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

Limited data are available to characterize sickle cell disease (SCD) related disease burden and outcomes. We assessed the feasibility of collecting data to estimate illness burden in adults with SCD; investigated factors associated with health-related quality of life (HRQoL); and estimated societal burden. We recruited 32 adults with SCD. We collected data on fatigue, HRQoL 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. Mean (standard deviation) age was 36.7 (10.6) years, 84.4% had hemoglobin (Hb)SS/Sthal0 disease, and 81.3% reported chronic pain (experiencing pain on ≥3 days per week in the past 6 months). Mean EQ-5D-3L VAS was 63.4, index score was 0.79. The mean fatigue score was 57.9 (range 33.7-75.9). Higher fatigue score was correlated with lower 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 vs. 57.1, p=0.04) and EQ-5D index scores (0.72 vs. 0.89, 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 (estimated from lost earnings of participants), and $44,160 ($31,873) for medical costs. Fatigue, SCD complications, hospitalization and chronic pain negatively impact HRQoL in this cohort. This sample experienced a high economic burden, largely from outpatient doctor visits.

Living Profiles: design of a health media platform for teens with special healthcare needs.

Living Profiles is a health media platform in development that aggregates multiple data flows to help teens with special healthcare needs (SHCN), particularly with regard to self-management and independence. A teen-oriented personal health record (PHR) incorporates typical teen behaviors and attitudes about health and wellness, encompasses how teens perceive and convey quality of life, and aligns with data related to their chronic medical condition. We have conceived a secure personalized user interface called the Quality of Life Timeline, which will assist with the transition from pediatric care to an adult provider through modules that include a mood meter, reminder device, and teleport medicine. With this personalized PHR, teens with SHCN can better understand their condition and its effects on daily activities and life goals and vice versa; additionally, use of this PHR allows for better information sharing and communication between providers and patients. The use of a teen-oriented tool such as Living Profiles can impact teens' overall quality of life and disease self-management, important attributes for a successful transition program.