title: Changing demographics and outcomes of lung transplantation recipients with cystic fibrosis.
abstract: Cystic fibrosis (CF) is one of the most common diagnoses in adult and pediatric patients undergoing lung transplantation (LTx). A changing pattern of indications for LTx among patients with CF has been noted. This study analyzes the prevalence and characteristics of patients with CF who underwent LTx in the current era.
  
  
  
  A retrospective analysis was performed using data from the United Network of Organ Sharing database of all LTx performed from 1999 to 2013 (N = 20,345). Sub-analyses focused on children (<18 years old). Patients with CF who underwent LTx were assigned to early (1999-2003), mid (2004-2008), and current (2009-2013) eras based on the date of the procedure as well as before and after implementation of the new lung allocation score system in 2005.
  
  
  
  CF was the indication for LTx in 14% (2,877) of who patients underwent LTx, a decrease from >17% in the early era to <13% in the current era (p < 0.001). In the pediatric cohort, CF was the indication for LTx in 383 (53%) patients, a proportion that also decreased across eras (early, 60%; mid, 53%; current, 47%; p = 0.009). The mean age of patients with CF undergoing LTx increased across the eras (early, 28 years ± 10; mid. 28 years ± 10; current, 30 years ± 11; p < 0.001). Pre-transplant ventilator use and incidence of pan-resistant infections also increased (p < 0.001), whereas pre-transplant forced expiratory volume in 1 second and waitlist times decreased (p < 0.001) in patients with CF. Graft survival across the eras remained similar (p > 0.05) at 5.1 years overall.
  
  
  
  The proportion of LTx performed for CF has significantly decreased over time, a trend especially pronounced in pediatric patients. The change in pre-transplant characteristics across eras indicates a trend to perform LTx in more clinically ill and older patients with CF. The overall post-LTx survival has not changed.
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio. Electronic address: naritaku@gmail.com.
authors.forename: Naritaka
authors.initials: N
authors.lastname: Kimura
authors.affiliation: Department of Family Medicine, University of Oklahoma School of Community Medicine, Tulsa, Oklahoma.
authors.forename: Muhammad S
authors.initials: MS
authors.lastname: Khan
authors.affiliation: Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: Marc
authors.initials: M
authors.lastname: Schecter
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: Raheel
authors.initials: R
authors.lastname: Rizwan
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: Roosevelt
authors.initials: R
authors.lastname: Bryant
authors.affiliation: Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: Erin
authors.initials: E
authors.lastname: Wells
authors.affiliation: Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: Christopher
authors.initials: C
authors.lastname: Towe
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: Farhan
authors.initials: F
authors.lastname: Zafar
authors.affiliation: Division of Pediatric Cardiothoracic Surgery, The Heart Institute, Cincinnati Children's Hospital Medical Center, University of Cincinnati, Cincinnati, Ohio.
authors.forename: David L S
authors.initials: DL
authors.lastname: Morales
mesh_terms: D000328:Adult; D003550:Cystic Fibrosis; D003710:Demography; D005541:Forced Expiratory Volume; D006801:Humans; D016040:Lung Transplantation; D012189:Retrospective Studies
keywords: cystic fibrosis; demographics; lung allocation score system; lung transplantation; mechanical ventilation; transplant candidacy