title: Reduced survival in adult cystic fibrosis despite attenuated lung function decline.
abstract: 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.
  
  
  
  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 15year survival rate calculations for patients diagnosed as adults. Cox Proportional hazards models using predictors affecting disease progression and survival without transplant were run.
  
  
  
  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 5years 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 ≥18years were 76% and 65% by 10 and 15years, respectively. Of adults with FEV1 of >70% predicted at diagnosis, 95% were alive without transplant at 10years, whereas of those with FEV1<40% predicted at diagnosis, 31% were alive without transplant at 10years.
  
  
  
  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 15years is not negligible.
authors: Department of Medicine, Columbia University Medical Center, 622 West 168th Street, Presbyterian Hospital Building, 8th Floor, New York, NY 10032, United States. Electronic address: ck2132@cumc.columbia.edu.
authors: Claire
authors: C
authors: Keating
authors: Department of Medicine, Columbia University Medical Center, 622 West 168th Street, Presbyterian Hospital Building, 8th Floor, New York, NY 10032, United States.
authors: Armeen D
authors: AD
authors: Poor
authors: Department of Biostatistics, Mailman School of Public Health, Columbia University, 722 West 168th Street, New York, NY 10032, United States.
authors: Xinhua
authors: X
authors: Liu
authors: Department of Biostatistics, Mailman School of Public Health, Columbia University, 722 West 168th Street, New York, NY 10032, United States.
authors: Codruta
authors: C
authors: Chiuzan
authors: Department of Biostatistics, Mailman School of Public Health, Columbia University, 722 West 168th Street, New York, NY 10032, United States.
authors: Daniel
authors: D
authors: Backenroth
authors: Department of Biostatistics, Mailman School of Public Health, Columbia University, 722 West 168th Street, New York, NY 10032, United States.
authors: Yuan
authors: Y
authors: Zhang
authors: Department of Medicine, Columbia University Medical Center, 622 West 168th Street, Presbyterian Hospital Building, 8th Floor, New York, NY 10032, United States.
authors: Emily
authors: E
authors: DiMango
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis / Q000175:diagnosis / Q000382:microbiology / Q000401:mortality / Q000503:physiopathology; D018450:Disease Progression; D005260:Female; D005541:Forced Expiratory Volume; D006801:Humans; D053208:Kaplan-Meier Estimate; D016040:Lung Transplantation / Q000706:statistics & numerical data*; D008297:Male; D011379:Prognosis; D016016:Proportional Hazards Models; D011552:Pseudomonas Infections / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D012141:Respiratory Tract Infections / Q000175:diagnosis / Q000453:epidemiology / Q000503:physiopathology; D015996:Survival Rate; D014481:United States / Q000453:epidemiology
keywords: Adult diagnosis cystic fibrosis; Cystic fibrosis; Lung function; Survival