The Impact of Vaso-Occlusive Crises and Disease Severity on Quality of Life and Productivity Among Patients with Sickle Cell Disease in the US

Jason Shafrin, Howard H Z Thom, Edna Keeney, Daisy M Gaunt, Lauren Zhao*, Menaka Bhor, Avery Rizio, Lanetta Bronte-Hall, Nirmish Shah

*Corresponding author for this work

Research output: Contribution to journalArticle (Academic Journal)peer-review


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.

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).

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.
Original languageEnglish
Number of pages9
JournalCurrent Medical Research and Opinion
Early online date27 Mar 2021
Publication statusE-pub ahead of print - 27 Mar 2021


  • Sickle cell disease
  • vaso-occlusive crisis
  • quality of life

Cite this