FAMILY ADAPTATION TO FRAGILE X SYNDROME ADOLESCENTS AND ADULTS

A.具体目标 有一个患有发育障碍(DD)的儿子或女儿给家庭带来了许多挑战(Minnes， 1998年)。在大多数家庭中，母亲承担了最大份额的育儿责任(Essex&Hong，2005； Hastings，2003；Heller等人，1997)。因此，母亲对这些问题的经历和反应 挑战是她自身健康和幸福以及护理质量的重要决定因素 她能够创造的支持她孩子发展的背景(Stoneman，1997)：作为一个群体， 脆性X综合征(FXS)--智力残疾的主要遗传原因--的个体 糟糕的是，显示的心理健康状况不太像患有 唐氏综合症(DS)比自闭症患者的母亲(Abbeduto，Seltzer，Shattuck，Krauss，Orsmond， &Murphy，2004)。患有FXS的母亲对孩子的未来也有更悲观的看法 对家庭中的关系和互动模式的看法不如有孩子的母亲积极 DS再次与自闭症患者的母亲更相似(Abbeduto等人，2004；Lewis等人， 2006a)。然而，在FXS患者的母亲中，他们的 健康、心理健康和对家庭的看法(Bailey，Sideris，&Roberts，正在审查中)。一点儿 然而，关于个体母亲之间的这些差异的前因后果是已知的 使用FXS。母亲的幸福感以及对家庭关系和适应的看法与 FXS患者挑战行为的严重性，包括那些反映自闭症的行为， 以及母亲的特征(例如，教育)和背景变量(例如，患有 家庭和家庭收入中的外汇；Abbeduto等人，2004年)。然而，这些关系一直是 只检查同时测量的变量，因此无法确定影响的方向 (Esbensen、Seltzer和Abbeduto，正在印刷中)。此外，母亲的幸福感和 一方面，家庭环境，另一方面，孩子的行为，可能涉及许多干预 尚未调查的流程。生物脆弱性的变化如何与 母体基因(如CCG重复大小、激活率、FMR1基因水平)对母体的幸福感有影响 对压力的反应目前也是未知的(Esbensen等人，在出版中)。 鉴于家庭在FXS患者生活中的中心地位，服务系统对 家庭作为首要的支持来源，以及终生家庭照料的压力，已迫在眉睫 健康和科学需要了解影响个人适应的因素范围 FXS及其家人的福祉。对于那些有儿子的家庭来说，对这些数据的需求尤为迫切 或女儿处于青春期或青春期，因为这些时期与智商下降有关， 适应行为发展速度减慢，自闭症样行为严重程度增加 FXS患者(Hatton等人，2006年)。事实上，到目前为止的证据表明，育儿压力和 对于抚养青少年或患有FXS的成年人的母亲来说，心理健康方面的挑战比母亲更大 用FXS养育儿童(Esbensen等人，正在出版中)。因此，拟议的研究旨在审查 青少年和成人亲生母亲幸福感的纵向过程及其决定因素 在FXS中，这些母亲所经历的日常压力的性质，以及既有 由FXS塑造并使用FXS塑造个体。此外，这项研究将探索母亲之间的这两种差异 患有FXS的个体，以及这些母亲与母亲通常养育子女的不同程度 (TD)患有自闭症谱系障碍的儿女以及养育儿女的母亲 (ASD)。因此，这两个比较组将提供有用的基准来衡量母亲的水平 FXS样本的健康和福祉以及家庭适应。

A. SPECIFIC AIMS Having a son or daughter with a developmental disability (DD) poses many challenges for families (Minnes, 1998). In most families, mothers assume the greatest share of child care responsibilities (Essex & Hong, 2005; Hastings, 2003; Heller et al., 1997). Consequently, a mother's experience of, and response to, those challenges are important determinants of her own health and well-being and of the quality of the caretaking context that she is able to create to support her child's development (Stoneman, 1997): As a group, mothers of individuals with fragile X syndrome (FXS) - the leading inherited cause of intellectual disability - fare rather poorly, displaying profiles of psychological well-being that are less like those of mothers of individuals with Down syndrome (DS) than mothers of individuals with autism (Abbeduto, Seltzer, Shattuck, Krauss, Orsmond, & Murphy, 2004). Mothers of individuals with FXS also have more pessimistic views about their child's future and less positive views of relationships and patterns of interaction within the family than mothers of individuals with DS, again being more similar to mothers of individuals with autism (Abbeduto et al., 2004; Lewis et al., 2006a). Nevertheless, there is considerable variability among mothers of individuals with FXS as regards their health, psychological well-being, and perceptions of the family (Bailey, Sideris, & Roberts, under review). Little is known, however, about the antecedents or consequences of these differences among mothers of individuals with FXS. Maternal well-being and perceptions of family relationships and adaptation are correlated with the severity of the challenging behaviors of the individual with FXS, including those behaviors reflective of autism, as well as with maternal characteristics (e.g., education) and contextual variables (e.g., number of children with FXS in the family and family income; Abbeduto et al., 2004). However, these relationships have been examined only for concurrently measured variables, making it impossible to determine the direction of effects (Esbensen, Seltzer, & Abbeduto, in press). Moreover, the pathways between maternal well-being and the family environment, on the one hand, and child behavior, on the other, likely involve a number of intervening processes that have not yet been investigated. How variations in biological vulnerability associated with maternal genotype (e.g., CCG repeat size, activation ratio, FMR1 mRNA levels) contribute to maternal wellbeing and responses to stress are unknown at this time as well (Esbensen et al., in press). Given the centrality of the family in the life of a person with FXS, the reliance of the service system on the family as the primary source of support, and the stress of lifelong family caregiving, there is an urgent public health and scientific need to understand the range of factors that influence the adaptation of individuals with FXS and the well-being of their families. The need for such data is particularly pressing for families whose son or daughter is in adolescence or young adulthood because these periods are associated with a decline in IQ, a slowing rate of adaptive behavior development, and an increase in severity of autistic-like behaviors for individuals with FXS (Hatton et al., 2006). Indeed, the evidence to date suggests that parenting stress and mental health challenges are greater for mothers parenting adolescents or adults with FXS than for mothers parenting children with FXS (Esbensen et al., in press). The proposed study, therefore, is designed to examine the longitudinal course and determinants of the well-being of the biological mothers of adolescents and adults with FXS, the nature of the daily stresses experienced by these mothers, and the family context that is both shaped by and shapes the individual with FXS. Moreover, the study will explore both variation among mothers of individuals with FXS and the extent to which such mothers differ from mothers parenting typically developing (TD) sons and daughters as well as mothers parenting sons and daughters with an autism spectrum disorder (ASD). Thus, these two comparison groups will provide useful benchmarks for gauging levels of maternal health and well-being and family adaptation for the FXS sample.