Long-term non-invasive ventilation in cystic fibrosis -- experience over two decades.

Abstract

BACKGROUND:

Non-invasive ventilation (NIV) is accepted as a bridge to lung transplantation in cystic fibrosis (CF) but there is little evidence to support its use outside this setting.

METHODS:

We reviewed the records of all patients with CF who received domiciliary NIV at our centre between 1991 and 2010.

RESULTS:

Of 47 patients studied, 36% underwent lung transplantation, 28% died without transplantation and 30% remain alive on NIV. Median duration of NIV was 16 months (range 2-90). Mean FEV(1) fell by 212 ml over the year before NIV but increased by 18 ml in the following year (p<0.01). Individual response to NIV was associated with lower baseline and more rapid decline in FEV(1). From 1991 to 2000, 70% underwent lung transplantation; from 2001 to 2010 only 27% were transplanted.

CONCLUSIONS:

NIV may slow or reverse the decline in lung function in advanced CF. NIV is increasingly used beyond a bridge to transplantation at our centre.

• Authors: William G Flight, Jonathan Shaw, Susan Johnson, A Kevin Webb, Andrew M Jones, Andrew M Bentley, Rowland J Bright-Thomas
• Journal: Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society (J Cyst Fibros) Vol. 11 Issue 3 Pg. 187-92 (May 2012) ISSN: 1873-5010 [Electronic] Netherlands
• PMID: 22177738 (Publication Type: Comparative Study, Journal Article)
• Copyright: Copyright © 2011 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

Topics

• Adolescent
• Adult
• Cystic Fibrosis (complications, mortality, therapy)
• Female
• Follow-Up Studies
• Forced Expiratory Volume
• Humans
• Lung Transplantation
• Male
• Middle Aged
• Positive-Pressure Respiration (methods)
• Preoperative Care (methods)
• Prognosis
• Respiratory Insufficiency (etiology, physiopathology, therapy)
• Retrospective Studies
• Survival Rate
• Time Factors
• United Kingdom (epidemiology)
• Young Adult