Immunologic Research

, 51:61

Global study of primary immunodeficiency diseases (PI)—diagnosis, treatment, and economic impact: an updated report from the Jeffrey Modell Foundation

  • Vicki Modell
  • Bonnie Gee
  • David B. Lewis
  • Jordan S. Orange
  • Chaim M. Roifman
  • John M. Routes
  • Ricardo U. Sorensen
  • Luigi D. Notarangelo
  • Fred Modell
Article

DOI: 10.1007/s12026-011-8241-y

Cite this article as:
Modell, V., Gee, B., Lewis, D.B. et al. Immunol Res (2011) 51: 61. doi:10.1007/s12026-011-8241-y

Abstract

A large population of patients with recurring infections are undiagnosed or under diagnosed and Primary Immunodeficiency (PI) is more common than had been previously estimated. The results strongly indicate the measurable impact of Physician Education and Public Awareness in identifying patients with an underlying PI. The Jeffrey Modell Centers Network (JMCN) provides the infrastructure for referral, diagnosis and appropriate treatment. All disease classifications and identified defects increased significantly over the study period. Quality of Life for referred and diagnosed patients dramatically improved compared to undiagnosed patients. There is a substantial cost savings for diagnosed patients compared to undiagnosed, even if regular IgG is required. The SPIRIT® Software successfully identified patients with PI in a large database and at three pilot sites. The Software was successfully tested for specificity and sensitivity.

Keywords

Primary immunodeficiencies (PI)Jeffrey Modell Foundation (JMF)Jeffrey Modell Centers Network (JMCN)Immunoglobulin replacement therapyAwarenessEconomic impactDiagnosisTreatment

Abbreviations

PI

Primary immunodeficiency

JMF

The Jeffrey Modell Foundation

JMCN

Jeffrey Modell Centers Network

PEPAC

Physician Education and Public Awareness Campaign

SPIRIT®

Software for primary immunodeficiency recognition, intervention, and tracking

IgG

Immunoglobulin replacement therapy

HSCT

Hematopoietic stem cell transplantation

ESID

European society for immunodeficiencies

Supplementary material

12026_2011_8241_MOESM1_ESM.docx (854 kb)
Supplementary material 1 (DOCX 853 kb)

Copyright information

© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  • Vicki Modell
    • 1
  • Bonnie Gee
    • 1
  • David B. Lewis
    • 2
  • Jordan S. Orange
    • 3
  • Chaim M. Roifman
    • 4
  • John M. Routes
    • 5
  • Ricardo U. Sorensen
    • 6
  • Luigi D. Notarangelo
    • 7
  • Fred Modell
    • 1
  1. 1.Jeffrey Modell FoundationNew YorkUSA
  2. 2.Department of Pediatrics, Interdepartmental Program in Immunology, and Institute for Immunity, Transplantation, and InfectionStanford UniversityStanfordUSA
  3. 3.Division of Allergy and Immunology, Children’s Hospital of Philadelphia Research Institute, Children’s Hospital of PhiladelphiaUniversity of Pennsylvania School of MedicinePhiladelphiaUSA
  4. 4.Canadian Centre for Primary Immunodeficiency, Division of Immunology and Allergy, Department of PediatricsThe Hospital for Sick Children and the University of TorontoTorontoCanada
  5. 5.Department of PediatricsMedical College of WisconsinMilwaukeeUSA
  6. 6.Department of Pediatrics, Louisiana State University Health Sciences Center, Division of Allergy/ImmunologyChildren’s Hospital of New OrleansNew OrleansUSA
  7. 7.Division of Immunology and The Manton Center for Orphan Disease ResearchChildren’s Hospital BostonBostonUSA