The impact of vaso-occlusive crises and disease severity on quality of life and productivity among patients with sickle cell disease in the US.

Journal Article (Journal Article)

AIM: Sickle cell disease (SCD) is a lifelong blood disorder affecting approximately 100,000 individuals in the United States (US). A number of new treatments have recently become available to improve SCD clinical outcomes, but it is unclear how treatment innovations that reduce disease severity could affect patients' humanistic and economic outcomes. METHODS AND MATERIALS: To answer this question, an online survey of US adult residents with a self-reported SCD diagnosis was conducted. Humanistic outcomes based on health-related quality of life (HRQoL)) were assessed during and outside of vaso-occlusive crises (VOCs). Economic outcomes were measured by annual household income and whether the respondent received disability insurance. RESULTS: Among the 301 respondents completing the survey, average age was 34.4 years and 73.4% were female. Average HRQoL, measured using health utilities, were 0.311 (95% CI: 0.286, 0.337) during a VOC and 0.738 (0.720, 0.756) not during a VOC. The likelihood of claiming disability insurance was correlated with more frequent VOCs (0 VOCs: 12% vs. ≥4 VOCs: 47%, p = .002) and disease severity (Severity Class II: 16% vs. Severity Class III: 39%, p = .03). There was a weak relationship between VOC frequency and household income (0 VOCs: $47,488 vs. ≥4 VOCs: $34,569, p = .06) and no evidence of a relationship between disease severity class and income (Severity Class II: $42,443 vs. Severity Class III: $36,842, p = .29). CONCLUSION: In conclusion, disease severity, strongly predicted worse self-reported HRQoL, moderately predicted increased likelihood of collecting disability insurance, and weakly predicted lower household income levels.

Full Text

Duke Authors

Cited Authors

  • Shafrin, J; Thom, HHZ; Keeney, E; Gaunt, DM; Zhao, LM; Bhor, M; Rizio, AA; Bronté-Hall, L; Shah, N

Published Date

  • May 1, 2021

Published In

Volume / Issue

  • 37 / 5

Start / End Page

  • 761 - 768

PubMed ID

  • 33686891

Electronic International Standard Serial Number (EISSN)

  • 1473-4877

Digital Object Identifier (DOI)

  • 10.1080/03007995.2021.1897556


  • eng

Conference Location

  • England