Developing an Intervention Model for Complex Communication Needs in Rett Syndrome

项目总结/摘要 尽管在理解Rett综合征（RTT）的遗传学方面有了深入的关注和进展， 仍然明显缺乏证据基础来指导针对传播的应用干预措施。我们长久以来- 长期目标是制定最佳实践，以改善通信成果和生活质量， 这将有助于帮助患有RTT和其他类似疾病的人，并克服提供服务的障碍。的目标 本申请旨在开发一种基于认知的干预模式，以改善沟通 成果和指标，并扩大我们的影响， 利用网真接触到更多的女孩和妇女，否则是不可行的。通过 根据我们初步研究的结果，这些目标的核心假设是， 患有RTT的女性有沟通的能力，她们会用特殊的行为来 在没有系统干预的情况下沟通。我们计划通过以下方式来检验这一中心假设： 以下两个具体目标：（1）制定一个基于预防的干预模式， RTT中复杂的通信需求和（2）评估网真作为一种创新的服务交付 用于在RTT中支持/递送通信干预的机制。我们将通过以下方式实现这些目标： 严格的受试者内实验设计和三阶段分析的组统计分析， 干预序列，至少有24名女孩和妇女与RTT。我们的研究将有助于基于证据的 通过为患有RTT的个体设定护理标准， RTT和相关的复杂通信需求，它将奠定基础的科学认识， 对该人群和其他具有复杂沟通的人群进行基于认知的干预的有效性 它将展示远程呈现的有效使用，这将极大地扩展交付能力， 有效照顾（国内，国际）受RTT影响的个人的沟通需求， 家庭我们的建议是创新的，在我们看来，因为它拒绝了假设， 不具备获得有意沟通技能所需的发展水平，相反， 使用网真作为接触受RTT影响的家庭的工具， 干预措施，以发展沟通技能，从而提高生活质量。

Project Summary/Abstract Despite intensive focus and advances in understanding the genetics of Rett syndrome (RTT), there remains a strikingly absent evidence base to guide applied interventions targeting communication. Our long- term goals are to develop best practices for improvement in communication outcomes and quality of life in individuals with RTT and other similar disorders and to overcome barriers to service delivery. The objectives of this application are to develop a model of reinforcement-based intervention for improving the communicative outcomes and indicators of quality of life for girls and women with RTT, and to broaden our impact through the use of telepresence to reach a larger number of girls and women than would otherwise be feasible. Through findings from our preliminary research, these objectives operate from the central hypothesis that girls and women with RTT have the capacity to communicate, and that they will use idiosyncratic behaviors to communicate in the absence of systematic intervention. We plan to test this central hypothesis through the following two specific aims: (1) to develop a reinforcement-based intervention model that addresses the complex communicative needs in RTT and (2) to evaluate telepresence as an innovative service delivery mechanism for supporting/delivering communication intervention in RTT. We will address these aims through rigorous within-subject experimental designs and group statistical analysis of a three-phase analysis and intervention sequence with at least 24 girls and women with RTT. Our study will contribute evidence-based communication intervention guidelines for individuals with RTT by setting a standard of care for individuals with RTT and related complex communication needs, it will lay the foundational scientific understanding of the efficacy of reinforcement-based intervention with this population and others with complex communication needs, and it will demonstrate the effective use of telepresence which will vastly expand capacity to deliver effective care (nationally, internationally) for communication needs to individuals affected by RTT and their families. Our proposal is innovative, in our opinion, because it rejects the supposition that individuals with RTT do not possess the developmental level necessary to acquire intentional communication skills and instead, uses telepresence as a vehicle for reaching families affected by RTT to provide reinforcement-based intervention to develop communication skills and, in turn, improve quality of life.