A contemporary survival analysis of individuals with cystic fibrosis: a cohort study.
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.
Adult CF Program, St Michael's Hospital, Toronto, ON, Canada Dept of Medicine, University of Toronto, Toronto, ON, Canada.
Anne L
AL
Stephenson
Keenan Research Centre in the Li Ka Shing Knowledge Institute of St Michael's Hospital, Toronto, ON, Canada.
Melissa
M
Tom
IRCM and CHUM Université de Montréal, Montreal, QC, Canada.
Yves
Y
Berthiaume
Dept of Medicine, University of Toronto, Toronto, ON, Canada Toronto Lung Transplant Program, University Health Network, Toronto, ON, Canada.
Lianne G
LG
Singer
The Ottawa Hospital Research Institute, University of Ottawa, Ottawa, ON, Canada.
Shawn D
SD
Aaron
The Ottawa Hospital Research Institute, University of Ottawa, Ottawa, ON, Canada Desautels Faculty of Management, McGill University, Montreal, QC, Canada.
G A
GA
Whitmore
Research Institute, The Hospital for Sick Children, Toronto, ON, Canada Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada.
Sanja
S
Stanojevic
D000328:Adult; D000367:Age Factors; D002170:Canada / Q000453:epidemiology; D002648:Child; D003550:Cystic Fibrosis / Q000150:complications / Q000401:mortality / Q000503:physiopathology; D003920:Diabetes Mellitus / Q000209:etiology / Q000401:mortality; D018450:Disease Progression; D005260:Female; D006801:Humans; D015994:Incidence; D007223:Infant; D008017:Life Expectancy; D008297:Male; D044342:Malnutrition / Q000209:etiology / Q000401:mortality; D008875:Middle Aged; D016016:Proportional Hazards Models; D012042:Registries; D012141:Respiratory Tract Infections / Q000209:etiology / Q000401:mortality; D012307:Risk Factors; D012737:Sex Factors