Hereditary angioedema (HAE), a rare but life-threatening condition, manifests as acute attacks of facial, laryngeal, genital, or peripheral swelling or abdominal pain secondary to intra-abdominal edema. Resulting from mutations affecting C1 esterase inhibitor (C1-INH), inhibitor of the first complement system component, attacks are not histamine-mediated and do not respond to antihistamines or corticosteroids. Low awareness and resemblance to other disorders often delay diagnosis; despite availability of C1-INH replacement in some countries, no approved, safe acute attack therapy exists in the United States. The biennial C1 Esterase Inhibitor Deficiency Workshops resulted from a European initiative for better knowledge and treatment of HAE and related diseases. This supplement contains work presented at the third workshop and expanded content toward a definitive picture of angioedema in the absence of allergy. Most notably, it includes cumulative genetic investigations; multinational laboratory diagnosis recommendations; current pathogenesis hypotheses; suggested prophylaxis and acute attack treatment, including home treatment; future treatment options; and analysis of patient subpopulations, including pediatric patients and patients whose angioedema worsened during pregnancy or hormone administration. Causes and management of acquired angioedema and a new type of angioedema with normal C1-INH are also discussed. Collaborative patient and physician efforts, crucial in rare diseases, are emphasized. This supplement seeks to raise awareness and aid diagnosis of HAE, optimize treatment for all patients, and provide a platform for further research in this rare, partially understood disorder.

This document and trademark(s) contained herein are protected by law. This representation of RAND intellectual property is provided for noncommercial use only. Unauthorized posting of this publication online is prohibited. Permission is given to duplicate this document for personal use only, as long as it is unaltered and complete. Permission is required from RAND to reproduce, or reuse in another form, any of its research documents for commercial use. For information on reprint and linking permissions, please visit www.rand.org/pubs/permissions.The RAND Corporation is a research organization that develops solutions to public policy challenges to help make communities throughout the world safer and more secure, healthier and more prosperous. RAND is nonprofit, nonpartisan, and committed to the public interest.RAND's publications do not necessarily reflect the opinions of its research clients and sponsors.Support RAND Make a tax-deductible charitable contribution at www.rand.org/giving/contribute www.rand.org Library of Congress Cataloging-in-Publication Data For more information on this publication, visit www.rand.org/t/RR1695Published by the RAND Corporation, Santa Monica, Calif. © Copyright 2018 RAND CorporationR® is a registered trademark. Cover photo credits from top to bottom:Photo by Cpl PrefaceThe Health Related Behaviors Survey (HRBS) is the U.S. Department of Defense's flagship survey for understanding the health, health-related behaviors, and well-being of service members. Originally implemented to assess substance use (i.e., illicit drugs, alcohol, and tobacco), the survey now includes content areas-such as mental and physical health, sexual behavior, and postdeployment problems-that may affect force readiness or the ability to meet the demands of military life. The HRBS is intended to supplement administrative data already collected by the armed forces. In 2014, the Defense Health Agency asked the RAND Corporation to review previous iterations of the HRBS, update survey content, administer a revised version of the survey, and analyze data from the resulting 2015 HRBS of active-duty personnel. The 2015 HRBS included U.S. Air Force, Army, Marine Corps, Navy, and Coast Guard personnel, and this report details the survey methodology and results. No expertise in health, health-related behaviors, or health care is required to read this report. However, it may be of most use to individuals who provide direct care related to the health and health-related behaviors of active-duty service members or who are responsible for making related policy decisions. Additional information can be found in a series of online appendixes.This research was sponsored by the Defense Health Agency and conducted within the Forces and Resources Policy Center of the RAND National Defense Research Institute, a federally funded research and development center sponsored by the Office of the Secretary of Defense, the Joint Staff, the Unified Combatant Commands, the Navy, the Marine Corps, the defense agencies, and the defense Intelli...

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This document and trademark(s) contained herein are protected by law. This representation of RAND intellectual property is provided for noncommercial use only. Unauthorized posting of this publication online is prohibited. Permission is given to duplicate this document for personal use only, as long as it is unaltered and complete. Permission is required from RAND to reproduce, or reuse in another form, any of its research documents for commercial use. For information on reprint and linking permissions, please visit www.rand.org/pubs/permissions.html.The RAND Corporation is a research organization that develops solutions to public policy challenges to help make communities throughout the world safer and more secure, healthier and more prosperous. RAND is nonprofit, nonpartisan, and committed to the public interest. RAND's publications do not necessarily reflect the opinions of its research clients and sponsors.Support RAND Make a tax-deductible charitable contribution at www.rand.org/giving/contribute www.rand.org Library of Congress Cataloging-in-Publication Data is available for this publication.ISBN: 978-0-8330-9436-0 For more information on this publication, visit www.rand.org/t/RR1530Published by the RAND Corporation, Santa Monica, Calif. © Copyright 2016 RAND CorporationR® is a registered trademark.iii Preface U.S. Department of Defense (DoD) policies have rendered both the physical and psychological aspects of "transgender conditions" as disqualifying conditions for accession and allow for the administrative discharge of service members who fall into these categories. However, in July 2015, Secretary of Defense Ashton Carter announced that DoD would "create a working group to study the policy and readiness implications of welcoming transgender persons to serve openly." In addition, he directed that "decision authority in all administrative discharges for those diagnosed with gender dysphoria 1 or who identify themselves as transgender be elevated to the Under Secretary of Defense (Personnel and Readiness), who will make determinations on all potential separations" (DoD, 2015b).It is against this backdrop that DoD is considering allowing transgender personnel to serve openly. To assist in identifying the potential implications of such a change in policy, the Office of the Under Secretary of Defense for Personnel and Readiness asked the RAND National Defense Research Institute to conduct a study to (1) identify the health care needs of the transgender population, transgender service members' potential health care utilization rates, and the costs associated with extending health care coverage for transition-related treatments; (2) assess the potential readiness implications of allowing transgender service members to serve openly; and (3) review the experiences of foreign militaries that permit transgender service members to serve openly. This report documents the findings from that study. This research should be of interest to DoD and military service leadership, members of Congress, and others who are in...

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