Sickle Cell Disease Functional Assessment Validation and Exercise Pilot Intervention - 1 PROJECT SUMMARY/ABSTRACT 2 Despite improvements in median life expectancy for sickle cell disease (SCD) from 14 years in 3 1973 to 61 years in recent cohorts from academic centers, adults with SCD experience 4 functional impairments. They develop deficits in physical, social, and cognitive function due to 5 both age-related changes and SCD complications, such as avascular necrosis, strokes, 6 cardiopulmonary disease, and sensory deficits. There are no validated assessment tools or 7 interventions to improve physical function in older adults with SCD. Failure to recognize and 8 manage vulnerabilities and functional decline can lead to disability and premature death. In a 9 pilot study we evaluated the feasibility if the Sickle Cell Disease Functional Assessment (SCD- 10 FA) and demonstrated that it was feasible, acceptable, and safe. In this study, we will evaluate 11 the validity and reliability of the SCD-FA in a prospective cohort of older adults with SCD (age ≥ 12 40 years) (Aim 1). We will evaluate the utility of the SCD-FA to predict number of days 13 hospitalized per year as the primary outcome, and Sickle Cell Day Hospital visits, emergency 14 department visits, dependence in Activities of Daily Living, and death as secondary outcomes. 15 We will also determine risk factors for impaired physical performance (Aim 2). The SCD-FA will 16 provide a method for: 1) assessing vulnerabilities in multiple health domains, 2) identifying 17 targets for interventions, and 3) measuring change in function. To address functional 18 impairments, we will adapt and pilot a personalized exercise training program in older adults 19 (age ≥ 40 years) with SCD (Aim 3). The intervention will be a virtual program adapted from 20 Gerofit, which is a supervised exercise and health promotion program for older adults that 21 includes exercises customized to each person’s health status and underlying functional deficits. 22 The expected outcome is to have pilot data to design a randomized control trial of a 23 personalized exercise intervention for older adults with SCD. 24 Dr. Oyedeji is an Assistant Professor of Medicine in the Division of Hematology at Duke 25 University. Her career development plan includes developing skills in health assessment tool 26 development and developing and implementing interventions with guidance from experts in 27 psychometrics, intervention development, implementation, geriatrics, and sickle cell disease. 28 This research coupled with coursework, professional development activities, and her mentorship 29 team will provide the necessary experience to support her success as she works towards her 30 goal of becoming an independent investigator and leader in SCD and aging.
