A contemporary survival analysis of individuals with cystic fibrosis: a cohort study

European Respiratory Journal - Tập 45 Số 3 - Trang 670-679 - 2015
Anne L. Stephenson1,2, Melissa Tom3, Yves Berthiaume4, L.G. Singer2,5, Shawn D. Aaron6, G. À. Whitmore7,6, Sanja Stanojevic8,9
1Adult CF Program, St Michael's Hospital, Toronto, ON, Canada
2Dept. of Medicine, University of Toronto, Toronto, ON, Canada
3Keenan Research Centre in the Li Ka Shing Knowledge Institute of St. Michael's Hospital, Toronto, ON, Canada
4IRCM and CHUM Université de Montréal, Montreal, QC, Canada
5Toronto Lung Transplant Program, University Health Network, Toronto, ON, Canada
6The Ottawa Hospital Research Institute, University of Ottawa, Ottawa, ON, Canada
7Desautels Faculty of Management, McGill University, Montreal, QC, Canada
8Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada
9Research Institute, The Hospital for Sick Children, Toronto, ON, Canada

Tóm tắt

Previously established predictors of survival may no longer apply in the current era of cystic fibrosis (CF) care. Our objective was to identify risk factors associated with survival in a contemporary CF population.We used the Canadian CF Registry, a population-based cohort, to calculate median age of survival and summarise patient characteristics from 1990 to 2012. Clinical, demographic and geographical factors, and survival were estimated for a contemporary cohort (2000–2012) using Cox proportional hazards models.There were 5787 individuals in the registry between 1990 and 2012. Median survival age increased from 31.9 years (95% CI 28.3–35.2 years) in 1990 to 49.7 years (95% CI 46.1–52.2 years) in the most current 5-year window ending in 2012. Median forced expiratory volume in 1 s improved (p=0.04) and fewer subjects were malnourished (p<0.001) over time. Malnourished patients (hazard ratio (HR) 2.1, 95% CI 1.6–2.8), those with multiple exacerbations (HR 4.5, 95% CI 3.2–6.4) and women with CF-related diabetes (HR 1.8, 95% CI 1.2–2.7) were at increased risk of death.Life expectancy in Canadians with CF is increasing. Modifiable risk factors such as malnutrition and pulmonary exacerbations are associated with an increased risk of death. The sex gap in CF survival may be explained by an increased hazard for death in women with CF-related diabetes.

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European Respiratory Journal

Tập 45 Số 3

670-679

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