Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis



Shoaib Faruqi*, Dejene Shiferaw, Alyn H. Morice
Department of Respiratory Medicine, Castle Hill Hospital, Centre for Cardiovascular and Metabolic Research, Hull York Medical School, University of Hull, Yorkshire, UK


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© Faruqi et al.; Licensee Bentham Open

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.

* Address correspondence to this author at the Department of Respiratory Medicine, Castle Hill Hospital, Centre for Cardiovascular and Metabolic Research, Hull York Medical School, University of Hull, Yorkshire, United Kingdom; Fax: 01482624068l; Email: shoaib.faruqi@hey.nhs.uk


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.

Keywords: Cystic fibrosis, Cough, Ivacaftor, Gastro-oesophageal reflux, G551D mutation, Lung function.