• Christopher H Goss, Jenna Sykes, Sanja Stanojevic, Bruce Marshall, Kristofer Petren, Josh Ostrenga, Aliza Fink, Alexander Elbert, Bradley S Quon, and Anne L Stephenson.
• 1 Division of Pulmonary and Critical Care Medicine, Department of Medicine and Pediatrics, University of Washington Medical Center, Seattle, Washington.
• Am. J. Respir. Crit. Care Med. 2018 Mar 15; 197 (6): 768775768-775.

RationaleA 10-year gap in the median age of survival for patients with cystic fibrosis (CF) was reported between patients living in Canada compared with patients living in the United States.ObjectivesBecause both malnutrition and poor lung function are associated with an increased risk of mortality in CF, we investigated the temporal and longitudinal trends in lung function and nutrition between Canada and the United States.MethodsThis cohort study used Canadian CF Registry and U.S. CF Foundation Patient Registry data from 1990 to 2013. A unified dataset was created to harmonize the variables collected within the two registries for the purpose of comparing outcomes between the two countries.Measurements And Main ResultsWe conducted three analyses: survival differences by birth cohort; population trends for FEV1 and body mass index (BMI) over time; and individual patient FEV1 and BMI trajectories. The study included a total of 37,772 patients in the United States and 5,149 patients in Canada. Patients with CF experienced significant improvements in nutritional status and lung function in both Canada and the United States during the study. In addition, the survival gap between the two countries is narrowing within younger birth cohorts. The improvements for the patients within the United States were most prominent in the BMI trajectories, where patients born after 1990 in the United States have higher BMI that has persisted over time.ConclusionsThe reasons for the observed improvements, and catch-up in the United States, are likely multifactorial and include the introduction of high-fat, high-calorie diets; introduction of newborn screening; and/or improved access to care for CF children in the United States.

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