Validation of a health-related quality of life instrument for primary ciliary dyskinesia (QOL-PCD)
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Published version
Date
2017-09
Authors
Behan, Laura
Leigh, Margaret W.
Dell, Sharon D.
Dunn Galvin, Audrey
Quittner, Alexandra L.
Lucas, Jane S.
Journal Title
Journal ISSN
Volume Title
Publisher
BMJ Publishing Group
Published Version
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.
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Keywords
QOL-PCD , Primary ciliary dyskinesia (PCD) , Psychometric validation , Genetic disorder , Treatment strategies
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