-threatening lung complications or increasing resistance to antibiotics used to treat lung infections, lung transplantation may be an option. Because both lungs are affected by cystic fibrosis, both need to be replaced. Lung transplants for people with cystic fibrosis are controversial because studies indicate the procedure is associated with many complications, and may not prolong life or enhance quality of life.
- Feeding tube. Cystic fibrosis interferes with digestion, so you can't absorb nutrients from food very well. Your doctor may suggest using a feeding tube to deliver extra nutrition while you sleep. This tube may be threaded through your nose to your stomach or surgically implanted into the abdomen.
- Bowel surgery. If a blockage develops in your bowel, you may need surgery to remove it. Intussusception, where a section of bowel has folded in on itself, also may require surgical repair.
- What is cystic fibrosis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/cf/. Accessed March 26, 2012.
- Goldman L, et al. Cecil Medicine. 24th ed. Philadelphia, Pa.: Saunders Elsevier; 2012. http://www.mdconsult.com/books/about.do?about=true&eid=4-u1.0-B978-1-4377-1604-7..C2009-0-42832-0--TOP&isbn=978-1-4377-1604-7&uniqId=327451096-2. Accessed March 26, 2012.
- Kliegman RM, et al. Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa.: Saunders Elsevier; 2011. http://www.mdconsult.com/books/about.do?about=true&eid=4-u1.0-B978-1-4377-0755-7..C2009-0-60010-6--TOP&isbn=978-1-4377-0755-7&uniqId=327451096-2. Accessed March 27, 2012.
- Cystic fibrosis. National Library of Medicine. http://ghr.nlm.nih.gov/condition/cystic-fibrosis. Accessed April 2, 2012.
- What is bronchiectasis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/brn/brn_whatis.html. Accessed March 27, 2012.
- Cohen-Cymberknoh M, et al. Managing cystic fibrosis: strategies that increase life expectancy and improve quality of life. American Journal of Respiratory and Critical Care Medicine. 2011;183:1463.
- Rogan MP, et al. Cystic fibrosis transmembrane conductance regulator intracellular processing, trafficking, and opportunities for mutation-specific treatment. Chest. 2011;139:1480.
- Mogayzel PJ, et al. Update in cystic fibrosis 2010. American Journal of Respiratory and Critical Care Medicine. 2011;183:1620.