SURVIVCF: Survival and Transplantation in Canadians with Cystic Fibrosis

Canada boasts the highest median survival age for individuals living with cystic fibrosis (CF) in the world. Which factors explain the improvements seen in survival over time in Canada and which groups are at risk for early death has not been established in the current era of CF care. Two pivotal Canadian studies, published over 15 years ago, provided clinically useful information on predictors of survival in CF; however, treatment, complications and prognosis have dramatically changed over the past two decades. Despite advances in care, progressive lung disease continues to be the primary cause of death in CF and ultimately lung transplantation is offered to those with end-stage lung disease. Accurately predicting life expectancy in CF is critical so that healthcare providers can alter modifiable risk factors, intensify medical therapy, consider new therapies, or optimize timing for lung transplantation referral and listing. We propose a population-based cohort study using linked national CF registry data to systematically identify demographic, clinical, and geographic factors associated with survival in a contemporary CF sample and to identify the best combination of factors which accurately predict survival. Finallly, we will evaluate the impact of lung transplantation on survival in the Canadian CF population and identify the groups most likely to benefit.