Reduced survival in adult cystic fibrosis despite attenuated lung function decline

Background: There is limited data on disease progression and survival in adult diagnosis cystic fibrosis (CF). This study evaluates change of lung function over time and rates of death/lung transplant in adult diagnosis CF. Methods: The CF Foundation Patient Registry was reviewed for patients diagnosed 1993-2003. Rate of FEV1 decline was calculated up to 2010 for age-groups 6-11, 12-17, and 18 and above. Kaplan Meier method was used for 10 and 15 year survival rate calculations for patients diagnosed as adults. Cox Proportional hazards models using predictors affecting disease progression and survival without transplant were run. Results: Between 1993 and 2003, 11,884 patients were diagnosed with CF, of which 2848 were ages 6 and older. Annual rate of change of FEV1% predicted over 5 years differed by diagnosis age group: 1.42% per year for ages 6-11, 2.04% for ages 12-17 and 1.13% for ages 18-65 (p < 0.0001). Pseudomonas aeruginosa infection was associated with faster rates of lung function decline in all age groups. Survival without transplant for CF patients diagnosed at 18 years were 76% and 65% by 10 and 15 years, respectively. Of adults with FEV1 of >70% predicted at diagnosis, 95% were alive without transplant at 10 years, whereas of those with FEV1 < 40% predicted at diagnosis, 31% were alive without transplant at 10 years. Conclusions: Lung function declines at a slower rate in adult diagnosis CF. However, particularly in those with low lung function at diagnosis, rates of death or transplant in adult diagnosis CF after 10 and 15 years is not negligible. (C) 2016 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.