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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
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.
0003-9888
1-7
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
48a7b1af-4d2b-4ec7-8927-84361a3c62a9
Jenkinson, Elizabeth
b1997162-8fa8-4d72-9b3a-a4b52e2dbef4
Byrne, Christopher
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Child, Jenny
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Davies, Justin
9f18fcad-f488-4c72-ac23-c154995443a9
Inskip, Hazel
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Lokulo-Sodipe, Oluwakemi
d428f857-0e58-4964-b1f0-136af7432805
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, 1-7. (doi:10.1136/archdischild-2018-314952).

Record type: Article

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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More information

Submitted date: 2018
Accepted/In Press date: 3 June 2018
e-pub ahead of print date: 28 June 2018

Identifiers

Local EPrints ID: 421665
URI: https://eprints.soton.ac.uk/id/eprint/421665
ISSN: 0003-9888
PURE UUID: 3563c26b-2741-40a7-92a9-9204d6aa2199
ORCID for Christopher Byrne: ORCID iD orcid.org/0000-0001-6322-7753
ORCID for Hazel Inskip: ORCID iD orcid.org/0000-0001-8897-1749
ORCID for Oluwakemi Lokulo-Sodipe: ORCID iD orcid.org/0000-0002-8169-3384
ORCID for Deborah Mackay: ORCID iD orcid.org/0000-0003-3088-4401
ORCID for Isabel Temple: ORCID iD orcid.org/0000-0002-6045-1781

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Date deposited: 19 Jun 2018 16:30
Last modified: 01 Jun 2019 00:36

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Contributors

Author: Lisa Ballard
Author: Elizabeth Jenkinson
Author: Jenny Child
Author: Justin Davies
Author: Hazel Inskip ORCID iD
Author: Oluwakemi Lokulo-Sodipe ORCID iD
Author: Deborah Mackay ORCID iD
Author: Emma L. Wakeling
Author: Isabel Temple ORCID iD
Author: Angela Fenwick

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