title: What is the role of noninvasive ventilation in cystic fibrosis?
abstract: OBJECTIVE
  The use of noninvasive ventilatory support in patients with cystic fibrosis (CF) has increased exponentially over the past 2 decades. This review examines the current knowledge and considers potential future directions for use of noninvasive ventilation in CF patients.
  
  
  RESULTS
  Noninvasive ventilation was originally reported as a bridge to transplantation in CF patients with severe respiratory failure but is now used as a long-term treatment modality for patients with respiratory failure independent of transplant status. In 2013 to 2014, over 400 publications on noninvasive ventilation demonstrate its increasing clinical application, however only seven reference CF. Recent technological advances and potential benefits to CF patients are considered.
  
  
  CONCLUSIONS
  The role of noninvasive ventilation in CF patients in chronic respiratory failure is established, but future prospective studies are needed to determine further indications and optimal timing of this intervention. Developments in both ventilator and interface design may enhance the efficacy of ventilation in CF patients but require careful individualized assessment and regular review. The implications on treatment burden and quality of life in CF also need to be studied.
authors.affiliation: aManchester Adult Cystic Fibrosis Centre, North West Lung Centre, University Hospital of South Manchester bRespiratory Research Group, Institute of Inflammation and Repair, University of Manchester, Manchester, UK.
authors.forename: Rowland J
authors.initials: RJ
authors.lastname: Bright-Thomas
authors.forename: Susan C
authors.initials: SC
authors.lastname: Johnson
mesh_terms: D003550:Cystic Fibrosis / Q000150:complications / Q000503:physiopathology / Q000628:therapy*; D006801:Humans; D016040:Lung Transplantation; D063087:Noninvasive Ventilation; D018579:Patient Selection; D011446:Prospective Studies; D011788:Quality of Life; D012131:Respiratory Insufficiency / Q000209:etiology / Q000503:physiopathology / Q000628:therapy*; D012132:Respiratory Muscles / Q000503:physiopathology*; D016896:Treatment Outcome; D015919:Ventilators, Negative-Pressure