Article Text

Download PDFPDF
Three modulator drugs are better than two in cystic fibrosis

Statistics from

The development of radically new treatments for cystic fibrosis (CF) proceeds apace. In February 2018 Archivist reported on trials of double-modulator therapies (tezacaftor-ivacaftor) that produced modest benefits (doi:10.1136/archdischild-2017–3 14 602). Adding a third agent could, in theory, further enhance the intracellular functioning of the defective CFTR protein by binding to different sites on the molecule, particularly in those with the most common Phe508del mutation.

Two such agents which showed promise in vitro were used in …

View Full Text


  • Patient consent for publication Not required.

  • Competing interests None declared.

  • Provenance and peer review Commissioned; internally peer reviewed.

Request Permissions

If you wish to reuse any or all of this article please use the link below which will take you to the Copyright Clearance Center’s RightsLink service. You will be able to get a quick price and instant permission to reuse the content in many different ways.