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

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

Abstract

© 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.

Citation

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. https://doi.org/10.2174/1874306401610010105

• 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
• DOI: https://doi.org/10.2174/1874306401610010105
• Keywords: Cystic fibrosis; Cough; Ivacaftor; Gastro-oesophageal reflux; G551D mutation; Lung function
• Public URL: https://hull-repository.worktribe.com/output/3609886