Open Access

Carlo Castellani, Alistair J. A. Duff, Scott C. Bell, Harry 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 Schwarzenberg, Alan R. Smyth, Giovanni Taccetti, Thomas O. F. Wagner, Susan P. Wolfe, Pavel Drevinek

• 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.