ECFS best practice guidelines: the 2018 revision

Carlo Castellani, Alistair J.A. Duff, Scott C. Bell, Harry G.M. Heijerman, Anne Munck, Felix Ratjen, Isabelle Sermet-Gaudelus, Kevin W. Southern, Jurg Barben, Patrick A. Flume, Pavla Hodková, Nataliya Kashirskaya, Maya N. Kirszenbaum, Sue Madge, Helen Oxley, Barry Plant, Sarah Jane Schwarzenberg, Alan R. Smyth, Giovanni Taccetti, Thomas O.F. WagnerSusan P. Wolfe, Pavel Drevinek

Research output: Contribution to journalReview articlepeer-review

427 Scopus citations


Developments in managing CF continue to drive dramatic improvements in survival. As newborn screening rolls-out across Europe, CF centres are increasingly caring for cohorts of patients who have minimal lung disease on diagnosis. With the introduction of mutation-specific therapies and the prospect of truly personalised medicine, patients have the potential to enjoy good quality of life in adulthood with ever-increasing life expectancy. The landmark Standards of Care published in 2005 set out what high quality CF care is and how it can be delivered throughout Europe. This underwent a fundamental re-write in 2014, resulting in three documents; center framework, quality management and best practice guidelines. This document is a revision of the latter, updating standards for best practice in key aspects of CF care, in the context of a fast-moving and dynamic field. In continuing to give a broad overview of the standards expected for newborn screening, diagnosis, preventative treatment of lung disease, nutrition, complications, transplant/end of life care and psychological support, this consensus on best practice is expected to prove useful to clinical teams both in countries where CF care is developing and those with established CF centres. The document is an ECFS product and endorsed by the CF Network in ERN LUNG and CF Europe.

Original languageEnglish (US)
Pages (from-to)153-178
Number of pages26
JournalJournal of Cystic Fibrosis
Issue number2
StatePublished - Mar 2018

Bibliographical note

Funding Information:
C Castellani: personal fees from PTC therapeutics, Gilead, Vertex, Pharmaxis outside the submitted work; A.J.A. Duff: grants and personal fees from Chiesi Pharmaceuticals UK Ltd., personal fees from Novartis Pharmaceuticals UK Ltd., personal fees and non-financial support from Profile Pharma/Zambon and TEVA Ltd., outside the submitted work; S.C. Bell personal fees and travel support from Rempex and Novartis, grants and non-financial support from Vertex, grants, non-financial support and travel support from Galapagos, non-financial support and travel support from Abbvie, travel support from Gilead, outside the submitted work; H.G.M. Heijerman: personal fees from Vertex, Horizon Pharma, PTC and Gilead outside the submitted work; A. Munck reports personal fees from Vertex, Novartis and Mayoli Spindler outside the submitted work; F. Ratjen: reports grants and personal fees from Vertex and personal fees from Novartis and Genetech, outside the submitted work; P.A. Flume: grants and personal fees from Bayer Healthcare AG, Corbus Pharmaceuticals, Insmed, Pharmaxis Limited, Proteostasis Therapeutics, Savara Pharma and Vertex Pharmaceuticals, grants from the Cystic Fibrosis Foundation, Galapagos, National Institutes of Health, Novartis, Novoteris, Pro-QR, Sound Pharmaceuticals Inc. outside the submitted work; P. Hodková: personal fees from Vertex outside the submitted work; N. Kashirskaya: personal fees from Abbott and Chiesi Farmaceutici outside the submitted work; M. Kirszenbaum reports personal fees from Vertex outside the submitted work; S. Madge reports grants from Gilead and Medscape, travel support from Horizon and Vertex outside the submitted work; H. Oxley: non-financial support from TEVA outside the submitted work; S.J. Schwarzenberg: personal fees from Spark HealthCare outside the submitted work; T.O.F. Wagner: grants from Bayer, Boehringer, Novartis and Vertex outside the submitted work; P. Drevinek: personal fees from Vertex, Galapagos and ProQR outside the submitted work and financial support from The European Cystic Fibrosis Society for the ongoing Standards of Care Project; J. Barben, B.Plant, I. Sermet-Gaudelus, A.R. Smyth, K.W. Southern, G. Taccetti and S.P. Wolfe have no conflicts of interest to report.

Publisher Copyright:
© 2018 The Author(s)


  • Best practice
  • Consensus
  • Cystic fibrosis
  • Guidelines
  • Multidisciplinary management
  • Standards of care


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