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Training Hereditary Angioedema Patients to Self-administer Intravenous C1 Esterase Inhibitor Concentrate

Shapiro, Ralph S. MD; Zacek, Lisa RN

doi: 10.1097/NAN.0000000000000049

Hereditary angioedema (HAE) is a rare disorder that causes periodic attacks of sometimes painful swelling that may affect any organ system. HAE results in significant morbidity and diminished quality of life and requires patients to seek urgent medical care. HAE can be treated with C1 esterase inhibitor concentrate (C1-INH), icatibant, and ecallantide. Recent consensus guidelines recommend that all HAE patients be considered for training in self-administration of therapy to treat acute attacks or to prevent attacks. Many patients have safely and successfully self-administered intravenous infusions of C1-INH, resulting in rapid treatment, shortened attacks, and improved quality of life. With proper patient selection and adequate guidance and follow-up, self-administered C1-INH therapy is a viable and favorable option to treat HAE, particularly in patients with a moderate to high frequency of attacks.

Midwest Immunology Clinic, Plymouth, Minnesota.

Ralph S. Shapiro, MD, founded the Midwest Immunology Clinic in 1995, where he currently serves as director. His current practice involves the diagnosis and care of primary immune deficiency and autoimmune disorders. Research interests include genetic defects in primary immunodeficiencies, developing novel therapeutic approaches to diseases, and drug development pertaining to IVIG, SCIG, and C1 esterase inhibitors. Dr Shapiro's prior experience includes a fellowship in pediatric hematology and oncology at the University of Minnesota, and attending staff in immunology and bone marrow transplantation at the University of Minnesota.

Lisa Zacek, RN, currently serves as the infusion center manager at the Midwest Immunology Clinic, where she has worked since 2001. Her responsibilities include oversight of the infusion center, study coordinator, and nurse consultant for the CSL SHARE program (SC training).

Corresponding Author: Ralph S. Shapiro, MD, Midwest Immunology Clinic, 15700 37th Ave, Suite 110, Plymouth, MN 55446 (

Ralph S. Shapiro and Lisa Zacek have both served as paid consultants for CSL Behring.

© Copyright 2014 by Infusion Nurses Society