Prevention of Complications of Thalassemia

摘要： 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治疗100多例地中海贫血的最大项目 旨在促进地中海贫血患者健康和提高生活质量的患者及其家人 病人。具体地说，NYPH-WMC的目标是提供全面的医疗、心理和社会保护 基因服务。多学科现场诊断、治疗和支持小组，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，护士从业者， 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，改善管理。 每两个月进行一次定期输血，每月进行一次化验，每季度进行一次评估， 年度评估包括肝脏、内分泌和心脏评估、听力和眼睛检查 对临床结果进行监测和监测。咨询式全面医疗保健 向当地和地区的地中海贫血患者和医疗保健社区提供服务 供应商。还提供有文化能力的筛查、遗传咨询和产前诊断。 现场和外展方案，以确定哪些人可以从教育、治疗和 预防服务以及评估未得到充分服务的人的需求。与当地和 国家消费者组织由地中海贫血行动小组(TAG)、库利的 贫血基金会(CAF)和小型非联邦资助地区的当地医疗保健提供者 照顾较少地中海贫血患者的机构。一年一度的患者/家长教育会议 为医疗保健提供者举办和每年一次的医疗更新研讨会。全面 医疗保健服务旨在实现和衡量特定的结果，以预防和减少 输血铁超载实施疾控中心地中海贫血通用数据的并发症 用于调查疑似血清转换、血液传播病原体的采集监测计划 以及可能的细菌污染以及其他数据收集和监测工作。叙述： 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 通过为地中海贫血患者提供一种促进健康和提高生活质量的方法 预防评估网络，以监测血液安全和提供全面的医疗保健 监测和预防输血铁超载并发症的服务，并进行研究 加强预防和实施预防战略，以倡导和促进 制定公共卫生政策。

ABSTRACT: 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.