RESEARCH ARTICLE
Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis
Shoaib Faruqi*, Dejene Shiferaw, Alyn H. Morice
Article Information
Identifiers and Pagination:
Year: 2016Volume: 10
First Page: 105
Last Page: 108
Publisher ID: TORMJ-10-105
DOI: 10.2174/1874306401610010105
Article History:
Received Date: 10/11/2016Revision Received Date: 14/11/2016
Acceptance Date: 14/11/2016
Electronic publication date: 30/12/2016
Collection year: 2016

open-access license: 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) (https://creativecommons.org/licenses/by-nc/4.0/legalcode), which permits unrestricted, non-commercial use, distribution and reproduction in any medium, provided the work is properly cited.
Abstract
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.