Costs and Impact of Disease in Adults with Sickle Cell Disease: A Pilot Study

Abstract

Background: The Consensus Study report from National Academy of Science, Engineering, Medicine on September 2020 found a lack of data to characterize sickle cell disease (SCD) related disease burden, outcomes, and the unmet needs. This study’s objectives were to 1) assess the feasibility of collecting data to estimate illness burden in adults with SCD; 2) investigate factors associated with health-related quality of life (HRQoL); and 3) estimate the societal burden. Method: We recruited 32 adults aged ≥18 years with a diagnosis of SCD who obtained care at two adult SCD specialty centers in the US. We collected data on fatigue, HRQoL measured by the EQ-5D-3L and the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-Me), and the Work Productivity and Activity Impairment via patient survey. Healthcare utilization was abstracted for the 12 months prior to enrollment using medical chart review. Factors associated with HRQoL scores were assessed by Pearson correlations. Results: Data collection was feasible, although prescription data could not be used to estimate medication costs. Mean age was 36.7±10.6 (standard deviation) years, 84.4% had hemoglobin (Hb)SS/Sbthal0 disease, and 81.3% reported chronic pain (experiencing pain on ≥3 days per week in the past 6 months). Mean EQ-5D VAS was 63.4. The mean EQ-5D index score was 0.79. ASCQ-Me scores are comparable to the referent population of adults with SCD. The mean fatigue score was 57.9 (range 33.7-75.9) and was negatively correlated with the EQ-5D index score (correlation coefficient r=-0.35, p=0.049), and ASCQ-Me scores, including pain (r=-0.47, p=0.006), sleep (r=-0.38, p=0.03), and emotion (r=-0.79, p<0.0001). The number of hospitalizations was negatively correlated with HRQoL (all p<0.05). Patients who reported chronic pain had significantly lower mean ASCQ-Me sleep scores (48.3±10.1 vs. 57.1±9.1, p=0.04) and EQ-5D index scores (0.72±0.21 vs. 0.89±0.09, p=0.002) than those without chronic pain. Mean estimated annual per-person costs were $51,779 (median: $36,366) for total costs, $7,619 ($0) for indirect costs, and $44,160 ($31,873) for medical costs. Conclusions: Fatigue, SCD complications, hospitalization and chronic pain negatively impact HRQoL in this small cohort of US adults with SCD. This sample experienced a high economic burden, largely from outpatient doctor visits.