title: A quality-of-life measure for adults with primary ciliary dyskinesia: QOL-PCD.
abstract: Primary ciliary dyskinesia (PCD) is characterised by chronic suppurative lung disease, rhino-sinusitis, hearing impairment and sub-fertility. We have developed the first multidimensional measure to assess health-related quality of life (HRQoL) in adults with PCD (QOL-PCD).Following a literature review and expert panel meeting, open-ended interviews with patients investigated the impact of PCD on HRQoL in the UK and North America (n=21). Transcripts were content analysed to derive saturation matrices. Items were rated for relevance by patients (n=49). Saturation matrices, relevance scores, literature review, evaluation of existing measures, and expert opinion contributed to development of a preliminary questionnaire. The questionnaire was refined following cognitive interviews (n=18).Open-ended interviews identified a spectrum of issues unique to adults with PCD. Saturation matrices confirmed comprehensive coverage of content. QOL-PCD includes 48 items covering the following seven domains: Physical Functioning, Emotional Functioning, Treatment Burden, Respiratory and Sinus Symptoms, Ears and Hearing, Social Functioning, and Vitality and Health Perceptions. Cognitive testing confirmed that content was comprehensive and the items were well-understood by respondents.Content validity and cognitive testing supported the items and structure. QOL-PCD has been translated into other languages and is awaiting psychometric testing.
authors.affiliation: Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK Academic Unit of Clinical and Experimental Sciences Faculty of Medicine, University of Southampton, Southampton, UK Both authors contributed equally jlucas1@soton.ac.uk.
authors.forename: Jane S
authors.initials: JS
authors.lastname: Lucas
authors.affiliation: Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK Academic Unit of Clinical and Experimental Sciences Faculty of Medicine, University of Southampton, Southampton, UK Dept of Applied Psychology, University College Cork, Cork, Ireland Both authors contributed equally.
authors.forename: Laura
authors.initials: L
authors.lastname: Behan
authors.affiliation: Dept of Applied Psychology, University College Cork, Cork, Ireland.
authors.forename: Audrey
authors.initials: A
authors.lastname: Dunn Galvin
authors.affiliation: Dept of Psychology, University of Miami, Coral Gables, FL, USA.
authors.forename: Adrianne
authors.initials: A
authors.lastname: Alpern
authors.affiliation: Dept of Psychology, University of Miami, Coral Gables, FL, USA.
authors.forename: Anjana M
authors.initials: AM
authors.lastname: Morris
authors.affiliation: Primary Ciliary Dyskinesia Centre, University Hospital Southampton NHS Foundation Trust, Southampton, UK NIHR Southampton Respiratory Biomedical Research Unit, University of Southampton and University Hospital Southampton NHS Foundation Trust, Southampton, UK Academic Unit of Clinical and Experimental Sciences Faculty of Medicine, University of Southampton, Southampton, UK.
authors.forename: Mary P
authors.initials: MP
authors.lastname: Carroll
authors.affiliation: Dept of Medicine and Pediatrics, University of North Carolina School of Medicine, Chapel Hill, NC, USA.
authors.forename: Michael R
authors.initials: MR
authors.lastname: Knowles
authors.affiliation: Dept of Medicine and Pediatrics, University of North Carolina School of Medicine, Chapel Hill, NC, USA.
authors.forename: Margaret W
authors.initials: MW
authors.lastname: Leigh
authors.affiliation: Dept of Psychology, University of Miami, Coral Gables, FL, USA.
authors.forename: Alexandra L
authors.initials: AL
authors.lastname: Quittner
mesh_terms: D006801:Humans; D007619:Kartagener Syndrome / Q000453:epidemiology* / Q000523:psychology*; D009656:North America; D011594:Psychometrics / Q000379:methods*; D011788:Quality of Life; D011795:Surveys and Questionnaires; D006113:United Kingdom