• is an online educational resource on the genetics of cystic fibrosis (CF) and the role of CF transmembrane conductance regulator (CFTR) in normal human physiology as well as in CF.
  • The information contained herein is intended for healthcare professionals working within the European Union (EU).
  • The content of this website has been developed independently by a group of CF healthcare professionals and is for general information purposes only.
  • All of the information about CFTR and CFTR modulators has been obtained from referenced sources and is in the public domain.
  • This website is not intended to promote or advocate a particular course of treatment or any specific drug, whether licensed or unlicensed, nor is it intended to imply or suggest that any drug be used for any purpose other than that which for which it is licensed in the EU.


  • While we endeavour to keep the information up to date and correct, we make no representations or warranties of any kind, express or implied, about the completeness, accuracy, reliability, suitability or availability with respect to the website or the information and related graphics, or products and treatments, contained on the website for any purpose. Any reliance you place on such information is therefore strictly at your own risk.
  • In no event will we be liable for any loss or damage including without limitation, indirect or consequential loss or damage, or any loss or damage whatsoever arising from, or in connection with, the use of this website.
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About CF

In cystic fibrosis (CF), the body’s exocrine glands produce abnormal amounts of mucus-like secretions. This has greatest impact in the lungs, pancreas, liver and intestine, resulting in respiratory infections and complications associated with malnutrition.1

Milestones in CF treatment

Since CF was first described in 1938, there have been significant improvements in the median predicted survival age of sufferers: from only 6 months in 1938, through to about 20 years in 1970, and to 37 years in 2009.2,3 CFTR modulators represent a more recent milestone in CF therapy, as drugs in this class actually treat the underlying cause of CF and not just the symptoms.4


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2. Cohen-Cymberknoh et al. Am J Respir Crit Care Med 2011;183:1463–71
3. Lenny W et al. Thorax 2007;62:1021–2
4. Clancy JP et al. Am J Respir Crit Care Med 2012;186:593–7
5. Andersen DH. Am J Dis Child 1938;56:344–99
6. Di Sant’ Agnese PA et al. Pediatrics 1953;12:549–63
7. Gibson LE & Cooke RE. Pediatrics 1959;23:545–9
8. Quinton PM. Nature 1983;301:421–2
9. Kerem B-S et al. Science 1989;245:1073–80
10. Shak S et al. Proc Natl Acad Sci USA 1990;87:9188–92
11. Zabner J et al. Cell 1993;75:207–16
12. Ramsey BW et al. N Engl J Med 1999;340:23–30
13. European Cystic Fibrosis Society Conference, Dublin, Ireland, 6–9 June 2012
14. European Medicines Agency. Kalydeco (ivacaftor) authorization details. Accessed online 11 Oct 2012