Simpson Sheila A
Clinical Genetics Centre, First Floor Argyll House, Cornhill Road, Aberdeen, Scotland, UK.
Brain Res Bull. 2007 Apr 30;72(2-3):179-81. doi: 10.1016/j.brainresbull.2006.10.022. Epub 2006 Nov 16.
Huntington's disease (HD) is a relentlessly progressive neurodegenerative disease, which is presently incurable. Despite the hope for future therapies that exists for the families, they meantime are aware that there is little that their clinicians can do to delay either onset or progression of the disease. There are unique issues to address in a disorder where patients are aware in advance that their ability to communicate, as well as their cognitive capacity, will become impaired. Most affected individuals have experience of the disease in its late stages because of their parent's and other family member's illness. Many have their own ideas and anxieties about how their own care ought to proceed. There are no published guidelines for management of this stage of disease, although all clinicians involved in the care of HD will have their own experiences to share. This paper describes one method for such management, which includes discussion about placement for care advance directives for feeding and treatment.
亨廷顿舞蹈症（HD）是一种无情进展的神经退行性疾病，目前无法治愈。尽管患者家庭对未来的治疗抱有希望，但他们同时也意识到临床医生几乎无法延缓该病的发病或进展。对于这样一种疾病，患者提前知晓其沟通能力和认知能力将会受损，因此存在一些独特的问题需要解决。大多数受影响个体都经历过该病的晚期阶段，因为他们的父母和其他家庭成员患有此病。许多人对自己的护理应如何进行有自己的想法和焦虑。虽然参与HD护理的所有临床医生都有自己的经验可分享，但目前尚无关于该病此阶段管理的已发表指南。本文描述了一种这样的管理方法，其中包括关于护理安置、喂养预先指示和治疗的讨论。
