<p>Patient-Reported Outcomes and Economic Burden of Adults with Sickle Cell Disease in the United States: A Systematic Review</p>

Abstract

Purpose: To systematically estimate the patient-reported outcomes (PROs) and economic burden of sickle cell disease (SCD) among adults in the United States (US). Patients and Methods: Two systematic literature reviews (SLRs), one each for the PROs and economic topics, were performed using MEDLINE and Embase to identify observational studies of adults with SCD. Included studies were published between 2007 and 2018 and evaluated health-related quality of life (HRQL), function, healthcare resource utilization (HCRU), or costs. Given the high degree of clinical and methodological heterogeneity, findings were summarized qualitatively. Results: The SLRs identified 7 studies evaluating the PROs and 15 studies evaluating the economic burden meeting the pre-specified selection criteria. The PRO evidence showed the prevalence of depression and anxiety to be 21-33% and 7-36%, respectively, in adults with SCD. The mean SF-36 physical summary scores ranged from 33.6 to 59.0 and from 46.3 to 61.5 for the mental summary scores. Overall HRQL for adults with SCD was poor and significantly worse in those with opioid use. Adult SCD patients were found to have varying rates of emergency department (ED) utilization (0.3-3.5 annual ED visits), hospitalizations (0.5-27.9 per patient per year), and/or readmission (12-41%). Key factors associated with significant HCRU were age, dental infection, and SCD-related complications. SCD specialized care settings and SCD intensive management strategy were reported to significantly decrease the number of hospitalizations. Conclusion: This systematic evidence synthesis found that disease burden measured by PROs and economic burden of SCD on adults in the US are substantial despite the availability of approved SCD treatments during 2007-2018. The use of hydroxyurea, optimal management with opioids, and employing intensive treatment strategies may help decrease the overall burden to patients and healthcare systems. Published data on costs associated with SCD are limited and highlight the need for more economic studies to characterize the full burden of the disease.

The Prevalence of Depression and Anxiety Among Sickle Cell Disease Patients in King Abdulaziz University Hospital

“…These findings are conflict with the results conducted by Tabuk and Gana's study that showed the rate of depression is higher in females [11,21]. Similarly, the results reported by the Southern Region of Saudi Arabia also show the rate of depression and anxiety is higher in females [19]. However, these results failed to indicate any statistical significance in our study.…”

“…This study aimed to determine the prevalence and identify the relationship of sociodemographics with depression and anxiety of SCD patients at King Abdulaziz University Hospital, Jeddah, Saudi Arabia. Other researches found that depressed patients will have significantly higher health care utilization and inpatient costs than nondepressed patients [17][18][19]. So determining the risk factors that increase a psychological disorder in SCD patients will be a chance to decrease the cost and provide much better medical care to these patients.…”

Effects of Music Therapy on Quality of Life in Adults with Sickle Cell Disease (MUSIQOLS): A Mixed Methods Feasibility Study

“…4 A recent systematic review found depression and anxiety occurrence to be 21-33% and 7-36%, respectively, in adults with SCD. 5 Higher pain is also strongly associated with decreased work and school activity, social activity, and household chores. 6 Thus, new interventions are needed to improve the overall quality of life of patients with SCD in addition to pain outcomes.…”

Improving Outcomes for Patients With Sickle Cell Disease in the United States

“…6 Additionally, the disease burden to the patient imparts a shortened lifespan and the loss of potential income, in addition to a substantial detriment to the quality of life. 7,8 Despite the substantial personal and societal burden of SCD, compared with other genetic disorders SCD has received relatively little attention and minimal resources since it was first described in 1910. Investments in discoveries and clinical care infrastructure have been limited and have not kept pace with other equally devastating but less common inherited conditions.…”