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Quality of Life Research

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Open Access 01-09-2017 | Review

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

Authors: Laura Behan, Bruna Rubbo, Jane S. Lucas, Audrey Dunn Galvin

Published in: Quality of Life Research | Issue 9/2017

Abstract

Background

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.

Method

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.

Results

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.

Conclusion

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.

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Title: The patient’s experience of primary ciliary dyskinesia: a systematic review
Authors: Laura Behan
Bruna Rubbo
Jane S. Lucas
Audrey Dunn Galvin
Publication date: 01-09-2017
Publisher: Springer International Publishing
Published in: Quality of Life Research / Issue 9/2017
Print ISSN: 0962-9343
Electronic ISSN: 1573-2649
DOI: https://doi.org/10.1007/s11136-017-1564-y

Keynote webinar | Spotlight on medication adherence

Springer Medicine

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

Abstract

Background

Method

Results

Conclusion

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Method

Results

Conclusion

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