The lived experience of Silver-Russell syndrome: implications for management during childhood and into adulthood
The lived experience of Silver-Russell syndrome: implications for management during childhood and into adulthood
Objective: There is limited information on the psychosocial impact of growing up with Silver-Russell syndrome (SRS), characterised by slow growth in utero leading to short stature in adulthood. Such information could aid families in making difficult treatment decisions and guide management strategies for health professionals. We aimed to explore the lived experience of people with SRS across the lifespan.
Design/setting/patients: In-depth, semi-structured interviews were conducted between January 2015 and October 2016 with a sample of fifteen adults (six women) with genetically confirmed SRS from the UK. Qualitative interviews were transcribed and coded to identify similarities and differences: codes were then grouped to form overarching themes.
Results: Four themes were identified from participant accounts: 1) Appearance-related concerns extending beyond height; 2) Strategies to deal with real and perceived threats; 3) Women’s experiences of pain, disability, and feeling older than their years; and 4) Feeling overlooked in romantic relationships. These themes show that other factors, beyond short stature, affect patient well-being and indicate a mismatch exists between patient need and healthcare provision.
Conclusions: Challenges in SRS during childhood and adolescence were central to the psychosocial impact of SRS, and were not limited to height. These challenges, as well as symptoms such as pain and fatigue for women, have not previously been documented. To help individuals with SRS to develop strategies to manage psychosocial issues, we recommend clinicians incorporate psychological services as an integral part of multi-disciplinary teams managing individuals with SRS during childhood, adolescence, and adulthood.
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Ballard, Lisa
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Jenkinson, Elizabeth
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Byrne, Christopher
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Child, Jenny
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Davies, Justin
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Inskip, Hazel
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Lokulo-Sodipe, Oluwakemi
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Mackay, Deborah
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Wakeling, Emma L.
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Temple, Isabel
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Fenwick, Angela
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Ballard, Lisa
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Jenkinson, Elizabeth
b1997162-8fa8-4d72-9b3a-a4b52e2dbef4
Byrne, Christopher
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Child, Jenny
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Davies, Justin
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Inskip, Hazel
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Lokulo-Sodipe, Oluwakemi
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Mackay, Deborah
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Wakeling, Emma L.
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Temple, Isabel
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Fenwick, Angela
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Ballard, Lisa, Jenkinson, Elizabeth, Byrne, Christopher, Child, Jenny, Davies, Justin, Inskip, Hazel, Lokulo-Sodipe, Oluwakemi, Mackay, Deborah, Wakeling, Emma L., Temple, Isabel and Fenwick, Angela
(2018)
The lived experience of Silver-Russell syndrome: implications for management during childhood and into adulthood.
Archives of Disease in Childhood, .
(doi:10.1136/archdischild-2018-314952).
Abstract
Objective: There is limited information on the psychosocial impact of growing up with Silver-Russell syndrome (SRS), characterised by slow growth in utero leading to short stature in adulthood. Such information could aid families in making difficult treatment decisions and guide management strategies for health professionals. We aimed to explore the lived experience of people with SRS across the lifespan.
Design/setting/patients: In-depth, semi-structured interviews were conducted between January 2015 and October 2016 with a sample of fifteen adults (six women) with genetically confirmed SRS from the UK. Qualitative interviews were transcribed and coded to identify similarities and differences: codes were then grouped to form overarching themes.
Results: Four themes were identified from participant accounts: 1) Appearance-related concerns extending beyond height; 2) Strategies to deal with real and perceived threats; 3) Women’s experiences of pain, disability, and feeling older than their years; and 4) Feeling overlooked in romantic relationships. These themes show that other factors, beyond short stature, affect patient well-being and indicate a mismatch exists between patient need and healthcare provision.
Conclusions: Challenges in SRS during childhood and adolescence were central to the psychosocial impact of SRS, and were not limited to height. These challenges, as well as symptoms such as pain and fatigue for women, have not previously been documented. To help individuals with SRS to develop strategies to manage psychosocial issues, we recommend clinicians incorporate psychological services as an integral part of multi-disciplinary teams managing individuals with SRS during childhood, adolescence, and adulthood.
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The lived experience of Silver Russell syndrome implications for management during childhood and into adulthood
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Submitted date: 2018
Accepted/In Press date: 3 June 2018
e-pub ahead of print date: 28 June 2018
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Local EPrints ID: 421665
URI: http://eprints.soton.ac.uk/id/eprint/421665
ISSN: 0003-9888
PURE UUID: 3563c26b-2741-40a7-92a9-9204d6aa2199
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Date deposited: 19 Jun 2018 16:30
Last modified: 16 Mar 2024 06:43
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Author:
Elizabeth Jenkinson
Author:
Jenny Child
Author:
Oluwakemi Lokulo-Sodipe
Author:
Emma L. Wakeling
Author:
Angela Fenwick
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