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. It 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. These include a vibrating vest or a tube or mask you breathe into.
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
- Breathing techniques
- Psychological counseling and group support
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.
- Feeding tube. Cystic fibrosis interferes with digestion, so you can't absorb nutrients from food very well. Your doctor may suggest temporarily 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.
- 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. CF does not recur in transplanted lungs.
- What is cystic fibrosis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/cf/. Accessed March 23, 2015.
- Goldman L, et al. Cystic fibrosis. In: Goldman's Cecil Medicine. 24th ed. Philadelphia, Pa.: Saunders Elsevier; 2012. http://www.clinicalkey.com. Accessed March 23, 2015.
- Kliegman RM, et al. Cystic fibrosis. In: Nelson Textbook of Pediatrics. 19th ed. Philadelphia, Pa.: Saunders Elsevier; 2011. http://www.clinicalkey.com. Accessed March 23, 2015.
- Cystic fibrosis. National Library of Medicine. http://ghr.nlm.nih.gov/condition/cystic-fibrosis. Accessed March 23, 2015.
- What is bronchiectasis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/brn/. Accessed March 23, 2015.
- 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.
- AskMayoExpert. Cystic fibrosis. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2014.
- Katkin JP. Cystic fibrosis: Clinical manifestations and diagnosis. http://www.uptodate.com/home. Accessed March 23, 2015.
- Wenstrom KD. Cystic fibrosis: Prenatal genetic screening. http://www.uptodate.com/home. Accessed March 23, 2015.
- Simon RH. Cystic fibrosis: Overview of the treatment of lung disease. http://www.uptodate.com/home. Accessed March 23, 2015.
- Pianosi PT (expert opinion). Mayo Clinic, Rochester, Minn. June 30, 2015.
- Colombo C, et al. Guidelines for the diagnosis and management of distal intestinal obstruction syndrome in cystic fibrosis patients. Journal of Cystic Fibrosis. 2011;10(suppl):S24.
- Use the right GI medications. Cystic Fibrosis Foundation. http://www.cff.org/Adults/GI/Basics/GIMedications. Accessed July 1, 2015.