Woodward CDC NSBPR Registry 2018

项目概要/摘要 脊柱裂是最常见的复杂先天性疾病，对健康和质量有重大影响 生命虽然脊柱裂患者的预期寿命有所提高，但他们仍然面临着高风险。 发病率高，社区参与率低，医疗保健费用高，有些人报告的发病率较低， 生活质量多学科脊柱裂诊所提供全面，协调，以家庭为中心，最好的 优质护理，目标是促进最高水平的健康，生活质量，独立性和社区 参与整个生命周期。高质量的证据，以指导这些诊所和其他提供者提供 最有效的临床护理是有限的，并且诊所之间的护理实践存在很大差异。因此，我们认为， 迫切需要确定和传播基于证据的标准化临床护理实践， 导致早期识别常见并发症，及时提供具有成本效益的干预措施， 最佳的结果和整个生命周期的生活质量。国家脊柱裂患者登记处（NSBPR） 由疾病控制和预防中心于2008年建立，以确定和传播最佳 通过纵向数据收集、分析和传播调查结果，对护理做法进行评估。然而，变异 在多学科诊所中仍然存在护理和健康结果。该提案旨在推进 疾病预防控制中心的国家出生缺陷和发育障碍中心的使命是改善儿童的健康， 通过促进残疾儿童和成年人的健康和福祉，为儿童和成年人提供保健服务。客观 该提案的目的是通过改善脊柱裂临床护理， NSBPR纵向数据收集和分析以及NSBPR与其他电子健康记录的链接 (EHR)临床和管理数据。我们的目标是：1）确定与最佳治疗相关的临床护理实践 通过将NSBPR收集范围扩大到成年人， 标准化的数据收集程序，并增加NSBPR和EHR数据之间的联系。我们将（2） 通过利用我们实施标准化护理算法的经验和我们的 NSBPR/EHR数据链接，以表征睡眠呼吸障碍（SDB）的频率， 脊柱裂患者SDB相关症状的频率。我们还打算评估 由跨学科团队实施的标准化手动肌肉测试护理算法的潜力， 在5岁时预测Ambassador的第一年。通过研究上述目标，我们的团队准备 提高跨多学科诊所的NSBPR数据的质量和利用率，目的是 确定最佳临床护理实践，预防并发症，促进最佳健康，功能 独立性，社区参与，以及脊柱裂儿童和成人的生活质量。

PROJECT SUMMARY/ABSTRACT Spina bifida is the most common complex congenital disorder and has significant impact on health and quality of life. Although life expectancy has improved for individuals with spina bifida, they continue to experience high rates of morbidity, low rates of community participation, high health care costs, and for some, lower reported quality of life. Multidisciplinary spina bifida clinics provide comprehensive, coordinated, family-centered, best quality care with the goal of promoting the highest level of health, quality of life, independence, and community participation across the lifespan. High quality evidence to guide these clinics and other providers in providing the most effective clinical care is limited and a wide variation in care practices exists among clinics. Therefore, there is a critical need to identify and disseminate evidence-based, standardized clinical care practices that result in early identification of common complications, timely delivery of cost-effective interventions, and optimal outcomes and quality of life across the lifespan. The National Spina Bifida Patient Registry (NSBPR) was established by the Centers for Disease Control and Prevention in 2008 to pinpoint and disseminate best care practices through longitudinal data collection, analysis, and dissemination of findings. However, variation in care and health outcomes still exist across multidisciplinary clinics. This proposal aims to advance the mission of the CDC’s National Center on Birth Defects and Developmental Disabilities to improve the health of children and adults by promoting health and wellness among children and adults with disabilities. The objective of this proposal is to identify and disseminate best practices in spina bifida clinical care through improved NSBPR longitudinal data collection and analysis and linkage of NSBPR with additional electronic health record (EHR) clinical and administrative data. We aim to 1) identify the clinical care practices associated with best health outcomes for people living with spina bifida by expanding NSBPR collection to adults, developing standardized data collection procedures, and increasing linkages between NSBPR and EHR data. We will 2) advance the evidence base by leveraging our experience implementing standardized care algorithms and our NSBPR/EHR data linkages to characterize the frequency of sleep disordered breathing (SDB) and the frequency of associated symptoms of SDB in individuals with spina bifida. We also aim to 3) evaluate the potential of a standardized manual muscle testing care algorithm implemented by an interdisciplinary team in the first year of life to predict ambulation at age 5. By investigating the above aims, our team is poised to improve the quality and utilization of the NSBPR data across multidisciplinary clinics with the purpose of identifying best clinical care practices that prevent complications and promote optimal health, functional independence, community participation, and quality of life for children and adults with spina bifida.