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)患者的损害及处理 疼痛对患者、家庭和医疗保健来说是一个持续的挑战 供应商。医疗保健提供者对以下问题几乎没有达成共识 治疗SCD疼痛的最佳药理学和非药理学方法 管理层。此外，VOC发作及其医疗管理可以 产生生理和药理后遗症，可 对病人不利。相反，对VOC的优化管理 这些事件可能会有短期和长期的好处 有耐心的。该计划由三项综合临床研究组成 旨在解决VOC事件的最佳管理的研究 心理学、药理学和生理学的观点。 本项目的三个具体目标是：1)进行随机的 以家庭为基础的疼痛管理有效性的临床试验 旨在向儿童SCD患者及其初级患者提供教育的计划 护理者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 确定益处和风险的双盲随机临床试验 哌醋甲酯辅助治疗重度VOC疼痛的临床观察 住院患者；以及3)进行随机试验 激励性肺活量计在血管闭塞发作中的门诊应用 以确定使用此设置是否会阻止开发 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).