Characterizing Frailty in Older Adults with Sickle Cell Disease Using a Sickle Cell Frailty Index (FI-SCD)

患有镰状细胞病(SCD)的人比以往任何时候都活得更长。作为数量的 患有SCD的老年人越来越多，因此更需要数据来确定适当的 关爱这群人。作为SCD年龄的个体，他们表现出大量和早期的 多器官系统恶化，导致老年人常见的并发症 人群，如心肺疾病、感觉障碍、功能衰退和 认知障碍。未能识别漏洞和功能下降可能会导致 残疾、对日常生活活动的依赖以及过早死亡。虚弱是一种综合征 以减少生理储备和增加对导致 到不利的结果。患有SCD的人会经历各种压力源，例如 因血管痉挛疼痛危机和器官损伤而住院；然而，虚弱从来都不是 在患有SCD的人中定义。这项研究的长期目标是了解脆弱性， 老年SCD患者加速衰老、功能衰退，并制定干预措施 改善这些问题。这项研究的目的是描述老年人的虚弱。 使用基于实验室的镰刀细胞疾病脆弱指数(FI-SCD)的SCD和验证的 脆性的测量，油炸脆性表型(Fp)。为了实现这些目标，调查人员 将在一项单中心横断面研究中确定FI-SCD和FP的关系 在100名患有SCD的老年人中(定义为40岁及以上)。对于第一个目标，调查人员 使用与时间顺序老化相关的常规实验室变量开发了脆弱指数 以及SCD和老年病的死亡率。脆弱性指数得分的计算方法是 健康赤字，并除以评估的变量总数。调查人员将 比较FI-SCD和FP，使用相关系数和ROC曲线描述敏感度 FI-SCD对FP的特异性。对于第二个目标，调查人员将确定相关因素 通过比较老年SCD患者的虚弱分数和前肌疼痛强度， 刺激疼痛干扰、SCD基因型、SCD并发症和非SCD并存。 与FI-SCD和FP相关的因素将使用典型相关来确定。发展中 老年SCD患者的脆弱指数将提供一种早期风险分层的方法 并将创建一个框架，以制定干预措施，以预防脆弱，改善 功能，并降低死亡率。这项研究与美国国家老龄研究所的 通过生物医学研究改善美国老年人的健康和福祉的使命。

Individuals with sickle cell disease (SCD) are living longer than ever before. As the number of older adults living with SCD grows, there is a greater need for data to determine the appropriate care for this population. As individuals with SCD age, they demonstrate substantial and early deterioration of multiple organ systems, which causes complications often seen in geriatric populations, such as cardiopulmonary disease, sensory impairment, functional decline, and cognitive impairment. Failure to recognize vulnerabilities and functional decline can lead to disability, dependence in activities of daily living, and premature death. Frailty is a syndrome characterized by decreased physiologic reserve and increased vulnerability to stressors that lead to adverse outcomes. Individuals with SCD experience a variety of stressors, such as hospitalizations for vasooclussive pain crises and organ damage; however, frailty has never been defined in people with SCD. The long-term objective of this research is to understand frailty, accelerated aging, functional decline in older adults with SCD and develop interventions to ameliorate these issues. The purpose of this study is to characterize frailty in older adults with SCD using a laboratory-based Sickle Cell Disease Frailty Index (FI-SCD) and a validated measure of frailty, the Fried Frailty Phenotype (FP). To achieve these goals, the investigators will determine the relationship between FI-SCD and FP in a single-center cross-sectional study of 100 older adults with SCD (defined as age 40 and older). For the first aim, the investigators developed a frailty index using routine laboratory variables associated with chronological aging and mortality in SCD and geriatrics. A frailty index score will be calculated by taking the sum of heath deficits and dividing that by the total number of variables evaluated. The investigators will compare the FI-SCD to FP, using correlation coefficient and ROC curves to describe sensitivity and specificity of FI-SCD for FP. For the second aim, investigators will identify factors associated with frailty in older adults with SCD by comparing frailty scores to PROMIS Pain Intensity, PROMIS Pain Interference, SCD genotype, SCD-complications, and non-SCD comorbidities. Factors associated with FI-SCD and FP will be identified using canonical correlation. Developing a frailty index for older adults with SCD will provide a method for early risk stratification of this population and will create a framework for developing interventions to prevent frailty, improve function, and reduce mortality. This research is consistent with the National Institute of Aging’s mission of improving the health and well-being of older Americans through biomedical research.