The Economic Burden and Impact on HRQOL of Sickle Cell Disease

Laura Joszt, MA
Laura Joszt, MA

Laura is the editorial director of The American Journal of Managed Care® (AJMC®) and all its brands, including The American Journal of Accountable Care®, Evidence-Based Oncology™, and The Center for Biosimilars®. She has been working on AJMC® since 2014 and has been with AJMC®'s parent company, MJH Life Sciences, since 2011. She has an MA in business and economic reporting from New York University.

Patients with sickle cell disease face substantial health care utilization and impaired health-related quality of life (HRQOL).

The economic burden of sickle cell disease (SCD) is substantial: patients need routine and lifelong care, and the disease’s complications can be severe and fatal. These patients experience episodes of severe acute and chronic pain, and acute pain crises are associated with impaired health-related quality of life (HRQOL).

A poster presented at Virtual ISPOR 2021 assessed the cost of illness and the economic burden of SCD in the United States through a literature review using a systematic approach.1

“The study aims to help policymakers and payers with evidence-based decision-making for funding and efficient resource allocation for screening and treatments of SCD and hence the national burden of the disease,” the author explained.

There were 5 studies that were eligible for the review. Of those, 2 identified the cost of SCD as total cost of hospitalization and emergency department (ED), inpatient, and outpatient visits. One of these studies focused on adults and found the total direct cost of SCD was $2.98 billion in 2015 whereas the other focused on the pediatric population and found the total cost was $60.8 billion per 2015 data.

A third study focused on the costs of hospitalization and readmission due to sickle cell acute pain episodes. The remaining 2 studies reviewed health care utilization. One looked at hospital admissions, length of stay, and 30-day all-cause readmission, whereas the other looked at hospitalizations; ED, outpatient, and inpatient visits; and length of stay.

The author noted that none of the studies “included a full scope of a cost of illness study,” and therefore “there is still a gap in understanding the total healthcare expenditure of SCD in the US.”

She added that the disease’s complexity increases as patients age and the burden on health care resource utilization increases, as well. However, SCD has been underfunded compared with other, similar hereditary diseases.

“More research is required to identify the costs of SCD, both direct and indirect, as this disease inevitably disrupts the patients’ quality of life due to frequent and recurrent hospitalization,” she concluded.

A second poster investigated the factors associated with HRQOL and economic burden in patients with SCD.2

The researchers recruited 32 adult patients (≥18 years) with SCD for a pilot study to develop standardized SCD data collection using self-reporting from patients and clinical chart review. They collected data on patient sociodemographics, fatigue, chronic pain, and HRQOL.

The mean age of the 32 patients was 36.7 years, and 65.6% of them were female. The majority (84.4%) had hemoglobin SS sickle beta thalassemia (HbSS/Sbthal0), 81.3% had chronic pain, and the mean EuroQol-5 Dimension visual analogue scale (EQ-5D VAS) was 63.4. The mean EQ-5D VAS for the general US population between the ages of 33 and 44 years is 81.8.

The mean fatigue score was 57.9 and negatively correlated with the EQ-5D index score (0.79), as well as the Adult Sickle Cell Quality of Life Measurement Information System (ASCQ-ME) scores for pain, sleep, emotion, and social. Fatigue scores were correlated with ASCQ-ME pain episode frequency.

Compared with patients without chronic pain, those with chronic pain had a significantly lower mean ASCQ-ME sleep score (48.3 vs 57.1) and EQ-5D index score (0.72 vs 0.89).

“As the pilot standardized data collection tools appear feasible for adult patients and healthcare providers, the tools’ use should be expanded to the pediatric SCD population to better understand SCD burden throughout the lifespan,” the authors concluded.


1. Dina S. Cost of illness of sickle cell disease in the US, payers’ perspective: (CRESCENT). Presented at: Virtual ISPOR 2021; May 17-20, 2021. Poster PRO26.

2. Lanzkron S, Crook N, Wu J, et al. Health-related quality of life in persons with sickle cell disease. Presented at: Virtual ISPOR 2021; May 17-20, 2021. Poster PRO66.