Department of Allied Health Professions, Centre for Health and Clinical Research, University of the West of England, Bristol, UK.
Department of Health and Social Sciences, Centre for Appearance Research, University of the West of England, Bristol, UK.
Disabil Rehabil. 2022 Sep;44(18):5231-5240. doi: 10.1080/09638288.2021.1933618. Epub 2021 Jun 8.
Hypermobility Spectrum Disorders (HSD) and Hypermobile Ehlers-Danlos Syndrome (hEDS) are heritable connective tissue disorders associated with joint instability and pain, but with scant guidance for supporting patients. The aim was to determine recommendations for an HSD/hEDS self-management intervention.
Barriers to self-management were mapped onto the Theoretical Domains Framework (TDF) and Capability, Opportunity, Motivation-Behaviour (COM-B) model in a behavioural analysis. A modified Nominal Group Technique was used to prioritise behaviour change technique (BCT) interventions ( = 9 women).
Possible BCTs incorporated.
Incorporating self-help strategies, education to improve their knowledge of HSD/hEDS, and how to judge information about HSD/hEDS.
In activity pacing, assertiveness and communication skills, plus what to expect during pregnancy, when symptoms can worsen.
Support from occupational therapists to maintain independence at work and home.
That illustrates how other people with HSD/hEDS have coped with the psychosocial impact.
This study is the first to apply theoretically-informed approaches to the management of HSD/hEDS. Participants indicated poor access to psychological support, occupational therapy and a lack of knowledge about HSD/hEDS. Future research should evaluate which intervention options would be most acceptable and feasible.Implications for rehabilitationPatients with Hypermobility Spectrum Disorders or Hypermobile Ehlers-Danlos Syndrome can be active partners in the co-design of behaviour change interventions.Behaviour change interventions should target psychological support and patient education, particularly patient information.Additional behaviour change interventions included environmental restructuring and enablement; adaptations to participants' environment with input from occupational therapy.Participants were keen to suggest opportunities for behavioural modelling; positive fist-person modelling narratives, written by those with HSD/hEDS, which addressed how they coped with the psychosocial impact of their condition.
过度活动综合征（HSD）和过度活动型埃勒斯-当洛斯综合征（hEDS）是与关节不稳定和疼痛相关的遗传性结缔组织疾病，但目前针对这些疾病的支持患者的方法很少。本研究旨在确定 HSD/hEDS 自我管理干预措施的建议。
通过行为分析，将自我管理障碍映射到理论领域框架（TDF）和能力、机会、动机-行为（COM-B）模型上。使用改良的名义小组技术对行为改变技术（BCT）干预措施进行了优先级排序（=9 名女性）。
确定了可能的 BCT 干预措施。
纳入自我帮助策略、提高患者对 HSD/hEDS 的认识和了解相关知识的教育，以及如何判断有关 HSD/hEDS 的信息。
活动节奏训练、自信和沟通技巧，以及在怀孕期间、症状可能恶化时会发生什么。
职业治疗师的支持，以保持工作和家庭的独立性。
说明其他患有 HSD/hEDS 的人如何应对心理社会影响。
这项研究是首次将理论指导方法应用于 HSD/hEDS 的管理。参与者表示难以获得心理支持、职业治疗以及对 HSD/hEDS 的了解。未来的研究应该评估哪些干预措施最可行。
患有过度活动综合征或过度活动型埃勒斯-当洛斯综合征的患者可以成为行为改变干预措施共同设计的积极合作伙伴。行为改变干预措施应针对心理支持和患者教育，特别是患者信息。增加的行为改变干预措施包括环境重构和赋权；职业治疗提供意见，对参与者的环境进行调整。参与者非常希望有机会进行行为建模；那些患有 HSD/hEDS 的人以第一人称撰写的正面叙事，可以描述他们如何应对病情对心理社会的影响。
