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。的数量 随着SCD老年人的增长，更需要数据来确定适当的 照顾这个人口。随着SCD患者年龄的增长，他们表现出大量的早期 多个器官系统的恶化，导致老年人常见的并发症 人群，如心肺疾病，感觉障碍，功能下降， 认知障碍未能认识到脆弱性和功能衰退可能导致 残疾、日常生活活动依赖和过早死亡。脆弱是一种综合症 其特征是生理储备减少和对导致 到不利的结果。SCD患者会经历各种压力源，例如 住院治疗血管闭塞性疼痛危象和器官损伤;然而， 在SCD患者中的定义。这项研究的长期目标是了解脆弱， 加速衰老，功能衰退，并制定干预措施， 改善这些问题。本研究的目的是描述老年人的脆弱性， SCD使用基于实验室的镰状细胞病脆弱指数（FI-SCD）和经验证的 油炸脆弱表型（Fried Frailty Phenotype，FP）为了实现这些目标，研究人员 将在单中心横断面研究中确定FI-SCD和FP之间的关系 100名患有SCD的老年人（定义为40岁及以上）。为了达到第一个目标，研究人员 使用与实足年龄相关的常规实验室变量开发了一个虚弱指数 和老年病的死亡率。虚弱指数评分将通过以下各项的总和计算： 健康缺陷并除以评估的变量总数。调查人员将 比较FI-SCD与FP，用相关系数和ROC曲线描述敏感性 和特异性。对于第二个目标，研究人员将确定相关因素， 通过比较虚弱评分与PROMIS疼痛强度， PROMIS疼痛干扰、SCD基因型、SCD并发症和非SCD合并症。 将使用典型相关来确定与FI-SCD和FP相关的因素。发展中 老年SCD患者的虚弱指数将为这种疾病的早期风险分层提供一种方法。 并将建立一个框架，制定干预措施，以防止脆弱，改善 功能，降低死亡率。这项研究与国家老龄化研究所的研究结果一致。 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.