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A novel US-based grading system for disease severity in sickle cell disease: the Sickle Cell Outcome Grading System (SCOGS)

  • Tarun Aurora
  • , Renee Hodges
  • , Joseph R Wardell
  • , Martha Barton
  • , Janna Lipford
  • , Clifford M Takemoto
  • , Marisol Betensky
  • , Payal Desai
  • , Seethal A Jacob
  • , Parul Rai
  • , John Strouse
  • , Lewis L Hsu
  • , Melissa Azul
  • , Monica Hulbert
  • , Winfred Wang
  • , Ravi Talati
  • , Robert Liem
  • , Sheinei Alan
  • , Elizabeth Klings
  • , Abena Appiah-Kubi
  • Caterina Minniti, India Sisler, Patrick McGann, Madhav Vissa, Marquita Nelson, Stephanie Guarino, Robin Miller, Sharl Azar, Sherif Badawy, Christina Abrams, Sophie Lanzkron, Elna Saah, Farranz Alvarez Nunez, Nadirah El-Amin, Nickhill Bhakta, Jane S Hankins
  • School of Medicine, Emory University
  • St. Jude Children's Research Hospital
  • Johns Hopkins All Children's Hospital
  • Wake Forest University
  • Riley Children's Hospital
  • Duke University
  • University of Illinois Peoria
  • Medical College of Wisconsin
  • Harvard University
  • Cleveland Clinic
  • Ann & Robert H. Lurie Children's Hospital
  • Inova Health System
  • Boston University
  • Cohen Children's Medical Center of New York
  • Albert Einstein College of Medicine
  • Virginia Commonwealth University
  • Brown University Health
  • University of California San Francisco
  • University of Tennessee Health Sciences Center
  • Medical University of South Carolina
  • Thomas Jefferson University
  • University of Alabama at Birmingham
  • Orlando Health Arnold Palmer Hospital

Research output: Contribution to journalReview articlepeer-review

Abstract

Sickle cell disease is a haematological disorder marked by recurrent events and progressive organ dysfunction, yet a standardised framework to assess disease severity is lacking. We developed a novel US-based severity grading system through identification of sickle cell disease-related complications mapping all to the Common Terminology Criteria For Adverse Events and applying a five-level severity scale. A modified Delphi process involving 29 US-based sickle cell disease experts was conducted over three virtual rounds (from Oct 20 to Nov 8, 2023; from June 26 to Aug 1, 2024; and from Nov 6, 2024, to Jan 7, 2025), with the use of a 9-point Likert scale ratings and iterative feedback. An in-person consensus workshop was then held in Memphis, TN, USA on Jan 16-17, 2025. This workshop was funded by the project's NIH grant (1R01CA270157-01). The final classification includes 53 clinical outcomes, each with diagnostic criteria, grades, and temporal patterns. This standardised, consensus-driven system provides a new benchmark for assessing sickle cell disease severity, with applications in clinical trials, disease burden assessment, and predictive modelling.

Original languageEnglish
Pages (from-to)e418-e425
JournalThe Lancet Haematology
Volume13
Issue number6
DOIs
StatePublished - Jun 2026

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