Development of a severity classification system for sickle cell disease

Nirmish Shah, David Beenhouwer, Michael S. Broder, Lanetta Bronte-Hall, Laura M. De Castro, Sarah N. Gibbs, Victor R. Gordeuk, Julie Kanter, Elizabeth S. Klings, Thokozeni Lipato, Deepa Manwani, Brigid Scullin, Irina Yermilov, Wally R. Smith

Research output: Contribution to journalArticlepeer-review

7 Scopus citations


Purpose: There is no well-accepted classification system of overall sickle cell disease (SCD) severity. We sought to develop a system that could be tested as a clinical outcome predictor. Patients and Methods: Using validated methodology (RAND/UCLA modified Delphi panel), 10 multi-disciplinary expert clinicians collaboratively developed 180 simplified patient histories and rated each on multiple axes (estimated clinician follow-up frequency, risk of complications or death, quality of life, overall disease severity). Using ratings on overall disease severity, we developed a 3-level severity classification system ranging from Class I (least severe) to Class III (most severe). Results: The system defines patients as Class I who are 8-40 years with no end organ damage, no chronic pain, and <4 unscheduled acute care visits due to vaso-occlusive crises (VOC) in the last year. Patients <8 or >40 years with no end organ damage, no chronic pain, and <2 unscheduled acute care visits are also considered Class I. Patients any age with >5 unscheduled acute care visits and/or with severe damage to bone, retina, heart, lung, kidney, or brain are classified as Class III (except patients >25 years with severe retinopathy, no chronic pain, and 0-1 unscheduled acute care visits, who are considered Class II). Patients not meeting these Class I or III definitions are classified as Class II. Conclusion: This system consolidates patient characteristics into homogenous groups with respect to disease state to support clinical decision-making. The system is consistent with existing literature that increased unscheduled acute care visits and organ damage translate into clinically significant patient morbidity. Studies to further validate this system are planned.

Original languageEnglish (US)
Pages (from-to)625-633
Number of pages9
JournalClinicoEconomics and Outcomes Research
StatePublished - 2020


  • Chronic pain
  • Disease severity
  • Expert panel
  • Organ damage
  • Vaso-occlusive crises

ASJC Scopus subject areas

  • Economics, Econometrics and Finance (miscellaneous)
  • Health Policy


Dive into the research topics of 'Development of a severity classification system for sickle cell disease'. Together they form a unique fingerprint.

Cite this