Prevention of Complications of Thalassemia

描述（由申请人提供）：示范性纽约长老会医院-康奈尔大学威尔医学院（NYPH-WMC）地中海贫血中心是纽约地区最大的项目，为100多名地中海贫血患者及其家属提供护理，旨在促进地中海贫血患者的健康并提高其生活质量。 具体而言，NYPH-WMC旨在提供全面的医疗、心理和遗传服务。 一个多学科的现场诊断、治疗和支持团队，由主任、儿科血液学家、儿科和成人肝病学家、内分泌学家、心脏病学家、放射科医生、生物化学家、遗传学家、社会工作者和心理学家、遗传咨询师、执业护士组成，研究和外展协调员提供全面的预防服务，并参与临床和基础研究，以提高对地中海贫血的认识，管理每两个月进行一次定期输血，每月进行一次化学检查，每季度进行一次评估，每年进行一次评估，包括肝脏、内分泌和心脏评估，进行听力和眼科检查，并监测和监测临床结局。 向当地和区域地中海贫血患者和医疗保健提供者提供咨询性综合医疗保健服务。 还在现场和外展方案中提供文化能力筛查、遗传咨询和产前诊断，以确定那些可以从教育、治疗和预防服务中受益的人，并评估得不到充分服务的人的需求。 与地方和国家消费者组织保持合作，与地中海贫血行动小组、库利贫血基金会和较小的非联邦资助区域机构的地方保健提供者保持合作，这些机构照顾地中海贫血患者较少。 每年的病人/家长教育会议进行和年度医疗更新研讨会举行的医疗保健提供者。 全面的医疗保健服务旨在实现和衡量具体的成果，以预防和减少输血铁超载的并发症，实施疾病预防控制中心的地中海贫血通用数据收集监测计划，以调查疑似血清转化、血源性病原体和可能的细菌污染沿着其他数据收集和监测工作。说明：NYPH-WMC地中海贫血中心与疾病预防控制中心合作的总体目的是通过提供预防评估网络来监测血液安全性和提供全面的医疗保健服务来促进地中海贫血患者的健康和改善他们的生活质量，以监测和预防输血铁过载的并发症，并进行研究，以加强预防和实施预防战略，倡导和促进公共卫生政策的制定。

DESCRIPTION (provided by applicant): The model New York Presbyterian Hospital-Weill Medical College of Cornell University (NYPH-WMC) Thalassemia Center is the largest program in the New York area for the care of over 100 thalassemia patients and their families which aims to promote health and improve the quality of life of thalassemia patients. Specifically, the NYPH-WMC aims to provide comprehensive medical, psychosocial and genetic services. A multidisciplinary on-site diagnostic, treatment and support team comprised of the director, a pediatric hematologist, pediatric and adult hepatologists, endocrinologists, cardiologists, radiologist, biochemist, geneticist, social worker and psychologist, genetic counselor, nurse practitioner, and study and outreach coordinators delivers comprehensive preventive services and participates in clinical and basic research to advance the understanding of thalassemia and improve management. Regular transfusions are administered at bi-monthly visits, monthly chemistries, quarterly evaluations and annual assessments including hepatic, endocrine and cardiac evaluations, hearing and eye exams are performed with surveillance and monitoring of clinical outcomes. Consultative comprehensive healthcare services are provided to the local and regional community of thalassemia patients and healthcare providers. Culturally competent screening, genetic counseling and prenatal diagnosis are also provided on-site and in outreach programs to identify those who can benefit from education, treatment and prevention services as well as to assess the needs of the underserved. Collaborations with local and national consumer organizations are maintained with the Thalassemia Action Group (TAG), the Cooley's Anemia Foundation (CAF) and local healthcare providers at smaller non-federally funded regional institutions which care for fewer thalassemia patients. Annual patient/parent educational conferences are conducted and annual medical update symposiums are held for healthcare providers. Comprehensive healthcare services are directed at attaining and measuring specific outcomes to prevent and reduce complications of transfusional iron overload implementing the CDC's Thalassemia Universal Data Collection Surveillance Program for the investigation of seroconversion suspected, blood borne agents and possible bacterial contamination along with other data collection and surveillance efforts. NARRATIVE: The overall purpose of the NYPH-WMC Thalassemia Center's partnership with the CDC is aimed at promoting the health and improving the quality of life of patients with thalassemia by providing a prevention evaluation network to monitor blood safety and the delivery of comprehensive healthcare services to monitor and prevent the complications of transfusional iron overload, and to conduct research to enhance prevention and implement preventive strategies that will advocate and contribute to the development of public health policies.