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