Frailty phenotype in adults with sickle cell disease

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BACKGROUND: People with sickle cell disease (SCD) are at risk for accelerated biological aging and functional decline due to both age and SCD-related stressors. Frailty is characterized by decreased physiologic reserve and increased vulnerability to stressors that can lead to disability and death. The purpose of this study was to evaluate frailty phenotype in adults with SCD and identify factors associated with frailty. METHODS: Frailty was defined by meeting ≥3 of the following criteria: slowness, weakness, weight loss, low physical activity, and exhaustion. Participants meeting 1-2 criteria were prefrail and "robust" if no criteria were present. RESULTS: We analyzed frailty in 137 adults (age ≥ 18) with SCD in a single-center cross-sectional study (mean age 45; range 19-82). Three (2%) participants were frail, 47% prefrail, and 51% robust. Compared to robust, frail/prefrail participants were more likely to have emergency department visits in the past year (66% vs 46%; p < .02), hypertension (42% vs 19%; p < .003), diabetes (16% vs 4%; p < .02), more SCD complications (2.8 vs 2.1; p < .02), and significantly worse socioeconomic status, cognitive function, depression, physical function, and disability in activities of daily living. CONCLUSIONS: The prevalence of prefrailty in this sample of young and predominantly middle-aged adults with SCD was similar to older adults in the general population, further supporting that accelerated aging occurs in this population and emphasizing the need for early identification and initiation of interventions to prevent and attenuate frailty in SCD. In future studies, we will evaluate SCD-specific cutpoints for frailty components that predict clinically meaningful outcomes.

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