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Effect of ivacaftor on objective and subjective measures of cough in patients with cystic fibrosis

Faruqi, Shoaib; Shiferaw, Dejene; Morice, Alyn H.


Shoaib Faruqi

Dejene Shiferaw


© Faruqi et al. Background and Objectives: Cough is a major symptom in cystic fibrosis. Ivacaftor is a novel drug which targets the G551D mutation and has been demonstrated to improve lung function and weight in the long term. It also improves symptoms of extra-oesophageal reflux. We wanted to evaluate the effect of ivacaftor on cough in cystic fibrosis. Methods: In two patients with cystic fibrosis the Hull Airway Reflux Questionnaire (HARQ) was completed and objective cough counts were measured prior to and within 4 weeks after initiation of treatment with ivacaftor. Spirometry was also undertaken and weight checked at these time frames. Results: In the first patient the HARQ score decreased from 29 to 11 and objective cough counts from 29 to 9 cough events per hour. Similarly in the second patient the HARQ score decreased from 13 to 9 and objective cough count from 76 to 5 cough events per hour. There was no significant change in spirometric parameters or weight. Conclusion: We have observed early subjective and objective improvement in cough measures on treatment with ivacaftor. We suggest that this improvement could be attributed to improvement of gastro-intestinal function and that cough metrics could be used as early and accurate end points of drug efficacy.


Faruqi, S., Shiferaw, D., & Morice, A. H. (2016). Effect of ivacaftor on objective and subjective measures of cough in patients with cystic fibrosis. Open Respiratory Medicine Journal, 10, 105-108.

Journal Article Type Article
Acceptance Date Nov 14, 2016
Online Publication Date Dec 30, 2016
Publication Date 2016
Deposit Date May 12, 2022
Publicly Available Date May 16, 2022
Journal Open Respiratory Medicine Journal
Electronic ISSN 1874-3064
Publisher Bentham Open
Peer Reviewed Peer Reviewed
Volume 10
Pages 105-108
Keywords Cystic fibrosis; Cough; Ivacaftor; Gastro-oesophageal reflux; G551D mutation; Lung function
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Copyright Statement
© Faruqi et al.; Licensee Bentham Open
This is an open access article licensed under the terms of the Creative Commons Attribution-Non-Commercial 4.0 International Public License
(CC BY-NC 4.0) (, which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.

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