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Treatments and drugs
By Mayo Clinic staffThere is no cure for cystic fibrosis, but treatments can ease symptoms and reduce complications.
Medications
- Antibiotics. These drugs are used to treat and prevent lung infections. They may be swallowed in pill form, inhaled in a mist or delivered intravenously.
- Mucus-thinning drugs. Drugs that reduce the stickiness of your mucus make it easier to cough up the mucus, which improves lung function.
- Bronchodilators. Medications such as albuterol — delivered by an inhaler or a nebulizer — help keep your airways open by relaxing the muscles around your bronchial tubes.
Therapy
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.
There also are mechanical devices that help loosen lung mucus. They include:
- Chest clapper. This hand-held device can mimic the effect of cupped hands clapping over the ribs.
- Inflatable vest. This device vibrates at high frequency to loosen chest mucus.
- Breathing devices. Performing specific breathing exercises while exhaling through the device's tube or a mask may also be helpful.
Surgical and other procedures
- 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.
- Lung transplant. 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. Because both lungs are affected by cystic fibrosis, both need to be replaced. Lung transplantation is a major operation and may lead to serious complications, especially post-surgical infections.
- Bowel surgery. If you have developed a blockage in your bowel, you may need emergency surgery to remove it — especially if that part of your bowel has died. Intussusception, where a section of bowel has folded in on itself, also may require surgical repair.
References
- What is cystic fibrosis? National Heart Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/cf/cf_all.html. Accessed Dec. 9, 2009.
- Welsh MJ. Cystic fibrosis. In: Goldman L, et al. Cecil Medicine. 23rd ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/175336803-3/0/1492/366.html#4-u1.0-B978-1-4160-2805-5..50094-X_3680. Accessed Dec. 11, 2009.
- Boat TF, et al. Cystic fibrosis. In: Kliegman RM, et al. Nelson Textbook of Pediatrics. 18th ed. Philadelphia, Pa.: Saunders Elsevier; 2007. http://www.mdconsult.com/das/book/body/175336803-3/0/1608/945.html#4-u1.0-B978-1-4160-2450-7..50402-3--cesec14_7740. Accessed Dec. 11, 2009.
- Katkin JP. Cystic fibrosis: Clinical manifestations and diagnosis. http://www.uptodate.com/hme/index.html. Accessed Dec. 11, 2009.
- Cystic fibrosis. Genetics Home Reference. National Institutes of Health. http://ghr.nlm.nih.gov/condition=cysticfibrosis. Accessed Dec. 11, 2009.
- What is bronchiectasis? National Heart, Lung and Blood Institute. http://www.nhlbi.nih.gov/health/dci/Diseases/brn/brn_whatis.html. Accessed Jan. 4, 2010.
- Simon RH. Cystic fibrosis: Overview of the treatment of lung disease. http://www.uptodate.com/home/index.html. Accessed Dec. 14, 2009.
- An introduction to postural drainage and percussion. Cystic Fibrosis Foundation. http://www.cff.org/UploadedFiles/treatments/Therapies/Respiratory/PosturalDrainage/An%20Introduction%20to%20Postural%20Drainage%20and%20Percussion%201-2006.pdf. Accessed Dec. 14, 2009.
- Airway clearance techniques. Cystic Fibrosis Foundation. http://www.cff.org/treatments/Therapies/Respiratory/AirwayClearance/. Accessed Dec. 14, 2009.
- Baker RD, et al. Cystic fibrosis: Nutritional issues. http://www.uptodate.com/home/index.html. Accessed Dec. 14, 2009.
- Wenstrom KD. Cystic fibrosis: Prenatal genetic screening. http://www.uptodate.com/home/index.html. Accessed Dec. 14, 2009.
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