Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor

Flume, Patrick A, Wainwright, Claire E, Elizabeth Tullis, D, Rodriguez, Sally, Niknian, Minoo, Higgins, Mark, Davies, Jane C and Wagener, Jeffrey S (2018) Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor. Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society, 17 1: 83-88. doi:10.1016/j.jcf.2017.06.002


Author Flume, Patrick A
Wainwright, Claire E
Elizabeth Tullis, D
Rodriguez, Sally
Niknian, Minoo
Higgins, Mark
Davies, Jane C
Wagener, Jeffrey S
Title Recovery of lung function following a pulmonary exacerbation in patients with cystic fibrosis and the G551D-CFTR mutation treated with ivacaftor
Journal name Journal of cystic fibrosis : official journal of the European Cystic Fibrosis Society   Check publisher's open access policy
ISSN 1873-5010
Publication date 2018-01-01
Sub-type Article (original research)
DOI 10.1016/j.jcf.2017.06.002
Open Access Status Not yet assessed
Volume 17
Issue 1
Start page 83
End page 88
Total pages 6
Abstract Pulmonary exacerbations (PEx) are associated with acute loss of lung function that is often not recovered after treatment. We investigated lung function recovery following PEx for ivacaftor- and placebo-treated subjects.

Short- and long-term pulmonary function recovery data after PEx were summarized from a placebo-controlled trial in 161 cystic fibrosis patients≥12years old with the G551D-CFTR mutation (NCT00909532). Short-term recovery was measured 2 to 8weeks after treatment, and long-term recovery was determined at the end-of-study, both compared with baseline measured just prior to the PEx.

Fewer patients receiving ivacaftor experienced a PEx than patients receiving placebo (33.7% vs. 56.4%; P=0.004) and had a lower adjusted incidence rate of PEx (0.589 vs. 1.382; P<0.001). The proportion of PEx followed by full short-term recovery of percent predicted forced expiratory volume in 1s was similar (ivacaftor vs. placebo, 57.1% vs. 53.7), as was the proportion of patients having long-term recovery (46.4% vs. 47.7%).

Ivacaftor treatment reduces the frequency of PEx but does not improve on the rate of complete lung function recovery after PEx when compared with placebo.
Keyword Cystic fibrosis
Cystic fibrosis transmembrane conductance regulator
Ivacaftor
Pulmonary exacerbations
Pulmonary function
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collection: Pubmed Import
 
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Created: Wed, 10 Jan 2018, 16:30:48 EST