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 J Schwarzenberg, Alan R. Smyth, Giovanni Taccetti, Thomas O.F. Wagner & 2 others Susan P. Wolfe, Pavel Drevinek

Research output: Contribution to journalReview article

39 Citations (Scopus)

Abstract

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
Volume17
Issue number2
DOIs
StatePublished - Mar 1 2018

Fingerprint

Practice Guidelines
Lung Diseases
Newborn Infant
Precision Medicine
Terminal Care
Quality of Health Care
Standard of Care
Life Expectancy
Consensus
Quality of Life
Psychology
Transplants
Mutation
Survival
Therapeutics

Keywords

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

PubMed: MeSH publication types

  • Journal Article
  • Review

Cite this

Castellani, C., Duff, A. J. A., Bell, S. C., Heijerman, H. G. M., Munck, A., Ratjen, F., ... Drevinek, P. (2018). ECFS best practice guidelines: the 2018 revision. Journal of Cystic Fibrosis, 17(2), 153-178. https://doi.org/10.1016/j.jcf.2018.02.006

ECFS best practice guidelines : the 2018 revision. / Castellani, Carlo; Duff, Alistair J.A.; Bell, Scott C.; Heijerman, Harry G.M.; Munck, Anne; Ratjen, Felix; Sermet-Gaudelus, Isabelle; Southern, Kevin W.; Barben, Jurg; Flume, Patrick A.; Hodková, Pavla; Kashirskaya, Nataliya; Kirszenbaum, Maya N.; Madge, Sue; Oxley, Helen; Plant, Barry; Schwarzenberg, Sarah J; Smyth, Alan R.; Taccetti, Giovanni; Wagner, Thomas O.F.; Wolfe, Susan P.; Drevinek, Pavel.

In: Journal of Cystic Fibrosis, Vol. 17, No. 2, 01.03.2018, p. 153-178.

Research output: Contribution to journalReview article

Castellani, C, Duff, AJA, Bell, SC, Heijerman, HGM, Munck, A, Ratjen, F, Sermet-Gaudelus, I, Southern, KW, Barben, J, Flume, PA, Hodková, P, Kashirskaya, N, Kirszenbaum, MN, Madge, S, Oxley, H, Plant, B, Schwarzenberg, SJ, Smyth, AR, Taccetti, G, Wagner, TOF, Wolfe, SP & Drevinek, P 2018, 'ECFS best practice guidelines: the 2018 revision', Journal of Cystic Fibrosis, vol. 17, no. 2, pp. 153-178. https://doi.org/10.1016/j.jcf.2018.02.006
Castellani C, Duff AJA, Bell SC, Heijerman HGM, Munck A, Ratjen F et al. ECFS best practice guidelines: the 2018 revision. Journal of Cystic Fibrosis. 2018 Mar 1;17(2):153-178. https://doi.org/10.1016/j.jcf.2018.02.006
Castellani, Carlo ; Duff, Alistair J.A. ; Bell, Scott C. ; Heijerman, Harry G.M. ; Munck, Anne ; Ratjen, Felix ; Sermet-Gaudelus, Isabelle ; Southern, Kevin W. ; Barben, Jurg ; Flume, Patrick A. ; Hodková, Pavla ; Kashirskaya, Nataliya ; Kirszenbaum, Maya N. ; Madge, Sue ; Oxley, Helen ; Plant, Barry ; Schwarzenberg, Sarah J ; Smyth, Alan R. ; Taccetti, Giovanni ; Wagner, Thomas O.F. ; Wolfe, Susan P. ; Drevinek, Pavel. / ECFS best practice guidelines : the 2018 revision. In: Journal of Cystic Fibrosis. 2018 ; Vol. 17, No. 2. pp. 153-178.
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