Abstract：
BACKGROUND: Self-determination refers to setting goals and making decisions regarding one\'s own life with support from others as needed. Research on people with intellectual and developmental disabilities has established the importance of self-determination for quality of life outcomes, such as increased independence and life satisfaction. However, self-determination has not been characterised specifically in fragile X syndrome (FXS), the leading inherited cause of intellectual disability. Relative to youth with other forms of intellectual and developmental disabilities, youth with FXS may face exceptional barriers to the development of self-determined behaviour. In addition to intellectual disability, the FXS behavioural profile is characterised by high rates of autism and anxiety that may further limit opportunities for youth with FXS. The heritable nature of the condition can also yield a distinctive family environment, with siblings and parents also living with fragile X or its associated conditions. Considering these unique challenges, the present study examined self-determination in young adult males and females with FXS and explored whether factors such as language skills, adaptive behaviour and autism traits were associated with self-determination capacity and opportunities.
METHODS: The present study included 9 females and 36 males with FXS between the ages of 17 and 25 years. Caregivers (mothers or fathers) completed the American Institute for Research Self-Determination Assessment, which is a questionnaire that yields three scores: self-determination capacity, opportunities for self-determination at home and opportunities for self-determination at school.
RESULTS: Caregivers endorsed a wide range of self-determination capacity and opportunities, with ratings for opportunities at home and school exceeding ratings of capacity. Better adaptive behaviour skills were associated with more self-determination capacity, and the presence of more autism traits was associated with fewer opportunities at school.
CONCLUSIONS: Results from this study contribute to our understanding of avenues to best support young adults with FXS as they transition to adulthood. Our findings also have implications for practice, such that interventions targeting adaptive behaviours and self-determination may be an effective approach for promoting autonomy and independence for young adults with FXS. Additionally, caregivers and educators should continue to provide opportunities to practise self-determination, regardless of their perception of capacity.
METHODS: The present study included 9 females and 36 males with FXS between the ages of 17 and 25 years. Caregivers (mothers or fathers) completed the American Institute for Research Self-Determination Assessment, which is a questionnaire that yields three scores: self-determination capacity, opportunities for self-determination at home and opportunities for self-determination at school.
RESULTS: Caregivers endorsed a wide range of self-determination capacity and opportunities, with ratings for opportunities at home and school exceeding ratings of capacity. Better adaptive behaviour skills were associated with more self-determination capacity, and the presence of more autism traits was associated with fewer opportunities at school.
CONCLUSIONS: Results from this study contribute to our understanding of avenues to best support young adults with FXS as they transition to adulthood. Our findings also have implications for practice, such that interventions targeting adaptive behaviours and self-determination may be an effective approach for promoting autonomy and independence for young adults with FXS. Additionally, caregivers and educators should continue to provide opportunities to practise self-determination, regardless of their perception of capacity.
摘要：
背景：自决是指根据需要在他人的支持下设定目标并做出关于自己生活的决定。对智力和发育障碍者的研究确立了自决对生活质量的重要性，例如增加独立性和生活满意度。然而,在脆性X综合征(FXS)中没有明确的自决特征，智力残疾的主要遗传原因。相对于有其他形式智力和发育障碍的青年，患有FXS的青年在发展自我决定的行为方面可能面临特殊的障碍。除了智力残疾，FXS行为特征表现为自闭症和焦虑的高发率,这可能进一步限制FXS青少年的机会.条件的遗传性也可以产生独特的家庭环境，兄弟姐妹和父母也患有脆弱的X或其相关疾病。考虑到这些独特的挑战，本研究调查了使用FXS的年轻成年男性和女性的自决，并探讨了语言技能等因素是否，适应行为和自闭症特征与自我决定能力和机会相关。
方法：本研究包括年龄在17至25岁之间的9名女性和36名男性FXS。照顾者（母亲或父亲）完成了美国研究所的自我决定评估，这是一个问卷，产生三个分数:自决能力，在家里自决的机会和在学校自决的机会。
结果：护理人员认可了广泛的自决能力和机会，家庭和学校的机会评级超过容量评级。更好的适应行为技能与更多的自决能力相关，更多的自闭症特征的存在与学校的机会减少有关。
结论：这项研究的结果有助于我们理解最佳支持FXS年轻人过渡到成年期的途径。我们的发现对实践也有启示，因此，针对适应性行为和自决的干预措施可能是促进FXS年轻成年人自主性和独立性的有效方法。此外,护理人员和教育工作者应继续提供实践自决的机会，不管他们对能力的看法。
方法：本研究包括年龄在17至25岁之间的9名女性和36名男性FXS。照顾者（母亲或父亲）完成了美国研究所的自我决定评估，这是一个问卷，产生三个分数:自决能力，在家里自决的机会和在学校自决的机会。
结果：护理人员认可了广泛的自决能力和机会，家庭和学校的机会评级超过容量评级。更好的适应行为技能与更多的自决能力相关，更多的自闭症特征的存在与学校的机会减少有关。
结论：这项研究的结果有助于我们理解最佳支持FXS年轻人过渡到成年期的途径。我们的发现对实践也有启示，因此，针对适应性行为和自决的干预措施可能是促进FXS年轻成年人自主性和独立性的有效方法。此外,护理人员和教育工作者应继续提供实践自决的机会，不管他们对能力的看法。
