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...
Autores principales: | , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Bentham Open
2016
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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. |
format | Online Article Text |
id | pubmed-5299558 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2016 |
publisher | Bentham Open |
record_format | MEDLINE/PubMed |
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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