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.
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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.
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