Woodward CDC NSBPR Registry 2018

项目摘要/摘要 脊柱裂是最常见的复杂先天性疾病，严重影响人们的健康和生活质量 生活的一部分。尽管脊柱裂患者的预期寿命有所改善，但他们的寿命仍然很高。 发病率低，社区参与率低，医疗保健费用高，对一些人来说，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、以家庭为中心的 高质量的护理，目标是促进最高水平的健康、生活质量、独立性和社区 在整个生命周期中参与。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，不同诊所的护理做法也存在很大差异。因此， 迫切需要确定和传播以证据为基础的标准化临床护理实践， 导致及早发现常见并发症，及时提供具有成本效益的干预措施，以及 在整个生命周期中实现最佳结果和生活质量。全国脊柱裂患者登记(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、分析和结果传播进行护理实践。然而，变化 在护理和健康方面的结果仍然存在于多学科诊所。这项建议旨在推进 美国疾病控制与预防中心国家出生缺陷和发育障碍中心改善儿童健康的使命 通过促进残疾儿童和成人的健康和健康，促进儿童和成人的健康。目标是 这项建议的目的是确定和传播脊柱裂临床护理的最佳做法，通过改进 NSBPR纵向数据收集和分析以及NSBPR与附加电子健康记录的关联 (电子病历)临床和管理数据。我们的目标是1)确定与最佳 通过将NSBPR收集扩大到成年人，开发出 标准化的数据收集程序，并加强NSBPR和EHR数据之间的联系。我们会的 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障碍呼吸(SDB)的频率和 脊柱裂患者伴发SDB症状的频率。我们的目标还包括评估 由一个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岁时预测行走能力的第一年。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，以预防并发症并促进最佳健康、功能 脊柱裂儿童和成人的独立性、社区参与和生活质量。

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.