Survival in Cystic Fibrosis: Trends, Clinical Factors, and Prediction Models

Cystic fibrosis (CF) is an autosomal recessive genetic disease that results in multiple medical complications, and ultimately decreased survival of affected patients. Due to multiple advances in early diagnosis, nutrition, and aggressive treatment of complications, survival of these patients has improved dramatically in the past 40 years. However, despite improved care, patients still die at a relatively young age, most commonly due to respiratory failure. In order to extend survival, the sickest patients with CF are considered for lung transplant. An accurate understanding of a CF patient's expected survival and health trajectory is critical for appropriate patient selection and timing of transplantation. This review discusses how survival in CF has changed over the past four decades, the clinical factors that are associated with survival, as well as the use of models to predict survival and optimal timing of lung transplant in patients with CF.