MANAGEMENT OF SICKLE CELL VASO-0CCLUSIVE EPISODES

血管闭塞（VOC）发作是发病率的主要来源， 镰状细胞病（SCD）患者的损害，以及管理 疼痛是对患者、家庭和医疗保健的持续挑战 提供商医疗保健提供者之间几乎没有共识， 治疗SCD疼痛的最佳药物和非药物方法 管理此外，VOC发作及其医疗管理可以 产生生理和药理学后遗症， 对患者有害。相反，VOC的优化管理 事件可能会有短期以及长期的好处， 病人该计划包括三个综合临床研究 研究解决挥发性有机化合物事件的最佳管理， 心理学、药理学和生理学观点。 该项目的三个具体目标是：1）进行随机 临床试验，以测试以家庭为基础的疼痛管理的疗效 旨在教导患有SCD的儿科患者及其主要 护理人员如何通过使用以下组合来最佳地控制疼痛 药理学和非药理学策略; 2）进行 确定获益和风险的双盲随机临床试验 哌醋甲酯辅助治疗重度VOC疼痛 住院患者;和3）进行随机试验， 在血管闭塞发作期间门诊使用激励性肺量计 以确定使用此设置是否会阻止 急性胸部综合征（ACS）。

Vaso-occlusive (VOC) episodes represent the major source of morbidity and impairment for patients with sickle cell disease (SCD), and the management of pain is a continued challenge to patients, families, and health care providers. There is little consensus among medical care providers about the optimal pharmacological and non-pharmacological approaches to SCD pain management. In addition, VOC episodes and its medical management can produce physiological and pharmacological sequelae which can be detrimental to the patients. Conversely, optimal management of the VOC episodes is likely to have short-term as well as long term benefits of the patient. This program consists of three integrated clinical research studies which address the optimal management of VOC episodes from psychological, pharmacological and physiological perspectives. The three specific aims of this project are: 1) to conduct a randomized clinical trial to test the efficacy of a family-based pain management program designed to teach pediatric patients with SCD and their primary caregivers how to optimally control pain by using a combination of pharmacological and non-pharmacological strategies; 2) to conduct a double-blind randomized clinical trial to determine the benefits and risks of adjuvant use of methylphenidate in the treatment of severe VOC pain in hospitalized patients; and 3) to conduct a randomized trail of the outpatient use of the incentive spirometer during vaso-occlusive episodes to determine if its use this setting will prevent the development of the acute chest syndrome (ACS).