Tarolli Christopher G, Chesire Amy M, Biglan Kevin M
Department of Neurology, University of Rochester, Rochester, NY, USA.
Early Phase Clinical Development, Neurosciences, Eli Lilly and Company, Indianapolis, IN, USA.
Tremor Other Hyperkinet Mov (N Y). 2017 Apr 11;7:454. doi: 10.7916/D88057C7. eCollection 2017.
Huntington disease is a fatal, autosomal dominant, neurodegenerative disorder manifest by the triad of a movement disorder, behavioral disturbances, and dementia. At present, no curative or disease modifying therapies exist for the condition and current treatments are symptomatic. Palliative care is an approach to care that focuses on symptom relief, patient and caregiver support, and end of life care. There is increasing evidence of the benefit of palliative care throughout the course of neurodegenerative conditions including Parkinson disease and amyotrophic lateral sclerosis. However, beyond its application at the end of life, little is known about the role of palliative care in Huntington disease.
In this article, we discuss what is known about palliative care in Huntington disease, specifically related to early disease burden, caregiver burnout, advance care planning, and end of life care.
We provide a review of the current literature and discuss our own care practices.
We conclude by discussing questions that remain unanswered and positing ideas for future work in the field.
亨廷顿舞蹈症是一种致命的常染色体显性神经退行性疾病，其特征为运动障碍、行为紊乱和痴呆三联征。目前，针对该疾病尚无治愈或改善病情的疗法，当前治疗仅为对症治疗。姑息治疗是一种以缓解症状、支持患者及照料者以及临终关怀为重点的护理方式。越来越多的证据表明，姑息治疗在包括帕金森病和肌萎缩侧索硬化症在内的神经退行性疾病的整个病程中都有益处。然而，除了在临终阶段的应用外，对于姑息治疗在亨廷顿舞蹈症中的作用知之甚少。
在本文中，我们讨论了关于亨廷顿舞蹈症姑息治疗的已知情况，特别是与疾病早期负担、照料者倦怠、预先护理计划和临终关怀相关的内容。
我们对当前文献进行了综述并讨论了我们自己的护理实践。
我们通过讨论仍未得到解答的问题并提出该领域未来工作的想法来得出结论。
