The Sickle Cell Disease Functional Assessment (SCD-FA) tool: a feasibility pilot study

Charity I Oyedeji, Katherine Hall, Alison Luciano, Miriam C Morey, John J Strouse, Charity I Oyedeji, Katherine Hall, Alison Luciano, Miriam C Morey, John J Strouse

Abstract

Background: The life expectancy for individuals with sickle cell disease (SCD) has greatly increased over the last 50 years. Adults with SCD experience multiple complications such as cardiopulmonary disease, strokes, and avascular necrosis that lead to limitations that geriatric populations often experience. There are no dedicated instruments to measure functional decline and functional age to determine risk of future adverse outcomes in older adults with SCD. The objective of this study was to assess the feasibility of performing the Sickle Cell Disease Functional Assessment (SCD-FA).

Methods: We enrolled 40 adults with SCD (20 younger adults aged 18-49 years as a comparison group and 20 older adults aged 50 years and older) in a single-center prospective cohort study. Participants were recruited from a comprehensive sickle cell clinic in an academic center in the southeastern United States. We included measures validated in an oncology geriatric assessment enriched with additional physical performance measures: usual gait speed, seated grip strength, Timed Up and Go, six-minute walk test, and 30-second chair stand. We also included an additional cognitive measure, which was the Montreal Cognitive Assessment, and additional patient-reported measures at the intersection of sickle cell disease and geriatrics. The primary outcome was the proportion completing the assessment. Secondary outcomes were the proportion consenting, duration of the assessment, acceptability, and adverse events.

Results: Eighty percent (44/55) of individuals approached consented, 91% (40/44) completed the SCD-FA in its entirety, and the median duration was 89 min (IQR 80-98). There were no identified adverse events. On the acceptability survey, 95% (38/40) reported the length as appropriate, 2.5% (1/40) reported a question as upsetting, and 5% (2/40) reported portions as difficult. Exploratory analyses of physical function showed 63% (25/40) had a slow usual gait speed (< 1.2 m/s).

Conclusion: The SCD-FA is feasible, acceptable, and safe and physical performance tests identified functional impairments in adults with SCD. These findings will inform the next phase of the study where we will assess the validity of the SCD-FA to predict patient-important outcomes in a larger sample of adults with SCD.

Keywords: Aging; Frailty; Functional assessment; Gait speed; Geriatric assessment; Geriatrics; Older adults; Sickle cell disease.

Conflict of interest statement

C.I.O., A.L., K.H., and M.M. declare no competing financial interests. J.S. serves as site PI for a clinical trial of ADAMTS13 for the treatment of SCD (Takeda).

© 2022. The Author(s).

Figures

Fig. 1
Fig. 1
Consort diagram. We approached 55 participants for the study, consented 44, removed 4 due to multiple missed study visits, and performed the Sickle Cell Disease Functional Assessment in 20 younger adults and 20 older adults. Reasons participants gave for declining to participate are listed
Fig. 2
Fig. 2
Usual gait speed (meters/second) by age and gender among individuals with sickle cell disease The figure displays the fastest of 2 usual gait speed trials stratified by age decade. The mean gait speed for all participants was 1.12 m/s (95% CI 1.06, 1.18). The majority of participants (63%) had a gait speed slower than 1.2 m/s (the speed necessary to safely cross the street at an intersection). Twenty-five percent had a gait speed slower than 1 m/s
Fig. 3
Fig. 3
Maximum seated grip strength by age group and gender in individuals with sickle cell disease. Boxplot of maximum seated grip strength of 3 trials on each hand. Mean grip strength by age group and gender is also represented by the star on each boxplot and compared to mean maximum grip strength normative values by age and gender in the United States published by Hanten et al. [35]

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