Dale Maria, Wood Ashleigh, Zarotti Nicolò, Eccles Fiona, Gunn Sarah, Kiani Reza, Mobley Amanda, Robertson Noelle, Simpson Jane
Huntington's Disease Service, Leicestershire Partnership NHS Trust, Mill Lodge, The Rise, Narborough, Leicester LE19 4SL, UK.
Department of Neuroscience, Psychology and Behaviour, University of Leicester, University Road, Leicester LE1 7RH, UK.
J Pers Med. 2022 Jul 27;12(8):1222. doi: 10.3390/jpm12081222.
Huntington's disease (HD) is an inherited, life-limiting neurodegenerative condition. People with HD experience changes in cognitive, motor and emotional functioning, and can also, mainly at later stages, exhibit behaviours that professionals and carers might find distressing such as hitting others, throwing objects, swearing or making inappropriate comments. While clinical formulation (an individualised approach used by mental health professionals to describe an individual's difficulties) is a helpful tool to conceptualise patients' wellbeing, a specific formulation framework has not yet been developed for HD. However, evidence has shown that formulation can help guide clinical interventions and increase consistency of approach across multi-disciplinary teams, refine risk management, and improve staff or carers' empathic skills and understanding of complex presentations. As a consequence, this paper proposes a new clinical formulation model for understanding distress among people with HD, based on a biopsychosocial framework. More specifically, this includes key elements centring on an individual's past experience and personal narratives, as well as anticipatory cognitions and emotions about the future. In-depth discussions regarding the components of the model and their importance in HD formulations are included, and a fictional yet representative case example is presented to illustrate their application within the context of personalised care.
亨廷顿舞蹈症（HD）是一种遗传性、会危及生命的神经退行性疾病。患有HD的人在认知、运动和情感功能方面会出现变化，并且在疾病后期，还可能表现出令专业人员和护理人员感到苦恼的行为，比如殴打他人、扔东西、咒骂或发表不当言论。虽然临床诊断（心理健康专业人员用来描述个体困难的一种个性化方法）是理解患者健康状况的有用工具，但尚未针对HD开发出具体的诊断框架。然而，有证据表明，诊断有助于指导临床干预，并提高多学科团队方法的一致性，完善风险管理，以及提高工作人员或护理人员的共情能力和对复杂症状的理解。因此，本文基于生物心理社会框架，提出了一种新的临床诊断模型，用于理解HD患者的痛苦。更具体地说，这包括以个人过去的经历和个人叙述为核心的关键要素，以及对未来的预期认知和情绪。文中还对该模型的组成部分及其在HD诊断中的重要性进行了深入讨论，并给出了一个虚构但具有代表性的案例，以说明它们在个性化护理背景下的应用。
