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Development of a Severity Classification System for Sickle Cell Disease.

Publication ,  Journal Article
Shah, N; Beenhouwer, D; Broder, MS; Bronte-Hall, L; De Castro, LM; Gibbs, SN; Gordeuk, VR; Kanter, J; Klings, ES; Lipato, T; Manwani, D ...
Published in: Clinicoecon Outcomes Res
2020

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.

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Published In

Clinicoecon Outcomes Res

DOI

ISSN

1178-6981

Publication Date

2020

Volume

12

Start / End Page

625 / 633

Location

New Zealand

Related Subject Headings

  • 4407 Policy and administration
  • 4203 Health services and systems
  • 3801 Applied economics
  • 1117 Public Health and Health Services
 

Citation

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Chicago
ICMJE
MLA
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Shah, N., Beenhouwer, D., Broder, M. S., Bronte-Hall, L., De Castro, L. M., Gibbs, S. N., … Smith, W. R. (2020). Development of a Severity Classification System for Sickle Cell Disease. Clinicoecon Outcomes Res, 12, 625–633. https://doi.org/10.2147/CEOR.S276121
Shah, Nirmish, David Beenhouwer, Michael S. Broder, Lanetta Bronte-Hall, Laura M. De Castro, Sarah N. Gibbs, Victor R. Gordeuk, et al. “Development of a Severity Classification System for Sickle Cell Disease.Clinicoecon Outcomes Res 12 (2020): 625–33. https://doi.org/10.2147/CEOR.S276121.
Shah N, Beenhouwer D, Broder MS, Bronte-Hall L, De Castro LM, Gibbs SN, et al. Development of a Severity Classification System for Sickle Cell Disease. Clinicoecon Outcomes Res. 2020;12:625–33.
Shah, Nirmish, et al. “Development of a Severity Classification System for Sickle Cell Disease.Clinicoecon Outcomes Res, vol. 12, 2020, pp. 625–33. Pubmed, doi:10.2147/CEOR.S276121.
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. Development of a Severity Classification System for Sickle Cell Disease. Clinicoecon Outcomes Res. 2020;12:625–633.

Published In

Clinicoecon Outcomes Res

DOI

ISSN

1178-6981

Publication Date

2020

Volume

12

Start / End Page

625 / 633

Location

New Zealand

Related Subject Headings

  • 4407 Policy and administration
  • 4203 Health services and systems
  • 3801 Applied economics
  • 1117 Public Health and Health Services