Grimstvedt Thea Nygaard, Miller Jeanette Ullmann, van Walsem Marleen Regina, Feragen Kristin J Billaud
Department of Special Needs Education, Faculty of Educational Sciences, University of Oslo, Oslo, Norway.
Centre for Rare Disorders, Oslo University Hospital, Oslo, Norway.
Int J Lang Commun Disord. 2021 Mar;56(2):330-345. doi: 10.1111/1460-6984.12604. Epub 2021 Feb 12.
Huntington's disease (HD) is a neurodegenerative disease characterized by a triad of motor, cognitive and psychological symptoms, leading to a gradual breakdown of communication skills. Few studies have investigated how people affected by HD and their professional caregivers, for example, medical doctors, physiotherapists and nurses, experience the patients' gradual loss of speech and language.
To examine communication-related experiences of patients and professional caregivers. Experiences with speech therapy and the use of augmentative and alternative communication aids (AAC) were also investigated.
METHODS & PROCEDURES: Seven individuals with HD and seven professional caregivers were interviewed individually, using a semi-structured interview guide. Transcripts were analysed using a conventional content analysis, and the results presented in three main categories.
OUTCOMES & RESULTS: Most individuals with HD were aware of having communication difficulties, struggling with understanding others as well as being understood. This was confirmed by professional caregivers, who also raised ethical issues encountered when patients struggled with communication. Both groups talked about external factors (such as noise or crowded social settings) as disrupting communication, and shared recommendations on how people in general, and speech and language therapists (SLTs) in particular, could optimize communication. Very few patients had received information about communication aids, and none was using AACs. Professional caregivers underlined the importance of interdisciplinary collaborations, including SLTs, in order to optimize care.
CONCLUSIONS & IMPLICATIONS: Findings shed a light on everyday communication challenges faced by people with HD and their professional caregivers, and the lack of implementation of communication aids in this group. The dramatic impact of HD on patients' communication skills underscores the need to include SLTs in the follow-up of this patient group, ideally from the early stages of the disease, while the patient is still capable of voicing his/her own wishes and thoughts. Future research that explores how to optimize communication and implement the use of AACs for individuals with HD is needed. What this paper adds What is already known on this subject Although the ability to communicate gradually deteriorates in individuals affected by Huntington's disease (HD), there is little knowledge about how affected individuals experience the loss of speech and language skills. Interdisciplinary care is recognised as essential for this patient group. However, professional caregivers' thoughts and experiences of communicating with their patients have not been fully explored. Recent years have seen a rapid growth of available communication supporting technologies that could potentially be helpful for individuals with HD, but limited attention has been given to this subject. What this paper adds to existing knowledge What do we now know as a result of this study that we did not know before the results highlight that patients are aware of problems with speech and language even in early phases of the disease, and include patients' personal outlook on problems with communication. Professional caregivers raised ethical issues encountered when patients struggled with communication. Both groups described specific strategies that could facilitate communication. There was a significant lack of experience and knowledge about augmentative and alternative communication aids (AACs). What are the potential or actual clinical implications of this work? Clinical implications of this study. The introduction of communication aids in the follow-up of patients with HD needs to be discussed with the patients in the early phases of the disease, and implemented while the individual still has the capacity to learn and take advantage of alternative communication support. Results underline the importance of including speech and language therapists (SLTs) into multidisciplinary care of patients with HD. SLTs should be available also for professional caregivers who need advice on how to facilitate conversations and social interactions, in order to optimise care of patients with HD.
亨廷顿舞蹈症（HD）是一种神经退行性疾病，其特征为运动、认知和心理症状三联征，导致沟通能力逐渐衰退。很少有研究调查受HD影响的人及其专业护理人员，如医生、物理治疗师和护士，如何看待患者言语和语言能力的逐渐丧失。
研究患者及专业护理人员与沟通相关的经历。同时调查言语治疗的经历以及辅助和替代沟通辅助工具（AAC）的使用情况。
采用半结构化访谈指南，分别对7名HD患者和7名专业护理人员进行了访谈。运用传统内容分析法对访谈记录进行分析，并将结果分为三大类呈现。
大多数HD患者意识到自己存在沟通困难，在理解他人以及让他人理解自己方面都有困难。专业护理人员也证实了这一点，他们还提出了患者在沟通困难时遇到的伦理问题。两组都谈到外部因素（如噪音或拥挤的社交场合）会干扰沟通，并分享了关于一般人，尤其是言语和语言治疗师（SLT）如何优化沟通的建议。很少有患者收到过关于沟通辅助工具的信息，且无人使用AAC。专业护理人员强调跨学科合作（包括SLT）对于优化护理的重要性。
研究结果揭示了HD患者及其专业护理人员在日常沟通中面临的挑战，以及该群体中沟通辅助工具的使用不足。HD对患者沟通能力的巨大影响凸显了在该患者群体的随访中纳入SLT的必要性，理想情况下是在疾病早期，患者仍能表达自己的愿望和想法时。未来需要开展研究，探索如何优化HD患者的沟通并实施AAC的使用。
本文补充内容
关于该主题的已有认知
尽管受亨廷顿舞蹈症（HD）影响的个体沟通能力会逐渐恶化，但对于受影响个体如何体验言语和语言技能丧失的了解却很少。跨学科护理被认为对该患者群体至关重要。然而，专业护理人员与患者沟通的想法和经历尚未得到充分探索。近年来，可用的沟通支持技术迅速发展，可能对HD患者有帮助，但对此主题的关注有限。
本文对现有知识的补充
这项研究让我们有了哪些之前不知道的新认识
结果表明，患者即使在疾病早期也意识到言语和语言问题，包括患者对沟通问题的个人看法。专业护理人员提出了患者在沟通困难时遇到的伦理问题。两组都描述了有助于沟通的具体策略。对于辅助和替代沟通辅助工具（AAC），明显缺乏经验和了解。
这项研究的潜在或实际临床意义是什么？
本研究的临床意义
在HD患者疾病早期就需要与他们讨论引入沟通辅助工具，并在个体仍有能力学习和利用替代沟通支持时实施。结果强调了将言语和语言治疗师（SLT）纳入HD患者多学科护理的重要性。对于需要关于如何促进对话和社交互动建议的专业护理人员，也应提供SLT，以优化对HD患者的护理。
