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

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dc.contributor.authorBehan, Laura
dc.contributor.authorLeigh, Margaret W.
dc.contributor.authorDell, Sharon D.
dc.contributor.authorDunn Galvin, Audrey
dc.contributor.authorQuittner, Alexandra L.
dc.contributor.authorLucas, Jane S.
dc.contributor.funderSeventh Framework Programmeen
dc.contributor.funderNational Institutes of Healthen
dc.contributor.funderGilead Sciencesen
dc.contributor.funderNational Institute for Health Researchen
dc.contributor.funderWellcome Trusten
dc.contributor.funderEuropean Cooperation in Science and Technologyen
dc.contributor.funderEuropean Commissionen
dc.contributor.funderAAIR Charityen
dc.contributor.funderEuropean Respiratory Societyen
dc.contributor.funderMaya’s March, The Hospital for Sick Children Foundation, Toronto, Ontario, Canadaen
dc.date.accessioned2018-01-30T13:04:41Z
dc.date.available2018-01-30T13:04:41Z
dc.date.issued2017-09
dc.date.updated2018-01-30T12:44:51Z
dc.description.abstractBackground: 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.sponsorshipNational 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.description.statusPeer revieweden
dc.description.versionPublished Versionen
dc.format.mimetypeapplication/pdfen
dc.identifier.citationBehan, 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-2016-209356en
dc.identifier.doi10.1136/thoraxjnl-2016-209356
dc.identifier.endpage839en
dc.identifier.issn0040-6376
dc.identifier.issued9en
dc.identifier.journaltitleThoraxen
dc.identifier.startpage832en
dc.identifier.urihttps://hdl.handle.net/10468/5351
dc.identifier.volume72en
dc.language.isoenen
dc.publisherBMJ Publishing Groupen
dc.relation.projectinfo:eu-repo/grantAgreement/EC/FP7::SP1::HEALTH/305404/EU/Better Experimental Screening and Treatment for Primary Ciliary Dyskinesia/BESTCILIAen
dc.relation.urihttp://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.urihttp://creativecommons.org/licenses/by-nc/4.0/en
dc.subjectQOL-PCDen
dc.subjectPrimary ciliary dyskinesia (PCD)en
dc.subjectPsychometric validationen
dc.subjectGenetic disorderen
dc.subjectTreatment strategiesen
dc.titleValidation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD)en
dc.typeArticle (peer-reviewed)en
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