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

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 o...

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Autores principales: Faruqi, Shoaib, Shiferaw, Dejene, Morice, Alyn H.
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Bentham Open 2016
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5299558/
https://www.ncbi.nlm.nih.gov/pubmed/28217196
http://dx.doi.org/10.2174/1874306401610010105
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author Faruqi, Shoaib
Shiferaw, Dejene
Morice, Alyn H.
author_facet Faruqi, Shoaib
Shiferaw, Dejene
Morice, Alyn H.
author_sort Faruqi, Shoaib
collection PubMed
description 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.
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spelling pubmed-52995582017-02-17 Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis Faruqi, Shoaib Shiferaw, Dejene Morice, Alyn H. Open Respir Med J Article 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. Bentham Open 2016-12-30 /pmc/articles/PMC5299558/ /pubmed/28217196 http://dx.doi.org/10.2174/1874306401610010105 Text en © Faruqi et al.; Licensee Bentham Open https://creativecommons.org/licenses/by/4.0/legalcode 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.
spellingShingle Article
Faruqi, Shoaib
Shiferaw, Dejene
Morice, Alyn H.
Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis
title Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis
title_full Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis
title_fullStr Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis
title_full_unstemmed Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis
title_short Effect of Ivacaftor on Objective and Subjective Measures of Cough in Patients with Cystic Fibrosis
title_sort effect of ivacaftor on objective and subjective measures of cough in patients with cystic fibrosis
topic Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC5299558/
https://www.ncbi.nlm.nih.gov/pubmed/28217196
http://dx.doi.org/10.2174/1874306401610010105
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