Standards for the care of people with cystic fibrosis (CF); Planning for a longer life

Andrea Gramegna; Charlotte Addy; Lorna Allen; Egil Bakkeheim; Catherine Brown; Thomas Daniels; Gwyneth Davies; Jane C Davies; Ken De Marie; Damian Downey; Imogen Felton; Sylvia Hafkemeyer; Samia Hamouda; Victoria Kendall; Ulrika Lindberg; Milan Macek; Sarah Mayell; Oren Pearlsman; Michael S Schechter; Lidia Salvatori; Dorota Sands; Carsten Schwarz; Michal Shteinberg; Julia Taylor; Jennifer L Taylor-Cousar; David Taylor-Robinson; Bethan Watkins; Marieke Verkleij; Amanda Bevan; Carlo Castellani; Pavel Drevinek; Silvia Gartner; Elise Lammertyn; Eddie Edwina C Landau; Peter G Middleton; Barry J Plant; Alan R Smyth; Silke van Koningsbruggen-Rietschel; Pierre-Régis Burgel; Kevin W Southern

doi:10.1016/j.jcf.2024.05.007

Standards for the care of people with cystic fibrosis (CF); Planning for a longer life

J Cyst Fibros. 2024 May;23(3):375-387. doi: 10.1016/j.jcf.2024.05.007. Epub 2024 May 24.

Authors

Andrea Gramegna¹, Charlotte Addy², Lorna Allen³, Egil Bakkeheim⁴, Catherine Brown⁵, Thomas Daniels⁶, Gwyneth Davies⁷, Jane C Davies⁸, Ken De Marie⁹, Damian Downey¹⁰, Imogen Felton¹¹, Sylvia Hafkemeyer¹², Samia Hamouda¹³, Victoria Kendall¹⁴, Ulrika Lindberg¹⁵, Milan Macek¹⁶, Sarah Mayell¹⁷, Oren Pearlsman¹⁸, Michael S Schechter¹⁹, Lidia Salvatori²⁰, Dorota Sands²¹, Carsten Schwarz²², Michal Shteinberg²³, Julia Taylor¹⁴, Jennifer L Taylor-Cousar²⁴, David Taylor-Robinson²⁵, Bethan Watkins², Marieke Verkleij²⁶, Amanda Bevan²⁷, Carlo Castellani²⁸, Pavel Drevinek²⁹, Silvia Gartner³⁰, Elise Lammertyn³¹, Eddie Edwina C Landau³², Peter G Middleton³³, Barry J Plant³⁴, Alan R Smyth³⁵, Silke van Koningsbruggen-Rietschel³⁶, Pierre-Régis Burgel³⁷, Kevin W Southern³⁸

Affiliations

¹ Department of Pathophysiology and Transplantation, Università degli Studi di Milano, Respiratory Unit; Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico, Milano, Italy.
² All Wales Adult Cystic Fibrosis Centre, University Hospital Llandough, Cardiff and Vale University Health Board, Cardiff, UK.
³ Cystic Fibrosis Trust (UK), 2nd Floor, One Aldgate, London, UK.
⁴ Norwegian Resource Centre for Cystic Fibrosis, Oslo University Hospital, Oslo, Norway.
⁵ West Midlands Adult CF Centre, Birmingham, UK.
⁶ NIHR Biomedical Research Centre, School of Clinical and Experimental Sciences, Faculty of Medicine, University of Southampton, Wessex Adult Cystic Fibrosis Service, University Hospital Southampton NHSFT, Southampton, UK.
⁷ Population, Policy and Practice Research and Teaching Department, UCL Great Ormond Street Institute of Child Health, Great Ormond Street Hospital for Children, London, UK.
⁸ National Heart & Lung Institute, Imperial College London, Imperial Biomedical Research Centre, Royal Brompton Hospital, London, UK.
⁹ Belgian CF Association, Brussels, Belgium.
¹⁰ Wellcome-Wolfson Institute for Experimental Medicine, Queen's University Belfast, Belfast, Northern Ireland.
¹¹ Adult Cystic Fibrosis Centre, Royal Brompton Hospital, Guys and St Thomas' Hospital NHS Foundation Trust, London UK, National Heart & Lung Institute, Imperial College London, Imperial Biomedical Research Centre.
¹² Mukoviszidose Institut GmbH, subsidiary of the German Cystic Fibrosis association Mukoviszidose e. V., Bonn, Germany.
¹³ Bechir Hamza Children's Hospital of Tunis, Faculty of Medicine of Tunis, University Al Manar, Tunis, Tunisia.
¹⁴ Manchester Adult Cystic Fibrosis Centre, Manchester University NHS Foundation Trust, Manchester, UK.
¹⁵ Skane University Hospital, Department of clinical sciences, Lund, Respiratory medicine and Allergology, Lund, Sweden.
¹⁶ Department of Biology and Medical Genetics, 2nd Faculty of Medicine Charles University and Motol University Hospital, Prague, Czech Republic.
¹⁷ Alder Hey Children's NHS Foundation Trust, Liverpool, UK.
¹⁸ Israeli Cystic Fibrosis Association, Israel.
¹⁹ Division of Pulmonary and Sleep Medicine, Virginia Commonwealth University, Children's Hospital of Richmond at VCU, USA.
²⁰ Cystic Fibrosis Europe, Brussels, Belgium.
²¹ Cystic Fibrosis Department, Institute of Mother and Child, Warsaw, Poland.
²² HMU-Health and Medical University, Division Cystic Fibrosis, CF Center, Clinic Westbrandenburg, Potsdam, Germany.
²³ Pulmonologuy institute and CF center, Carmel medical center and the Technion- Israel Institute of Technology, Haifa, Israel.
²⁴ Divisions of Pulmonary, Critical Care and Sleep Medicine and Pediatric Pulmonary Medicine, National Jewish Health, Denver, CO, USA, Division of Pulmonary Sciences and Critical Care Medicine, Anschutz Medical Campus, University of Colorado, Aurora, CO, USA.
²⁵ Department of Public Health, Policy and Systems, Waterhouse Building Block F, University of Liverpool, Liverpool, L69 3GB, UK.
²⁶ Amsterdam UMC location University of Amsterdam, Emma Children's Hospital, Child and Adolescent Psychiatry & Psychosocial Care, Amsterdam, the Netherlands.
²⁷ University Hospital Southampton NHS Foundation Trust, Southampton, UK.
²⁸ IRCCS Istituto Giannina Gaslini, Via Gerolamo Gaslini 5, 16147 Genova, Italy.
²⁹ Department of Medical Microbiology, Second Faculty of Medicine, Motol University Hospital, Charles University, Prague, Czech Republic.
³⁰ Cystic Fibrosis Unit and Pediatric Pulmonology, Hospital Universitari Vall d'Hebron, Barcelona, Spain.
³¹ Cystic Fibrosis Europe, the Belgian CF Association, Brussels, Belgium.
³² The Graub CF Center, Pulmonary Institute, Schneider Children's Medical Center, Petah Tikva, Israel.
³³ Westmead Clinical School, University of Sydney and CITRICA, Dept Respiratory & Sleep Medicine, Westmead Hospital, Westmead, Australia.
³⁴ Cork Centre for Cystic Fibrosis (3CF), Cork University Hospital, University College Cork, Cork, Ireland.
³⁵ School of Medicine, Dentistry and Biomedical Sciences, Queens University Belfast, Belfast and NIHR Nottingham Biomedical Research Centre, Nottingham, UK.
³⁶ CF Center Cologne, Children´s Hospital, Faculty of Medicine, University Hospital Cologne, Cologne, Germany.
³⁷ Respiratory Medicine and Cystic Fibrosis National Reference Center, Cochin Hospital, Assistance Publique Hôpitaux de Paris (AP-HP) and Université Paris-Cité, Institut Cochin, Inserm U1016, Paris, France.
³⁸ Department of Women's and Children's Health, University of Liverpool, Liverpool, UK. Electronic address: [email protected].

PMID: 38789317
DOI: 10.1016/j.jcf.2024.05.007

Abstract

This is the final of four papers updating standards for the care of people with CF. That this paper "Planning a longer life" was considered necessary, highlights how much CF care has progressed over the past decade. Several factors underpin this progress, notably increased numbers of people with CF with access to CFTR modulator therapy. As the landscape for CF changes, so do the hopes and aspirations of people with CF and their families. This paper reflects the need to consider people with CF not as a "problem" to be solved, but as a success, a potential and a voice to be heard. People with CF and the wider CF community have driven this approach, reflecting many of the topics in this paper. This exercise involved wide stakeholder engagement. People with CF are keen to contribute to research priorities and be involved in all stages of research. People with CF want healthcare professionals to respect them as individuals and consider the impact of our actions on the world around us. Navigating life presents challenges to all, but for people with CF these challenges are heightened and complex. In this paper we highlight the concerns and life moments that impact people with CF, and events that the CF team should aim to support, including the challenges around having a family. People with CF and their care teams must embrace the updated standards outlined in these four papers to enjoy the full potential for a healthier life.

Keywords: Cystic fibrosis; Family; Longer life; Standards of care.

Publication types

Review

MeSH terms

Cystic Fibrosis* / therapy
Humans
Quality of Life
Standard of Care