. 2024 Jun;59(6):1661-1676.

doi: 10.1002/ppul.26970. Epub 2024 Apr 12.

SHIFTing goals in cystic fibrosis-managing extrapulmonary disease in the era of CFTR modulator therapy; Proceedings of the International Shaping Initiatives and Future Trends (SHIFT) Symposium

Jonathan E M O'Donnell^{1

2}, Lucy A Hastings³, Julie N Briody⁴, Christine L Chan^{5

6}, Carla Colombo⁷, Tonia A Douglas^{8

9}, Steven D Freedman^{10

11}, Tanja Gonska^{12

13}, Jerry R Greenfield^{14

15

16}, Daniel H Leung^{17

18}, Adeline Y L Lim⁸, Antoinette Moran¹⁹, Bernadette J Prentice²⁰, Melissa S Putman²¹, Michael Trotter²², Elizabeth Tullis^{23

24}, Glen P Westall^{25

26}, Charles F Verge^{2

3}, Claire E Wainwright^{8

27}, Chee Y Ooi^{1

2}; SHIFT Symposium 2021

Collaborators, Affiliations

Collaborators

SHIFT Symposium 2021:
John F Engelhardt²⁸, Tamarah Katz²⁹, Susannah King³⁰, Peter J Lewindon^{9

31}, Angela G Matson³², Catherine M McDonald³³, Amir Moheet³⁴, Michael R Narkewicz³⁵, Mark Oliver³⁶, Grant A Ramm^{9

37}, Zachary M Sellers³⁸, Michael Wilschanski³⁹

Affiliations

¹ Department of Gastroenterology, Sydney Children's Hospital, University of New South Wales, Sydney, Australia.
² School of Clinical Medicine, Discipline of Pediatrics and Child Health, UNSW Medicine & Health, University of New South Wales, Sydney, Australia.
³ Department of Endocrinology, Sydney Children's Hospital Randwick, Sydney Children's Hospitals Network, Sydney, Australia.
⁴ Nuclear Medicine, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
⁵ University of Colorado Anschutz Medical Center, Aurora, Colorado, USA.
⁶ Children's Hospital Colorado, Aurora, Colorado, USA.
⁷ CF Center, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
⁸ Department of Respiratory and Sleep Medicine, Queensland Children's Hospital, South Brisbane, Australia.
⁹ Faculty of Medicine, The University of Queensland, Brisbane, Australia.
¹⁰ Israel Deaconess Medical Center, Boston, Massachusetts, USA.
¹¹ Harvard Medical School, Boston, Massachusetts, USA.
¹² Division of Gastroenterology, Hepatology and Nutrition, Dep of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
¹³ Research Institute, the Hospital for Sick Children, Toronto, Ontario, Canada.
¹⁴ Endocrinology and Diabetes, St Vincent's Hospital Sydney, Darlinghurst, Australia.
¹⁵ School of Clinical Medicine, The University of New South Wales, Sydney, Australia.
¹⁶ Garvan Institute, Sydney, Australia.
¹⁷ Baylor College of Medicine, Houston, Texas, USA.
¹⁸ Division of Pediatric Gastroenterology, Hepatology and Nutrition, Texas Children's Hospital, Houston, Texas, USA.
¹⁹ University of Minnesota, Minneapolis, Minnesota, USA.
²⁰ Department of Respiratory Medicine, Sydney Children's Hospital, Randwick, Australia.
²¹ Diabetes Research Center, Massachusetts General Hospital, Boston, Massachusetts, USA.
²² Queensland Lung Transplant Service, The Prince Charles Hospital, Brisbane, Australia.
²³ St Michael's Hospital, Toronto, Ontario, Canada.
²⁴ University of Toronto, Toronto, Ontario, Canada.
²⁵ Lung Transplant Service, Alfred Health, Melbourne, Australia.
²⁶ Central Clinical School, Monash University, Melbourne, Australia.
²⁷ Child Health Research Center (CHRC), The University of Queensland, Brisbane, Australia.
²⁸ Department of Anatomy and Cell Biology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
²⁹ Department of Nutrition and Dietetics, Sydney Children's Hospital, Sydney, Australia.
³⁰ Nutrition Department, Alfred Health, Melbourne, Victoria, Australia.
³¹ Department of Gastroenterology, Hepatology and Liver Transplant, Queensland Children's Hospital, Brisbane, Australia.
³² CF Endocrine Service Adult Cystic Fibrosis Center (ACFC) The Prince Charles Hospital, Australia.
³³ Primary Children's Hospital, Salt Lake City, Utah, USA.
³⁴ Department of Medicine, University of Minnesota, Minneapolis, Minnesota, USA.
³⁵ Digestive Health Institute, Children's Hospital Colorado and Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Aurora, Colorado, USA.
³⁶ Department of Gastroenterology, Royal Children's Hospital, University of Melbourne, Melbourne, Australia.
³⁷ Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, Herston, Australia.
³⁸ Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Stanford University, Palo Alto, California, USA.
³⁹ Pediatric Gastroenterology, Hadassah University Hospitals, Jerusalem, Israel.

PMID: 39903130
DOI: 10.1002/ppul.26970

SHIFTing goals in cystic fibrosis-managing extrapulmonary disease in the era of CFTR modulator therapy; Proceedings of the International Shaping Initiatives and Future Trends (SHIFT) Symposium

Jonathan E M O'Donnell et al. Pediatr Pulmonol. 2024 Jun.

. 2024 Jun;59(6):1661-1676.

doi: 10.1002/ppul.26970. Epub 2024 Apr 12.

Authors

Collaborators

SHIFT Symposium 2021:
John F Engelhardt²⁸, Tamarah Katz²⁹, Susannah King³⁰, Peter J Lewindon^{9

31}, Angela G Matson³², Catherine M McDonald³³, Amir Moheet³⁴, Michael R Narkewicz³⁵, Mark Oliver³⁶, Grant A Ramm^{9

37}, Zachary M Sellers³⁸, Michael Wilschanski³⁹

Affiliations

¹ Department of Gastroenterology, Sydney Children's Hospital, University of New South Wales, Sydney, Australia.
² School of Clinical Medicine, Discipline of Pediatrics and Child Health, UNSW Medicine & Health, University of New South Wales, Sydney, Australia.
³ Department of Endocrinology, Sydney Children's Hospital Randwick, Sydney Children's Hospitals Network, Sydney, Australia.
⁴ Nuclear Medicine, The Children's Hospital at Westmead, Sydney, New South Wales, Australia.
⁵ University of Colorado Anschutz Medical Center, Aurora, Colorado, USA.
⁶ Children's Hospital Colorado, Aurora, Colorado, USA.
⁷ CF Center, Fondazione IRCCS Ca' Granda, Ospedale Maggiore Policlinico, University of Milan, Milan, Italy.
⁸ Department of Respiratory and Sleep Medicine, Queensland Children's Hospital, South Brisbane, Australia.
⁹ Faculty of Medicine, The University of Queensland, Brisbane, Australia.
¹⁰ Israel Deaconess Medical Center, Boston, Massachusetts, USA.
¹¹ Harvard Medical School, Boston, Massachusetts, USA.
¹² Division of Gastroenterology, Hepatology and Nutrition, Dep of Pediatrics, University of Toronto, Toronto, Ontario, Canada.
¹³ Research Institute, the Hospital for Sick Children, Toronto, Ontario, Canada.
¹⁴ Endocrinology and Diabetes, St Vincent's Hospital Sydney, Darlinghurst, Australia.
¹⁵ School of Clinical Medicine, The University of New South Wales, Sydney, Australia.
¹⁶ Garvan Institute, Sydney, Australia.
¹⁷ Baylor College of Medicine, Houston, Texas, USA.
¹⁸ Division of Pediatric Gastroenterology, Hepatology and Nutrition, Texas Children's Hospital, Houston, Texas, USA.
¹⁹ University of Minnesota, Minneapolis, Minnesota, USA.
²⁰ Department of Respiratory Medicine, Sydney Children's Hospital, Randwick, Australia.
²¹ Diabetes Research Center, Massachusetts General Hospital, Boston, Massachusetts, USA.
²² Queensland Lung Transplant Service, The Prince Charles Hospital, Brisbane, Australia.
²³ St Michael's Hospital, Toronto, Ontario, Canada.
²⁴ University of Toronto, Toronto, Ontario, Canada.
²⁵ Lung Transplant Service, Alfred Health, Melbourne, Australia.
²⁶ Central Clinical School, Monash University, Melbourne, Australia.
²⁷ Child Health Research Center (CHRC), The University of Queensland, Brisbane, Australia.
²⁸ Department of Anatomy and Cell Biology, Roy J. and Lucille A. Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
²⁹ Department of Nutrition and Dietetics, Sydney Children's Hospital, Sydney, Australia.
³⁰ Nutrition Department, Alfred Health, Melbourne, Victoria, Australia.
³¹ Department of Gastroenterology, Hepatology and Liver Transplant, Queensland Children's Hospital, Brisbane, Australia.
³² CF Endocrine Service Adult Cystic Fibrosis Center (ACFC) The Prince Charles Hospital, Australia.
³³ Primary Children's Hospital, Salt Lake City, Utah, USA.
³⁴ Department of Medicine, University of Minnesota, Minneapolis, Minnesota, USA.
³⁵ Digestive Health Institute, Children's Hospital Colorado and Section of Pediatric Gastroenterology, Hepatology and Nutrition, University of Colorado School of Medicine, Aurora, Colorado, USA.
³⁶ Department of Gastroenterology, Royal Children's Hospital, University of Melbourne, Melbourne, Australia.
³⁷ Hepatic Fibrosis Group, QIMR Berghofer Medical Research Institute, Herston, Australia.
³⁸ Division of Pediatric Gastroenterology, Hepatology, and Nutrition, Stanford University, Palo Alto, California, USA.
³⁹ Pediatric Gastroenterology, Hadassah University Hospitals, Jerusalem, Israel.

PMID: 39903130
DOI: 10.1002/ppul.26970

Abstract

Background: Cystic fibrosis (CF) is a life-shortening multisystem genetic disease. Although progressive pulmonary disease is the predominant cause of morbidity and mortality, improvements in treatment for CF-related lung disease, with associated increase in longevity, have increased the prevalence of extrapulmonary manifestations¹.

Methods: To discuss these issues, a multidisciplinary meeting of international leaders and experts in the field was convened in November 2021 at the Shaping Initiatives and Future Trends Symposium with the goal of highlighting shifting management paradigms in CF. The main topics covered were: (1) nutrition and obesity, (2) exocrine pancreas, (3) CF-related diabetes, (4) CF liver disease, (5) CF-related bone disease, and (6) post-lung transplant care. This document summarizes the proceedings, highlighting the key priorities and important research questions that were discussed.

Results: Improved life expectancy, the advent of cystic fibrosis transmembrane conductance regulator modulators, and the increasing appreciation of the heterogeneity or spectrum of disease are leading to a shift in management for patients with cystic fibrosis. Care should be individualized to ensure that increased longevity is accompanied by improved extra-pulmonary care and reduced morbidity.

Keywords: cystic fibrosis; cystic fibrosis bone disease; cystic fibrosis related diabetes; cystic fibrosis related liver disease; exocrine pancreatic disease; extra‐pulmonary complications cystic fibrosis; lung transplant; nutrition.

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References

REFERENCES

1. Chin M, Brennan AL, Bell SC. Emerging nonpulmonary complications for adults with cystic fibrosis. Chest. 2022;161(5):1211‐1224.
1. Bell SC, Mall MA, Gutierrez H, et al. The future of cystic fibrosis care: a global perspective. Lancet Respir Med. 2020;8(1):65‐124.
1. Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr. 2013;162(3):530‐535.e1.
1. Pencharz PB, Durie PR. Pathogenesis of malnutrition in cystic fibrosis, and its treatment. Clin Nutr. 2000;19(6):387‐394.
1. Corey M, McLaughlin FJ, Williams M, Levison H. A comparison of survival, growth, and pulmonary function in patients with cystic fibrosis in Boston and Toronto. J Clin Epidemiol. 1988;41(6):583‐591.

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The SHIFT symposium was supported by Vertex Pharmaceuticals, with no involvement in manuscript

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SHIFTing goals in cystic fibrosis-managing extrapulmonary disease in the era of CFTR modulator therapy; Proceedings of the International Shaping Initiatives and Future Trends (SHIFT) Symposium

Collaborators

Affiliations

SHIFTing goals in cystic fibrosis-managing extrapulmonary disease in the era of CFTR modulator therapy; Proceedings of the International Shaping Initiatives and Future Trends (SHIFT) Symposium

Authors

Collaborators

Affiliations

Abstract

References

REFERENCES

Publication types

MeSH terms

Substances

Grants and funding

LinkOut - more resources

Full Text Sources

Medical