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Cystic fibrosis
Original research
Beyond borders: cystic fibrosis survival between Australia, Canada, France and New Zealand
  1. Adèle Coriati1,
  2. Xiayi Ma1,
  3. Jenna Sykes1,
  4. Sanja Stanojevic2,
  5. Rasa Ruseckaite3,
  6. Lydie Lemonnier4,
  7. Clémence Dehillotte4,
  8. Jan Tate5,
  9. Catherine Ann Byrnes6,7,
  10. Scott C Bell8,9,
  11. Pierre Regis Burgel10,11,12,
  12. Anne L Stephenson1,13,14
  1. 1 Department of Respirology, St Michael's Hospital, Toronto, Ontario, Canada
  2. 2 Community Health and Epidemiology, Dalhousie University, Halifax, Nova Scotia, Canada
  3. 3 Public Health and Preventative Medicine, Monash University, Clayton, Victoria, Australia
  4. 4 Association Vaincre la Mucoviscidose, Paris, Île-de-France, France
  5. 5 Paediatric Department, Starship Children's Health, Auckland, New Zealand
  6. 6 Paediatrics, Child and Youth Health, The University of Auckland School of Medicine, Auckland, New Zealand
  7. 7 Paediatric Respiratory Department, Starship Children's Health, Auckland, New Zealand
  8. 8 Department of Thoracic Medicine, The Prince Charles Hospital, Chermside, Queensland, Australia
  9. 9 Children’s Health Research Centre, The University of Queensland Faculty of Medicine and Biomedical Sciences, Herston, Queensland, Australia
  10. 10 Assistance Publique Hôpitaux de Paris, Department of Respiratory Medicine and French Cystic Fibrosis Reference Center, Hopital Cochin Pneumologie, Paris, Île-de-France, France
  11. 11 Institut Cochin, Université de Paris, Paris, Île-de-France, France
  12. 12 European Reference Network Respiratory Diseases, Frankfurt, Germany
  13. 13 St Michael's Hospital Li Ka Shing Knowledge Institute, Toronto, Ontario, Canada
  14. 14 Department of Medicine, University of Toronto, Toronto, Ontario, Canada
  1. Correspondence to Dr Anne L Stephenson, Dept. of Respirology, St Michael's Hospital, Toronto, Canada; Anne.Stephenson{at}unityhealth.to

Abstract

Background Life expectancy for people with cystic fibrosis (CF) varies considerably both within and between countries. The objective of this study was to compare survival among countries with single-payer healthcare systems while accounting for markers of disease severity.

Methods This cohort study used data from established national CF registries in Australia, Canada, France and New Zealand from 2015 to 2019. Median age of survival for each of the four countries was estimated using the Kaplan-Meier method. A Cox proportional hazards model was used to compare risk of death between Canada, France and Australia after adjusting for prognostic factors. Due to low number of deaths, New Zealand was not included in final adjusted models.

Results Between 2015 and 2019, a total of 14 842 people (3537 Australia, 4434 Canada, 6411 France and 460 New Zealand) were included. The median age of survival was highest in France 65.9 years (95% CI: 59.8 to 76.0) versus 53.3 years (95% CI: 48.9 to 59.8) for Australia, 55.4 years (95% CI: 51.3 to 59.2) for Canada and 54.8 years (95% CI: 40.7 to not available) for New Zealand. After adjusting for individual-level factors, the risk of death was significantly higher in Canada (HR 1.85, 95% CI: 1.48 to 2.32; p<0.001) and Australia (HR 2.08, 95% CI: 1.64 to 2.64; p<0.001) versus France.

Interpretation We observed significantly higher survival in France compared with countries with single-payer healthcare systems. The median age of survival in France exceeded 60 years of age despite having the highest proportion of underweight patients which may be due to differences in availability of transplant.

  • cystic fibrosis

Data availability statement

Data sharing not applicable as no datasets generated and/or analysed for this study.

https://doi.org/10.1136/thorax-2022-219086

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    Data availability statement

    Data sharing not applicable as no datasets generated and/or analysed for this study.

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    Footnotes

    • Twitter @CysFibMD

    • Contributors ALS, SS, PRB, SCB contributed to study concept and design. ALS, RR, LL, CD, JT, CAB contributed to acquisition of data. ALS, SS. PRB, AC, XM, JS, RR, LL, CD, JT, CAB, SCB contributed to analysis and interpretation of data. AC, ALS contributed to drafting of the manuscript. ALS, SS. PRB, AC, XM, JS, RR, LL, CD, JT, CAB, SCB contributed to critical revision of the manuscript for important intellectual content. XM, JS, SS, AC contributed to statistical analysis. ALS, SS, PRB, SCB obtained funding. ALS contributed to study supervision. ALS is the guarantor of the study, therefore accepts full responsibility for the work and/or the conduct of the study, had access to the data, and controlled the decision to publish.

    • Funding This study was funded by Cystic Fibrosis Canada (Grant #609767). AC was supported by a Post-Doctoral Fellowship from the Canadian Lung Association.

    • Disclaimer The study sponsor did not participate in the design and conduct of the study; collection, management, analysis and interpretation of the data; or preparation, review or approval of the manuscript; decision to submit the paper for publication.

    • Competing interests SS is a co-applicant of the CF Canada grant for this study, and received a grant from Vertex pharmaceuticals and the European Respiratory Society, unrelated to this study. SS also received consulting fees from Chiesi pharmaceuticals, unrelated to this study. CAB received support from Cystic Fibrosis New Zealand (for this study) and grants from HRC, Flu Lab and APP, unrelated to this study. CAB was also a trustee for the Bronchiectasis Foundation and chair of the clinical advisory panel for cystic fibrosis as well as the PORT CF NZ registry. PRB received grants from GSK, Vertex and Boehringer Ingelheim, unrelated to this study. PRB has also received consulting fees from Astra Zeneca, Chiesi, Insmed and Vertex as well as payment from Astra Zeneca, Chiesi, GSK, Novartis, Pfizer, Vertex and Zambon, unrelated to this study. PRB also received support for attending meetings and/or travels from Astra Zeneca and Zambon, unrelated to this study. SCB is a co-applicant of the CF Canada grant for this study, in addition to receiving grants from the CF Foundation, NHMRC and MRFF, unrelated to this study. SCB was chair of the Data Safety monitoring Board (DSMB) of the Phase IIA/IIB RCT Ataxia telangiectasia project and also participated in the DSMB of the RCT Inhaled hypertonic saline in people with bronchiectasis project, unrelated to this study. SCB was also a board member of Health Translation Qld Board, Gallipoli Medical Research Foundation and the European CF Society, unrelated to this study. ALS has received a grant from CF Canada for this study, as well as the CF Foundation and the Canadian institute for Health research, unrelated to this study. ALS received consulting fees from CF Canada (or her role as the Medical Director of the Canadian CF Registry) and payment form Vertex Pharmaceuticals, unrelated to this study. ALS has also participated in the DSMB of Vertex pharmaceuticals and Horizon Pharma, unrelated to this study.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.

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