This letter, sent to support the inclusion of both IVIg and SCIg in the EML 15th edition, is pertinent to this application for SCIg to be included in the EML for children. The indication for SCIg is restricted to patients with primary immune deficiencies [PIDs].

May 8, 2006 As international authorities in the diagnosis and management of primary immunodeficiency diseases (PIDs) brought together by the Jeffrey Modell Foundation, we the undersigned provide a statement of the vital importance of therapeutic immunoglobulin (polyvalent human immunoglobulins) to our patients. This includes both intravenous (IV) and subcutaneous (SC) immunoglobulin. PIDs are conditions caused by inherent defects of the immune system that lead to recurrent, severe or unusual infections, which can be fatal. Therapeutic immunoglobulins are the only effective therapy for life-long treatment of some PIDs. Use of therapeutic immunoglobulin in PIDs is efficacious in preventing infection-related death, increases lifespan, preserves organ function, and improves quality of life. Such therapies have been shown to be safe and cost-effective. In this time of challenges we therefore need to emphasize the essential nature of immunoglobulin in treating PID patients, especially since immunoglobulin therapy is absolutely indispensable and irreplaceable for treatment of PIDs. Signed, ________________________ ________________________ _________________________

Helen Chapel, M.D. Chaim Roifman, M.D. Jordan Orange, M.D., Ph.D. Oxford Radcliffe Hospital Hospital for Sick Children, Toronto Children’s Hospital of Philadelphia ________________________ ________________________ _________________________ Mary Ellen Conley, M.D. Alain Fischer, M.D., Ph.D. Charlotte Cunningham-Rundles, M.D., Ph.D. St. Jude Children’s Research Hosp. Hôpital Necker-Enfants Malades Mount Sinai Medical Center ________________________ ________________________ _________________________ Reinhard Seger, M.D., Ph.D. Amos Etzioni, M.D. David B. Lewis, M.D.

University Children’s Hospital, Switzerland Technion-Israel Institute of Technology Stanford University School of Medicine

________________________ ________________________ _________________________

This letter, sent to support the inclusion of both IVIg and SCIg in the EML 15th edition, is pertinent to this application for SCIg to be included in the EML for children. The indication for SCIg is restricted to patients with primary immune deficiencies [PIDs].

Raif Geha, M.D. Prof. Dr. med. Reinhold Schmidt Professor Lennart Hammarström Harvard Medical School Hannover Medical School Karolinska University Hospital in Huddinge ________________________ ________________________ _________________________ Ramsay Fuleihan, M.D. Timothy Niehues, M.D. Noorbibi Day-Good, Ph.D. Yale Child Health Research Center Universitats Klinikum Düsseldorf University of South Florida ________________________ ________________________ _________________________ Francisco A. Bonilla, M.D., Ph.D. Gary Kleiner, M.D., Ph.D. Jennifer Puck, M.D. Children’s Hospital Boston University of Miami University of California, San Francisco ________________________ ________________________ _________________________ Melvin Berger, M.D., Ph.D. Alessandro Aiuti, M.D., Ph.D. Hans D. Ochs, M.D. Case Western Reserve University San Raffaele Telethon Institute University of Washington School of Medicine School of Medicine for Gene Therapy, Italy Children’s Hospital Seattle ________________________ ________________________ _________________________

Martha Eibl, M.D. Lisa Kobrynski, M.D. Ricardo U. Sorensen, M.D. Biomedical Research Institute, Austria Emory University Louisiana State University Medical Center ________________________ ________________________ E. Richard Stiehm, M.D. Mark Ballow, M.D. Professor Luigi Notarangelo M.D. Mattel Children’s Hospital at UCLA Children’s Hospital of Buffalo University of Brescia, Italy