The Open Respiratory Medicine Journal


ISSN: 1874-3064 ― Volume 11, 2017

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

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.


Article Information


Identifiers and Pagination:

Year: 2016
Volume: 10
First Page: 105
Last Page: 108
Publisher Id: TORMJ-10-105
DOI: 10.2174/1874306401610010105

Article History:

Received Date: 10/11/2016
Revision Received Date: 14/11/2016
Acceptance Date: 14/11/2016
Electronic publication date: 30/12/2016
Collection year: 2016

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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


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