Background

Prader-Willi Syndrome (PWS) is a complex multisystem genetic disorder associated with several challenges for people with PWS themselves, and for their families and care givers. Support around access to food is a particular issue due to impaired satiety and, because of this, people with PWS eat excessive amounts of food (hyperphagia). Together with other aspects of the PWS phenotype including, in many cases, a reduced sensitivity to pain, hyperphagia results in life-threatening obesity and life-shortening complications for some people with PWS. Restrictions to liberty and/or access to food raise important legal and ethical considerations in the clinical management of children and adults with PWS. Particularly where disagreements arise and, in the absence of comprehensive guidance for care providers, the courts may be called upon to resolve these difficult issues.

Aims

1) To review case-law from English-speaking common law jurisdictions concerning support arrangements for people with PWS with a view to identifying issues that have required the intervention of the courts. 2) To identify principles on which to base clinical guidelines relating to the issues identified, ensuring that such guidelines are consistent with ethical and human rights imperatives.

Methods

Westlaw, Westlaw AU, and Lexis Nexis were searched for case law concerning the treatment or support of a person with PWS.

Results

Fifteen cases from jurisdictions in Australia, New Zealand, Canada, the United Kingdom and the United States of America met inclusion criteria. Areas requiring judicial decision making included a) detention in psychiatric hospital; b) support in least restrictive environments c) eligibility for support services; d) guardianship; e) access to special education. Judicial decisions are discussed in the context of the United Nations' Convention on the Rights of Persons with Disabilities.

中文翻译：

---

对与普拉德-威利综合征患者的支持和治疗有关的判例法的范围审查

背景

Prader-Willi 综合征 (PWS) 是一种复杂的多系统遗传疾病，与 PWS 患者及其家人和护理人员面临的若干挑战相关。由于饱腹感受损，获得食物的支持是一个特殊问题，因此，患有 PWS 的人会吃过量的食物（食欲过盛）。与 PWS 表型的其他方面一起，包括在许多情况下对疼痛的敏感性降低，食欲过盛会导致一些 PWS 患者危及生命的肥胖和缩短寿命的并发症。对自由和/或获得食物的限制在患有 PWS 的儿童和成人的临床管理中引起了重要的法律和伦理考虑。特别是在出现分歧的情况下，并且在缺乏针对护理提供者的全面指导的情况下，

宗旨

1) 审查来自讲英语的普通法司法管辖区的关于 PWS 患者支持安排的判例法，以确定需要法院干预的问题。2) 确定与所识别问题相关的临床指南所依据的原则，确保此类指南符合伦理和人权要求。

方法

搜索了 Westlaw、Westlaw AU 和 Lexis Nexis，以查找有关 PWS 患者的治疗或支持的判例法。

结果

来自澳大利亚、新西兰、加拿大、英国和美利坚合众国的司法管辖区的 15 起案件符合纳入标准。需要作出司法决定的领域包括 a) 拘留在精神病院；b) 在限制最少的环境中提供支持 c) 获得支持服务的资格；d) 监护权；e) 接受特殊教育。司法决定在联合国《残疾人权利公约》的背景下进行讨论。