The patient's experience of primary ciliary dyskinesia: a systematic review

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dc.contributor.author Behan, Laura
dc.contributor.author Rubbo, Bruna
dc.contributor.author Lucas, Jane S.
dc.contributor.author Dunn Galvin, Audrey
dc.date.accessioned 2018-07-18T11:56:21Z
dc.date.available 2018-07-18T11:56:21Z
dc.date.issued 2017
dc.identifier.citation Behan, L., Rubbo, B., Lucas, J. S. and Dunn Galvin, A. (2017) 'The patient’s experience of primary ciliary dyskinesia: a systematic review', Quality of Life Research, 26(9), pp. 2265-2285. 10.1007/s11136-017-1564-y en
dc.identifier.volume 26
dc.identifier.issued 9
dc.identifier.startpage 2265
dc.identifier.endpage 2285
dc.identifier.issn 0962-9343
dc.identifier.uri http://hdl.handle.net/10468/6476
dc.identifier.doi 10.1007/s11136-017-1564-y
dc.description.abstract Primary ciliary dyskinesia (PCD) is a rare genetic disorder characterised by progressive sinopulmonary disease, with symptoms starting soon after birth. The aim of this study is to critically review, analyse, and synthesise the literature in order to understand the experiences of patients with primary ciliary dyskinesia (PCD) and the impact on health-related quality of life. MEDLINE, EBSCO, Cumulative Index to Nursing and Allied Health Literature (CINAHL), PsycINFO and EMBASE were searched according to the inclusion criteria. A qualitative analysis of 14 studies was conducted. Fourteen studies were included in the review, five with qualitative methodologies. Studies originated from the UK, USA, Italy, Denmark and Belgium, one study included a survey distributed internationally. Significant relationships were found between age and worsening of respiratory symptoms, physical, and mental domains of health-related quality of life, with a greater decline compared with reference populations. Variations between the UK and Italy were found for health-related quality of life and its correlation with time since diagnosis. PCD was found to have a physical impact in all age groups: patients found it difficult to keep up with others, and found energy levels were easily depleted compared to family or peers. In terms of social impact, symptoms lead to embarrassment and a sense of isolation, with patients concealing symptoms and/or their diagnosis. In turn, isolation was also linked with the lack of public and medical knowledge. In relation to emotional impact, anxiety was reported in a number of qualitative studies; patients were anxious about getting sick or when thinking about their future health. The burden of treatment and factors influencing adherence were also discussed in depth. Health-related quality of life decreases with age in patients with PCD. For all age groups, PCD was found to greatly impact physical, emotional, social functioning, and treatment burden. More research is needed on the psychosocial impact of the illness, disease burden and its effect on quality of life. en
dc.description.sponsorship NHS England; National Institute for Health Research (Southampton Respiratory Biomedical Research Unit); National Institute for Health Research/ Wellcome Trust (Clinical Research Facility); AAIR Charity (1129698) en
dc.format.mimetype application/pdf en
dc.language.iso en en
dc.publisher Springer International Publishing AG en
dc.relation.uri https://link.springer.com/article/10.1007%2Fs11136-017-1564-y
dc.rights © 2017, the Authors. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. en
dc.rights.uri http://creativecommons.org/licenses/by/4.0/
dc.subject Primary ciliary dyskinesia en
dc.subject Patient perspective en
dc.subject Health-related quality of life en
dc.subject Patient experience en
dc.title The patient's experience of primary ciliary dyskinesia: a systematic review en
dc.type Review 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.description.version Published Version en
dc.contributor.funder National Health Service, United Kingdom
dc.contributor.funder AAIR Charity
dc.contributor.funder Wellcome Trust
dc.contributor.funder National Institute for Health Research
dc.description.status Peer reviewed en
dc.identifier.journaltitle Quality of Life Research en
dc.internal.IRISemailaddress a.dunngalvin@ucc.ie en


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© 2017, the Authors. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made. Except where otherwise noted, this item's license is described as © 2017, the Authors. This article is distributed under the terms of the Creative Commons Attribution 4.0 International License (http://creativecommons.org/licenses/by/4.0/), which permits unrestricted use, distribution, and reproduction in any medium, provided you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons license, and indicate if changes were made.
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