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Development of a Severity Classification System for Sickle Cell Disease

Authors Shah N, Beenhouwer D, Broder MS, Bronte-Hall L, De Castro LM, Gibbs SN, Gordeuk VR, Kanter J, Klings ES, Lipato T, Manwani D, Scullin B, Yermilov I, Smith WR

Received 8 August 2020

Accepted for publication 16 October 2020

Published 28 October 2020 Volume 2020:12 Pages 625—633


Checked for plagiarism Yes

Review by Single anonymous peer review

Peer reviewer comments 3

Editor who approved publication: Professor Dean Smith

Nirmish Shah,1 David Beenhouwer,2 Michael S Broder,2 Lanetta Bronte-Hall,3 Laura M De Castro,4 Sarah N Gibbs,2 Victor R Gordeuk,5 Julie Kanter,6 Elizabeth S Klings,7 Thokozeni Lipato,8 Deepa Manwani,9 Brigid Scullin,10 Irina Yermilov,2 Wally R Smith8

1Department of Medicine, Duke University, Durham, NC, USA; 2Partnership for Health Analytic Research (PHAR), LLC, Beverly Hills, CA, USA; 3Foundation for Sickle Cell Disease Research, Hollywood, FL, USA; 4Department of Medicine, University of Pittsburgh Medical Center, Pittsburgh, PA, USA; 5Department of Medicine, University of Illinois at Chicago, Chicago, IL, USA; 6Department of Medicine, University of Alabama at Birmingham, Birmingham, AL, USA; 7Department of Medicine, Boston University School of Medicine, Boston, MA, USA; 8Department of Internal Medicine, Virginia Commonwealth University, Richmond, VA, USA; 9Department of Pediatrics, Albert Einstein College of Medicine, Bronx, NY, USA; 10Department of Medicine, University of North Carolina, Chapel Hill, NC, USA

Correspondence: Nirmish Shah
Duke University, Department of Medicine, Duke South, Durham, NC 27710, USA
Tel +1 (919) 668-5178

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.

Keywords: expert panel, disease severity, vaso-occlusive crises, organ damage, chronic pain

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