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

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.

Characterising the prevalence of overweight and obese status among adults with sickle cell disease

“…Participants in that study also had a saturated fat and sugar intake that exceeded the national recommended daily allowance. This risk of metabolic syndrome and ischaemic heart disease has serious clinical implications, which requires more attention as adults with SCD already have an increased risk of cardiopulmonary disease, renal disease, and functional decline that increases with age 13,38,39 . Increasing BMI with age in adults without SCD is associated with a higher pulmonary artery systolic pressure by middle age 40 .…”

“…This risk of metabolic syndrome and ischaemic heart disease has serious clinical implications, which requires more attention as adults with SCD already have an increased risk of cardiopulmonary disease, renal disease, and functional decline that increases with age. 13,38,39 Increasing BMI with age in adults without SCD is associated with a higher pulmonary artery systolic pressure by middle age. 40 Although, tricuspid regurgitant jet velocity (TRV) was not measured in our study, it is important to note that obesity can influence TRV by potentially falsely elevating it; therefore, clinicians should be cautious when interpreting TRV when screening for pulmonary HTN by echocardiogram in obese patients with SCD.…”

Malnutrition in sickle cell anemia: Prevalence, impact, and interventions: A Review

“…A comprehensive screening tool must also account for the potential impact of coexisting conditions on nutritional status, acknowledging the complexity of healthcare needs in individuals with SCD. [67] Given that SCD affects both children and adults, an effective nutrition screening tool should be adaptable across different age groups. [67] Children may have distinct nutritional requirements due to growth and development, while adults may face challenges related to chronic pain and increased nutrient demands during crises.…”

“…[67] Given that SCD affects both children and adults, an effective nutrition screening tool should be adaptable across different age groups. [67] Children may have distinct nutritional requirements due to growth and development, while adults may face challenges related to chronic pain and increased nutrient demands during crises. A tool that considers these age-specific factors ensures a more accurate assessment of nutritional status and facilitates targeted interventions tailored to the unique needs of each population.…”

“…A comprehensive screening tool must also account for the potential impact of coexisting conditions on nutritional status, acknowledging the complexity of healthcare needs in individuals with SCD. [ 67 ]…”