Developing an Intervention Model for Complex Communication Needs in Rett Syndrome

项目摘要/摘要 尽管对Rett综合征(RTT)的遗传学研究取得了深入的关注和进展，但 仍然是一个明显缺乏指导针对通信的应用干预措施的证据基础。我们的长- 学期目标是制定改善沟通结果和生活质量的最佳实践 帮助患有RTT和其他类似障碍的患者，克服提供服务的障碍。的目标 本研究旨在开发一种基于强化的干预模式，以提高学生的交际能力 RTT女孩和妇女的结果和生活质量指标，并通过以下方式扩大我们的影响 使用网真覆盖比其他方式可行的更多的女孩和妇女。穿过 我们的初步研究发现，这些目标是基于这样一个中心假设，即女孩和 患有RTT的女性有沟通的能力，她们会使用特殊的行为来 在没有系统干预的情况下进行沟通。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)开发基于强化的干预模式，以解决 RTT中的复杂通信需求和(2)评估作为创新服务交付的网真 在RTT中支持/提供通信干预的机制。我们将通过以下方式实现这些目标 严格的受试者内试验设计和分组统计分析的三阶段分析和 对至少24名患有RTT的女孩和妇女进行干预。我们的研究将有助于以证据为基础 针对RTT患者的沟通干预指南，为患有RTT的患者设定护理标准 RTT及其相关的复杂通信需求，将为我们科学地理解 以强化为基础的干预对这一人群和其他具有复杂沟通的人群的效果 它将展示对网真的有效利用，这将极大地扩展交付能力 有效照顾(国家、国际)受RTT影响的个人的沟通需求 家人。在我们看来，我们的建议是创新的，因为它拒绝了RTT患者 不具备获得有意沟通技能所需的发展水平，相反， 使用网真作为一种工具，帮助受RTT影响的家庭提供基于增援的 进行干预以发展沟通技能，进而提高生活质量。

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.