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

William G. Flight; Jonathan Shaw; Susan Johnson; A. Kevin Webb; Andrew M. Jones; Andrew M. Bentley; Rowland J. Bright-Thomas

doi:10.1016/j.jcf.2011.11.006

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

William G. Flight^*, Jonathan Shaw, Susan Johnson, A. Kevin Webb, Andrew M. Jones, Andrew M. Bentley, Rowland J. Bright-Thomas

^*Corresponding author for this work

Manchester Adult Cystic Fibrosis Centre

Research output: Contribution to journal › Article › peer-review

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 16months (range 2-90). Mean FEV ₁ fell by 212ml over the year before NIV but increased by 18ml 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.

Original language	English
Pages (from-to)	187-192
Number of pages	6
Journal	Journal of Cystic Fibrosis
Volume	11
Issue number	3
DOIs	https://doi.org/10.1016/j.jcf.2011.11.006 https://doi.org/10.1016/j.jcf.2011.11.006 https://doi.org/10.1016/j.jcf.2011.11.006
Publication status	Published - 1 May 2012

Keywords

Cystic fibrosis
Lung transplantation
Non-invasive ventilation
Respiratory failure

UN SDGs

This output contributes to the following UN Sustainable Development Goals (SDGs)

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title = "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 16months (range 2-90). Mean FEV 1 fell by 212ml over the year before NIV but increased by 18ml 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.",

keywords = "Cystic fibrosis, Lung transplantation, Non-invasive ventilation, Respiratory failure",

author = "Flight, {William G.} and Jonathan Shaw and Susan Johnson and Webb, {A. Kevin} and Jones, {Andrew M.} and Bentley, {Andrew M.} and Bright-Thomas, {Rowland J.}",

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AU - Flight, William G.

AU - Shaw, Jonathan

AU - Johnson, Susan

AU - Webb, A. Kevin

AU - Jones, Andrew M.

AU - Bentley, Andrew M.

AU - Bright-Thomas, Rowland J.

PY - 2012/5/1

Y1 - 2012/5/1

N2 - 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 16months (range 2-90). Mean FEV 1 fell by 212ml over the year before NIV but increased by 18ml 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.

AB - 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 16months (range 2-90). Mean FEV 1 fell by 212ml over the year before NIV but increased by 18ml 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.

KW - Cystic fibrosis

KW - Lung transplantation

KW - Non-invasive ventilation

KW - Respiratory failure

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JO - Journal of Cystic Fibrosis

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ER -

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

Abstract

Keywords

UN SDGs

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