The Implementation of Cognitive Screening and Educational Support to Improve Outcomes of Adolescents and Young Adults with Sickle Cell Disease: From Clinic to the Community and Back

项目摘要/摘要 解决教育等因素的政策对健康的影响可能比所有医疗政策都大 进步加在一起。在美国，没有高中文凭的成年人可能会提前九年死亡 而不是大学毕业生。我们的团队显示，一名患有镰状细胞性贫血的15岁男性来自 低收入家庭有70%的风险在学校不及格，这使他面临辍学的风险。高 即使只有一门课不及格的学生，高中辍学的可能性也要高出33%。这些统计数据是 对于10万患有镰状细胞病(SCD)的美国人来说，这是一种慢性溶血性贫血， 会导致器官损伤和过早死亡。超过90%的人口是非裔美国人，60%-70%的人生活在 或接近贫困，并且他们患有与中枢神经系统高风险相关的慢性病 (CNS)损伤。青霉素预防、预防性经颅多普勒筛查等医学进展 长期输血和羟基尿素可降低死亡风险。尽管取得了这些进展，但中位存活率 患有SCD的人只有30-40岁，比非洲人的平均预期寿命少30-45岁 美国人。我们的多学科团队致力于认知功能和教育素养的提高 SCD患者的总体健康状况。一个患有镰状细胞性贫血的孩子，一个沉默的脑梗塞，和一个没有 据预测，受过高中教育的孩子的智商要比没有沉默的孩子低13分(SD=15)。 脑梗塞和受过大学教育的父母。来自NHLBI的2014年循证管理指南指出， 无症状性中枢神经系统梗死可表现为非局灶性体征，如发育迟缓或学习成绩差或下降。 儿童的表现或成人的社会角色或工作功能的变化。在他们的一生中，人们 当评估发现其中任何一种时，应考虑对SCD进行正式的神经认知评估 令人担忧的问题。我们假设，更好地实施这些建议的认知评估和 翻译成明确的学校教育支持计划将增加学生在 教育程度和促进更好的健康。这个应用程序的长期目标是改进 实施循证医学对青少年和成人SCD患者的健康和学业成就的影响 神经认知测试和干预指南。应对镰状细胞病的实施 我们建议以下目标：1)完成以社区为基础的需求评估 青少年和成人基本医疗保健和最佳教育水平的促进者和障碍 与SCD和他们的照顾者一起；2)设计和实施基于我们的 以前的工作重点是高危青少年的认知评估和教育成就 SCD；3)与数据协调处协作，参与开发SCD登记处 中心和NHLBI。共同努力，我们的目标将确立我们的网站能够改善 青少年和成年SCD患者通过发展多模式、多部门的干预措施 提高SCD患者接受循证护理的速度。

Project Summary/Abstract Policies that address factors such as education could have a bigger influence on health than all medical advances combined. Within the U.S., adults without a high school diploma are likely to die nine years sooner than college graduates. Our team showed that a 15-year-old male with sickle cell anemia who comes from a low-income family has a 70% risk of failing a grade in school, putting him at risk of becoming a dropout. High school students who fail even one course are 33% more likely to drop out of high school. These statistics are a stark reality for the 100,000 Americans who have sickle cell disease (SCD), a chronic hemolytic anemia that causes organ damage and early death. Over 90% of this population is African American, 60-70% are living at or near poverty, and they suffer from a chronic disease associated with a high risk of central nervous system (CNS) injury. Medical advances such as penicillin prophylaxis, transcranial Doppler screening with prophylactic chronic transfusion and hydroxyurea reduce the risk of death. Despite these advances, median survival of individuals with SCD remains just 30-40 years, 30-45 years less than the average life expectancy for African Americans. Our multidisciplinary team addresses cognitive function and educational attainment to improve overall health of people with SCD. A child with sickle cell anemia, a silent cerebral infarct, and a parent without a high school education is predicted to have a full-scale IQ 13 points (SD = 15) lower than a child with no silent infarct and a college-educated parent. The 2014 Evidence Based Management guidelines from NHLBI stated, “Silent CNS infarcts can present with non-focal signs such as developmental delays or poor or declining school performance in children or changes in social role or work functioning in adults. Throughout their lives, people with SCD should be considered for formal neurocognitive evaluation when assessments reveal any of these concerns.” We hypothesize that better implementation of these recommended cognitive evaluations and translation to well-defined plans for educational support in schools will increase students’ success in educational attainment and promote better health. The long-term goal of this application is to improve the health and academic attainment of adolescents and adults with SCD by implementing evidence based guidelines for neurocognitive testing and intervention. In response to the Sickle Cell Disease Implementation Consortium RFA, we propose the following aims: 1) To complete a community-based needs assessment of the facilitators and barriers to essential healthcare and optimal educational attainment for adolescents and adults with SCD and their caregivers; 2) To design and implement a novel implementation project based on our previous work that focuses on cognitive evaluation and educational attainment of higher risk adolescents with SCD; 3) To participate in the development of a SCD Registry in collaboration with the Data Coordinating Center and NHLBI. Together, our aims will establish that our site is able to improve health and well-being of adolescents and adults with SCD in through the development of multi-modal, multi-sector interventions aimed at improving the rate at which patients with SCD receive evidence-based care.