Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD)

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dc.contributor.author Behan, Laura
dc.contributor.author Leigh, Margaret W.
dc.contributor.author Dell, Sharon D.
dc.contributor.author Dunn Galvin, Audrey
dc.contributor.author Quittner, Alexandra L.
dc.contributor.author Lucas, Jane S.
dc.date.accessioned 2018-01-30T13:04:41Z
dc.date.available 2018-01-30T13:04:41Z
dc.date.issued 2017-09
dc.identifier.citation Behan, L., Leigh, M. W., Dell, S. D., Dunn Galvin, A., Quittner, A. L. and Lucas, J. S. (2017) 'Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD)', Thorax, 72(9), pp. 832-839. doi: 10.1136/thoraxjnl-2016-209356 en
dc.identifier.volume 72 en
dc.identifier.issued 9 en
dc.identifier.startpage 832 en
dc.identifier.endpage 839 en
dc.identifier.issn 0040-6376
dc.identifier.uri http://hdl.handle.net/10468/5351
dc.identifier.doi 10.1136/thoraxjnl-2016-209356
dc.description.abstract Background: Quality of life (QOL)-primary ciliary dyskinesia (PCD) is the first disease-specific, health-related QOL instrument for PCD. Psychometric validation of QOL-PCD assesses the performance of this measure in adults, including its reliability, validity and responsiveness to change. Methods: Seventy-two adults (mean (range) age: 33 years (18–79 years); mean (range) FEV1% predicted: 68 (26–115)) with PCD completed the 49-item QOL-PCD and generic QOL measures: Short-Form 36 Health Survey, Sino-Nasal Outcome Test 20 (SNOT-20) and St George Respiratory Questionnaire (SGRQ)-C. Thirty-five participants repeated QOL-PCD 10–14 days later to measure stability or reproducibility of the measure. Results: Multitrait analysis was used to evaluate how the items loaded on 10 hypothesised scales: physical, emotional, role and social functioning, treatment burden, vitality, health perceptions, upper respiratory symptoms, lower respiratory symptoms and ears and hearing symptoms. This analysis of item-to-total correlations led to 9 items being dropped; the validated measure now comprises 40 items. Each scale had excellent internal consistency (Cronbach's α: 0.74 to 0.94). Two-week test–retest demonstrated stability for all scales (intraclass coefficients 0.73 to 0.96). Significant correlations were obtained between QOL-PCD scores and age and FEV1. Strong relationships were also found between QOL-PCD scales and similar constructs on generic questionnaires, for example, lower respiratory symptoms and SGRQ-C (r=0.72, p<0.001), while weak correlations were found between measures of different constructs. Conclusions: QOL-PCD has demonstrated good internal consistency, test–retest reliability, convergent and divergent validity. QOL-PCD offers a promising tool for evaluating new therapies and for measuring symptoms, functioning and QOL during routine care. en
dc.description.sponsorship National Institutes of Health (NIH through the Genetic Disorders of Mucociliary Clearance Consortium, an initiative of the NIH Office of Rare Diseases Research at the National Center for Advancing Translational Science and the National Heart, Lung and Blood Institute); Maya’s March, The Hospital for Sick Children Foundation, Toronto, Ontario, Canada (Grant support); Gilead Sciences (Investigator-initiated grant); National PCD Centre in Southampton (commissioned and funded by NHS England); European Respiratory Society (ERS Task Force for PCD Diagnostics (ERS TF-2014-04)); European Cooperation in Science and Technology (COST Action BEAT-PCD (BM1407)) en
dc.format.mimetype application/pdf en
dc.language.iso en en
dc.publisher BMJ Publishing Group en
dc.relation.uri http://thorax.bmj.com/content/thoraxjnl/72/9/832.full.pdf
dc.rights © 2017 The Authors. Published by the BMJ Publishing Group Limited. This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ en
dc.rights.uri http://creativecommons.org/licenses/by-nc/4.0/ en
dc.subject QOL-PCD en
dc.subject Primary ciliary dyskinesia (PCD) en
dc.subject Psychometric validation en
dc.subject Genetic disorder en
dc.subject Treatment strategies en
dc.title Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD) en
dc.type Article (peer-reviewed) en
dc.internal.authorcontactother Audrey Dunn Galvin, Paediatrics & Child Health, University College Cork, Cork, Ireland. +353-21-490-3000 Email: a.dunngalvin@ucc.ie en
dc.internal.availability Full text available en
dc.date.updated 2018-01-30T12:44:51Z
dc.description.version Published Version en
dc.internal.rssid 423828486
dc.contributor.funder Seventh Framework Programme en
dc.contributor.funder National Institutes of Health en
dc.contributor.funder Gilead Sciences en
dc.contributor.funder National Institute for Health Research en
dc.contributor.funder Wellcome Trust en
dc.contributor.funder European Cooperation in Science and Technology en
dc.contributor.funder European Commission en
dc.contributor.funder AAIR Charity en
dc.contributor.funder European Respiratory Society en
dc.contributor.funder Maya’s March, The Hospital for Sick Children Foundation, Toronto, Ontario, Canada en
dc.description.status Peer reviewed en
dc.identifier.journaltitle Thorax en
dc.internal.copyrightchecked No !!CORA!! en
dc.internal.licenseacceptance Yes en
dc.internal.IRISemailaddress a.dunngalvin@ucc.ie en
dc.relation.project info:eu-repo/grantAgreement/EC/FP7::SP1::HEALTH/305404/EU/Better Experimental Screening and Treatment for Primary Ciliary Dyskinesia/BESTCILIA en


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© 2017 The Authors. Published by the BMJ Publishing Group Limited. This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ Except where otherwise noted, this item's license is described as © 2017 The Authors. Published by the BMJ Publishing Group Limited. This is an Open Access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/
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