Effectiveness of Expiratory Muscle Strength Training for Improving Communication in ALS

项目摘要 拟议项目的目标是为Brianna基弗女士提供在普渡大学接受培训的机会， 哥伦比亚大学演讲专家Jessica Huber、Michelle Troche和Kristine Marceau博士 科学、呼吸、咳嗽生理学和多级建模。为了实现这一目标，基弗女士将进行一次 肌萎缩侧索硬化症（ALS）患者呼气肌力训练（埃姆斯特）的研究 进行性肌萎缩是与ALS相关的运动神经元死亡的有害后果。 呼吸肌组织的萎缩尤其有害，因为说话和咳嗽需要大量的呼吸肌组织。 和协调的呼吸支持由于呼吸肌萎缩，ALS患者无法 产生足够的声门下压力来支持说话。患有ALS的人也无法产生强大的 当他们咳嗽时，呼气力有效地将异物从气道排出。因此，人们 ALS患者通常依赖替代通信设备和替代营养方法。失去 独立说话和进食的能力显著降低了ALS患者的生活质量。最近 研究发现，在早期阶段实施中等强度力量训练模式 是安全有效的。埃姆斯特是一种常用的加强呼吸肌组织的范例 改善语言和咳嗽。虽然埃姆斯特有大量文献支持其用于 对于患有其他神经退行性疾病的人，只有一项研究检查了埃姆斯特对 ALS患者拟议的项目旨在建立在Plowman和同事（2016）的研究结果，以解决 三大目标。具体目标1将定义基础呼吸支持模式如何介导 语言和咳嗽产生被ALS改变。我们怀疑患有ALS的人会利用更少的 与年龄相关的言语和咳嗽产生期间的高效和有效呼吸支持模式-以及 性别匹配的对照组。具体目标2将确定中等强度埃姆斯特如何改变 有效性和效率的讲话和咳嗽生产。在多基线延迟治疗中， 设计，ALS患者将接受为期六周的中等强度埃姆斯特方案。我们怀疑埃姆斯特 将提高说话和咳嗽产生的有效性和效率。最后，具体目标3将 确定埃姆斯特如何影响ALS患者的心理社会状态。我们怀疑语言能力的提高 咳嗽会对肌萎缩侧索硬化症患者的社会心理状态产生积极影响。共同的结果， 拟议的研究将提供急需的信息，以指导临床实践，在治疗 构音障碍和肌萎缩侧索硬化相关的味觉障碍。通过进行这项研究，并与她的导师在 各种各样的活动，基弗女士将大大扩展她的研究技能。具体来说，她将获得经验 ALS，反射性咳嗽产生，呼吸功能，多层次模型和临床试验管理。

PROJECT ABSTRACT The goal of the proposed project is to provide Ms. Brianna Kiefer with training opportunities at Purdue and Columbia University from Drs. Jessica Huber, Michelle Troche, and Kristine Marceau – experts in speech science, respiration, cough physiology, and multilevel modeling. To achieve this goal, Ms. Kiefer will conduct a study of expiratory muscle strength training (EMST) in people with Amyotrophic Lateral Sclerosis (ALS). Progressive muscular atrophy is a detrimental consequence of motor neuronal death associated with ALS. Atrophy of the respiratory musculature is particularly harmful since speech and cough production require ample and coordinated respiratory support. Due to respiratory musculature atrophy, people with ALS are unable to generate sufficient subglottal pressures to support speech. People with ALS are also unable to generate strong expiratory forces to effectively expel foreign materials from the airway when they cough. Consequently, people with ALS often rely on alternative communication devices and alternative methods of nutrition. Losing the abilities to speak and eat independently significantly reduces quality of life for people with ALS. Recent investigations have found that implementing moderate intensity strength training paradigms in the early stages of ALS is safe and effective. EMST is a commonly used paradigm that strengthens the respiratory musculature to improve speech and cough production. While EMST has a large body of literature to support its use for people with other neurodegenerative diseases, only one study has examined the effectiveness of EMST for people with ALS. The proposed project aims to build upon Plowman and colleagues (2016) findings to address three broad aims. Specific aim 1 will define how the underlying respiratory support patterns mediating speech and cough production are altered by ALS. We suspect that individuals with ALS will utilize less efficient and effective respiratory support patterns during speech and cough production relative to age- and sex-matched controls. Specific Aim 2 will determine how moderate intensity EMST modifies the effectiveness and efficiency of speech and cough production. In a multiple baseline, delayed-treatment design, people with ALS will undergo a six-week moderate intensity EMST protocol. We suspect that EMST will improve the effectiveness and efficiency of speech and cough production. And finally, Specific Aim 3 will identify how EMST affects the psychosocial states of people with ALS. We suspect that improved speech and cough production will positively impact the psychosocial states of people with ALS. Together, results from the proposed study will provide much needed information to guide clinical practice in the treatment of dysarthria and dystussia associated with ALS. Through conducting this study and engaging with her mentors in a variety of activities, Ms. Kiefer will greatly expand her research skills. Specifically, she will gain experience with ALS, reflexive cough production, respiratory function, multilevel models, and clinical trial management.