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

The patient's experience of primary ciliary dyskinesia: a systematic review
The patient's experience of primary ciliary dyskinesia: a systematic review
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.
0962-9343
Behan, Laura
cf1a7b5e-64c5-4b02-8db2-7ad96781d40d
Rubbo, Bruna
dc31cd48-3d84-41ab-a8b8-351c9914dca4
Lucas, Jane
5cb3546c-87b2-4e59-af48-402076e25313
Dunn Galvin, Audrey
df33ae04-bb19-4024-83ed-25c3e3a676e0
Behan, Laura
cf1a7b5e-64c5-4b02-8db2-7ad96781d40d
Rubbo, Bruna
dc31cd48-3d84-41ab-a8b8-351c9914dca4
Lucas, Jane
5cb3546c-87b2-4e59-af48-402076e25313
Dunn Galvin, Audrey
df33ae04-bb19-4024-83ed-25c3e3a676e0

Behan, Laura, Rubbo, Bruna, Lucas, Jane and Dunn Galvin, Audrey (2017) The patient's experience of primary ciliary dyskinesia: a systematic review. Quality of Life Research. (doi:10.1007/s11136-017-1564-y).

Record type: Article

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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More information

Accepted/In Press date: 24 March 2017
e-pub ahead of print date: 30 March 2017
Organisations: Cancer Sciences, Allergy & Inflammation Research, Clinical & Experimental Sciences

Identifiers

Local EPrints ID: 410980
URI: https://eprints.soton.ac.uk/id/eprint/410980
ISSN: 0962-9343
PURE UUID: b4fc571e-323a-4a64-a94c-d278b71c2f66
ORCID for Bruna Rubbo: ORCID iD orcid.org/0000-0002-1629-8601

Catalogue record

Date deposited: 12 Jun 2017 16:31
Last modified: 14 Mar 2019 01:32

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