Shapiro Ralph S, Zacek Lisa
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).
J Infus Nurs. 2014 Jul-Aug;37(4):284-90. 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.
遗传性血管性水肿（HAE）是一种罕见的疾病，会导致周期性发作的有时疼痛的肿胀，可能影响任何器官系统。HAE会导致显著的发病率和生活质量下降，需要患者寻求紧急医疗护理。HAE可用C1酯酶抑制剂浓缩物（C1-INH）、依卡替班和艾卡拉肽进行治疗。最近的共识指南建议，所有HAE患者都应考虑接受自我给药治疗的培训，以治疗急性发作或预防发作。许多患者已安全、成功地自行静脉输注C1-INH，从而实现快速治疗、缩短发作时间并改善生活质量。通过适当的患者选择、充分的指导和随访，自我给药的C1-INH疗法是治疗HAE的一种可行且有利的选择，尤其是对于发作频率为中度至高度的患者。
