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

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 SalvatoriDorota 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

Research output: Contribution to journal › Review article › peer-review

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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.

Original language	English
Pages (from-to)	375-387
Number of pages	13
Journal	Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society
Volume	23
Issue number	3
DOIs	https://doi.org/10.1016/j.jcf.2024.05.007
Publication status	Published - 15 Jun 2024

Bibliographical note

Keywords

Cystic Fibrosis/therapy
Humans
Standard of Care
Quality of Life

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10.1016/j.jcf.2024.05.007Licence: CC BY

Standards for the care of people with cystic fibrosis (CF); Planning for a longer life
Copyright 2024 the authors. This is an open access article published under a Creative Commons Attribution License (https://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution and reproduction in any medium, provided the author and source are cited.
Final published version, 715 KBLicence: CC BY

Cite this

Gramegna, A., Addy, C., Allen, L., Bakkeheim, E., Brown, C., Daniels, T., Davies, G., Davies, J. C., De Marie, K., Downey, D., Felton, I., Hafkemeyer, S., Hamouda, S., Kendall, V., Lindberg, U., Macek, M., Mayell, S., Pearlsman, O., Schechter, M. S., ... Southern, K. W. (2024). Standards for the care of people with cystic fibrosis (CF); Planning for a longer life. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 23(3), 375-387. https://doi.org/10.1016/j.jcf.2024.05.007

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

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/therapy, Humans, Standard of Care, Quality of Life",

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

note = "Copyright {\textcopyright} 2024. Published by Elsevier B.V.",

year = "2024",

month = jun,

day = "15",

doi = "10.1016/j.jcf.2024.05.007",

language = "English",

volume = "23",

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journal = "Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society",

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Gramegna, A, Addy, C, Allen, L, Bakkeheim, E, Brown, C, Daniels, T, Davies, G, Davies, JC, De Marie, K, Downey, D, Felton, I, Hafkemeyer, S, Hamouda, S, Kendall, V, Lindberg, U, Macek, M, Mayell, S, Pearlsman, O, Schechter, MS, Salvatori, L, Sands, D, Schwarz, C, Shteinberg, M, Taylor, J, Taylor-Cousar, JL, Taylor-Robinson, D, Watkins, B, Verkleij, M, Bevan, A, Castellani, C, Drevinek, P, Gartner, S, Lammertyn, E, Landau, EEC, Middleton, PG, Plant, BJ, Smyth, AR, van Koningsbruggen-Rietschel, S, Burgel, P-R & Southern, KW 2024, 'Standards for the care of people with cystic fibrosis (CF); Planning for a longer life', Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, vol. 23, no. 3, pp. 375-387. https://doi.org/10.1016/j.jcf.2024.05.007

TY - JOUR

T1 - Standards for the care of people with cystic fibrosis (CF); Planning for a longer life

AU - Gramegna, Andrea

AU - Addy, Charlotte

AU - Allen, Lorna

AU - Bakkeheim, Egil

AU - Brown, Catherine

AU - Daniels, Thomas

AU - Davies, Gwyneth

AU - Davies, Jane C

AU - De Marie, Ken

AU - Downey, Damian

AU - Felton, Imogen

AU - Hafkemeyer, Sylvia

AU - Hamouda, Samia

AU - Kendall, Victoria

AU - Lindberg, Ulrika

AU - Macek, Milan

AU - Mayell, Sarah

AU - Pearlsman, Oren

AU - Schechter, Michael S

AU - Salvatori, Lidia

AU - Sands, Dorota

AU - Schwarz, Carsten

AU - Shteinberg, Michal

AU - Taylor, Julia

AU - Taylor-Cousar, Jennifer L

AU - Taylor-Robinson, David

AU - Watkins, Bethan

AU - Verkleij, Marieke

AU - Bevan, Amanda

AU - Castellani, Carlo

AU - Drevinek, Pavel

AU - Gartner, Silvia

AU - Lammertyn, Elise

AU - Landau, Eddie Edwina C

AU - Middleton, Peter G

AU - Plant, Barry J

AU - Smyth, Alan R

AU - van Koningsbruggen-Rietschel, Silke

AU - Burgel, Pierre-Régis

AU - Southern, Kevin W

PY - 2024/6/15

Y1 - 2024/6/15

N2 - 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.

AB - 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.

KW - Cystic Fibrosis/therapy

KW - Humans

KW - Standard of Care

KW - Quality of Life

U2 - 10.1016/j.jcf.2024.05.007

DO - 10.1016/j.jcf.2024.05.007

M3 - Review article

C2 - 38789317

SN - 1569-1993

VL - 23

SP - 375

EP - 387

JO - Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

JF - Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society

IS - 3

ER -

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

Abstract

Bibliographical note

Keywords

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Cite this