ECFS best practice guidelines: the 2018 revision
Open accessAbstract
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.
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The resulting mean sweat chloride value in the ELX/TEZ/IVA group is below the diagnostic threshold for CF.
“the resulting mean value is below the diagnostic threshold for CF (figure 3B).18,19”
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- Authors
- Castellani, C., Duff, A. J. A., Bell, S. C., Heijerman, H. G. M., Munck, A., Ratjen, F., Sermet-Gaudelus, I., Southern, K. W., Barben, J., Flume, P. A., Hodková, P., Kashirskaya, N., Kirszenbaum, M. N., Madge, S., Oxley, H., Plant, B., Schwarzenberg, S. J., Smyth, A. R., Taccetti, G., Wagner, T. O. F., Wolfe, S. P., & Drevinek, P.
- Affiliations
- Istituto Giannina Gaslini (IT)
- Leeds General Infirmary (GB)
- Prince Charles Hospital (AU)
- University Medical Center Utrecht (NL)
- University of Toronto (CA)
- University of Liverpool (GB)
- Medical University of South Carolina (US)
- Research Centre for Medical Genetics (RU)
- Hôpital Necker-Enfants Malades (FR)
- Royal Brompton Hospital (GB)
- Wythenshawe Hospital (GB)
- University College Cork (IE)
- University of Minnesota (US)
- University of Nottingham (GB)
- Meyer Children's Hospital (IT)
- Goethe University Frankfurt (DE)
- Leeds Teaching Hospitals NHS Trust (GB)
- Corresponding author
- Duff, Alistair J.A.
- ORCID
- Venue
- Journal of Cystic Fibrosis
- Publisher
- Elsevier BV
- Published
- 2018-03
- Volume
- 17
- Issue
- 2
- Pages
- 153-178
- ISSN
- 1569-1993 (ISSN-L)
- OpenAlex
- W2792598062
- Cited by
- 765
- References
- 15
- Citations by year
- 2026: 48 · 2025: 75 · 2024: 98 · 2023: 104 · 2022: 119 · 2021: 128 · 2020: 105 · 2019: 66 · 2018: 21
- FWCI
- 14.02
- Type
- journal article
- Language
- en
- Open access
- hybrid
- doi.org
- Licence
- Locations
- Journal of Cystic Fibrosis (publishedVersion, cc-by-nc-nd)
- PubMed (publishedVersion)
- White Rose Research Online (University of Leeds, The University of Sheffield, University of York) (acceptedVersion)
- Arrow@dit (Dublin Institute of Technology) (acceptedVersion)
- Cork Open Research Archive (University College Cork, Ireland) (submittedVersion, cc-by-nc-nd)
- Utrecht University Repository (Utrecht University) (submittedVersion, other-oa)
- Publication Server of Goethe University Frankfurt am Main (Goethe University Frankfurt) (submittedVersion, cc-by-nc-nd)
- Funders
- National Institute for Health and Care Research (PB-PG-1207-15025)
- Topics
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Cystic Fibrosis Research Advances
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Neonatal Respiratory Health Research
- Health Sciences › Medicine › Public Health, Environmental and Occupational Health › Clinical practice guidelines implementation
- Keywords
- best practice guidelines, newborn screening, lung disease prevention, nutrition, complications, lung transplantation, end-of-life care, psychological support, personalized medicine, mutation-specific therapy, quality of care
- MeSH terms
- Adolescent, Adult, Child, Child, Preschool, Cystic Fibrosis, Europe, Humans, Infant, Infant, Newborn, Social Support, Terminal Care, Neonatal Screening, Practice Guidelines as Topic, Young Adult
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