Overview

Cystic fibrosis is an inherited disorder that causes severe damage to the lungs, digestive system and other organs in the body.

Cystic fibrosis affects the cells that produce mucus, sweat and digestive juices. These secreted fluids are normally thin and slippery. But in people with cystic fibrosis, a defective gene causes the secretions to become sticky and thick. Instead of acting as a lubricant, the secretions plug up tubes, ducts and passageways, especially in the lungs and pancreas.

Although cystic fibrosis requires daily care, people with the condition are usually able to attend school and work, and often have a better quality of life than people with cystic fibrosis had in previous decades. Improvements in screening and treatments mean people with cystic fibrosis now may live into their mid- to late 30s, on average, and some are living into their 40s and 50s.

Cystic fibrosis care at Mayo Clinic

Oct. 13, 2016
References
  1. What is cystic fibrosis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/cf. Accessed July 1, 2016.
  2. Goldman L, et al., eds. Cystic fibrosis. In: Goldman-Cecil Medicine. 25th ed. Philadelphia, Pa.: Saunders Elsevier; 2016. http://www.clinicalkey.com. Accessed April 13, 2016.
  3. Kliegman RM, et al. Cystic fibrosis. In: Nelson Textbook of Pediatrics. 20th ed. Philadelphia, Pa.: Elsevier; 2016. http://www.clinicalkey.com. Accessed April 13, 2016.
  4. National Library of Medicine. Cystic fibrosis. Genetics Home Reference. https://ghr.nlm.nih.gov/condition/cystic-fibrosis. Accessed May 3, 2016.
  5. What is bronchiectasis? National Heart, Lung, and Blood Institute. http://www.nhlbi.nih.gov/health/health-topics/topics/brn. Accessed April 13, 2016.
  6. AskMayoExpert. Cystic fibrosis. Rochester, Minn.: Mayo Foundation for Medical Education and Research; 2016.
  7. Katkin JP. Cystic fibrosis: Clinical manifestations and diagnosis. http://www.uptodate.com/home. Accessed April 13, 2016.
  8. Katkin JP. Cystic fibrosis: Genetics and pathogenesis. http://www.uptodate.com/home. Accessed April 28, 2016.
  9. Anatomic problems of the lower GI tract. National Institute of Diabetes and Digestive and Kidney Diseases. https://www.niddk.nih.gov/health-information/health-topics/digestive-diseases/anatomic-colon/Pages/facts.aspx#Intussusception. Accessed June 24, 2016.
  10. Simon RH. Cystic fibrosis: Overview of the treatment of lung disease. http://www.uptodate.com/home. Accessed April 13, 2016.
  11. Simon RH, et al. Cystic fibrosis: Investigational therapies. http://www.uptodate.com/home. Accessed June 28, 2016.
  12. Quon BS, et al. New and emerging targeted therapies for cystic fibrosis. BMJ. 2016;352:i859.
  13. Elborn JS. Cystic fibrosis. Lancet. In press. Accessed June 15, 2016.
  14. Orkambi (prescribing information). Boston, Mass.: Vertex Pharmaceuticals Inc.; 2016. http://pi.vrtx.com/files/uspi_lumacaftor_ivacaftor.pdf. Accessed June 30, 2016.
  15. Riggin ER. Allscripts EPSi. Mayo Clinic, Rochester, Minn. April 8, 2016.
  16. Erasmus DB (expert opinion). Mayo Clinic, Jacksonville, Fla. Aug. 4, 2016.

Connect with others

News, connections and conversations for your health

Recent posts