Caregivers' experience of sleep management in Smith-Magenis syndrome: a mixed-methods study.
Caregivers' experience of sleep management in Smith-Magenis syndrome: a mixed-methods study.
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DOI：
10.1186/s13023-021-02159-8
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发表时间：
2022-02-04
影响因子：
3.7
通讯作者：
Oliver C
中科院分区：
文献类型：
--
作者：
Agar G;Bissell S;Wilde L;Over N;Williams C;Richards C;Oliver C
Smith-Magenis综合征(SMS)是一种罕见的遗传综合征，与清晨醒来和白天嗜睡的独特特征有关。患有短信的儿童表现出很高的自我伤害和攻击性，比起同龄人，他们更喜欢成年人的关注，并有强烈的动机与特定的照顾者互动。此外，与认知能力相比，短信患者的适应能力较低，表现出高水平的冲动。综上所述，这些因素可能会导致个人彻夜未眠，需要警惕的照顾者监督。尽管有这些复杂性，但还没有研究描述照顾者采取的策略，以确保他们的孩子通过短信安全过夜，也没有研究考虑这些经历对照顾者或更广泛的家庭的影响。目前的研究使用混合方法来考虑不同年龄段短信患者照顾者的睡眠管理策略和挑战。护理者完成了一项关于睡眠管理和相关困难、干预措施的使用以及获得服务和支持的国际在线调查。在英国对14名照顾者进行了半结构化访谈，以增加对照顾者经验和英国环境变化优先事项的了解。访谈被逐字转录，并使用主题分析进行编码。来自在线调查(n = 40)的证据显示，睡眠不佳对短信患者和更广泛的家庭产生了广泛的影响。只有5%的照顾者报告睡眠问题对孩子没有影响，76%的人报告对自己有中度或极显著的影响。对于一些单独的照顾者来说，睡眠管理困难随着时间的推移而改善，而对于另一些人来说，报告没有变化。每周一次的休息成为49%的照顾者的理想安排，尽管只有14%的人能够享受到这一点。大多数照顾者(54%)没有得到喘息。定性访谈的主题分析揭示了短信行为表型的各个方面之间的相互作用，这可能导致与睡眠管理和安全有关的复杂和不寻常的陈述。描述了护理者在睡眠管理和支持方面的优先事项，并在使用短信敏感策略和提供喘息服务方面对服务产生了关键影响。网上版载有补充材料，可在10.1186/s13023-021-02159-8查阅。
Smith–Magenis syndrome (SMS) is a rare genetic syndrome associated with a unique profile of early morning waking and daytime sleepiness. Children with SMS evidence high rates of self-injury and aggression and have a preference for adult over peer attention, with strong motivation to interact with a particular caregiver. In addition, people with SMS have lower adaptive functioning skills relative to cognitive abilities and demonstrate high levels of impulsivity. Taken together, these factors may result in individuals being awake overnight requiring vigilant caregiver supervision. Despite these complexities, no study has described the strategies caregivers take to keep their children with SMS safe overnight or considered the impact of these experiences on caregivers or the wider family. The current study used a mixed-methods approach to consider sleep management strategies and challenges for caregivers of people with SMS at different ages. Caregivers completed an international online survey about sleep management and related difficulties, use of interventions and access to services and support. Semi-structured interviews were conducted with 14 caregivers in the UK to increase understanding of caregiver experiences and priorities for change in the UK context. Interviews were transcribed verbatim and coded using thematic analysis. Evidence from the online survey (n = 40) revealed wide-ranging impacts of poor sleep on the person with SMS and the wider family. Only 5% of caregivers reported that the sleep problems had no impact on their child, and 76% reported a moderately or extremely significant impact on themselves. For some individual caregivers, sleep management difficulties improved over time whereas for others no change was reported. Weekly respite emerged as the ideal provision for 49% of caregivers, although only 14% had access to this. The majority of caregivers (54%) received no respite. Thematic analysis of qualitative interviews revealed interactions between aspects of the behavioural phenotype of SMS which may contribute to complex and unusual presentations in relation to sleep management and safety. Caregivers’ priorities for sleep management and support were delineated, with key implications for services in terms of the use of SMS-sensitive strategies and respite provision. The online version contains supplementary material available at 10.1186/s13023-021-02159-8.
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影响因子：
8
作者：
Jan, James E.;Owens, Judith A.;Ipsiroglu, Osman S.
通讯作者：
Ipsiroglu, Osman S.
影响因子：
4
作者：
Potocki, L;Glaze, D;Lupski, JR
通讯作者：
Lupski, JR
DOI：
10.1111/j.1365-2788.2010.01337.x
发表时间：
2011-02-01
影响因子：
3.6
作者：
Arron, K.;Oliver, C.;Burbidge, C.
通讯作者：
Burbidge, C.
DOI：
10.1046/j.1365-2788.1998.4260481.x
发表时间：
1998-12-01
影响因子：
3.6
作者：
Dykens, EM;Smith, ACM
通讯作者：
Smith, ACM
影响因子：
1.5
作者：
Nag, Heidi Elisabeth;Hoxmark, Lise Beate;Naerland, Terje
通讯作者：
Naerland, Terje