Hereditary angioedema (HAE) is a rare disease. Regardless, patients with HAE have access to multiple state-of-the-art medications available for on-demand use and prevention that reduce the frequency and burden of HAE attacks. These treatments have greatly reduced the burden of disease and helped patients achieve improved quality of life. However, with greater numbers of therapeutic options, HAE care has become more complex. In this review, we addressed essential elements of an individualized comprehensive management plan for a patient with HAE. We focused on access to an expert physician, ongoing patient education, access to effective treatment options, coordination of care and management of treatment logistics, ongoing monitoring of attacks and treatments, and other resources for patient support. This plan will need to be communicated with the patient and other care providers, especially during emergent conditions, and accommodate the patient’s lifestyle with consideration for work, school, travel, etc. Periodically, the physician and the patient will need to review information about attacks, triggers, and treatments to identify areas for improvement and update the plan.

Background: Hereditary angioedema (HAE), a rare disease that is characterized by painful and recurring non-allergic swelling episodes, is caused by the deficiency or dysfunction of C1 inhibitor (C1INH) protein. A comprehensive HAE management plan may require long-term prophylaxis (LTP) in addition to on-demand treatment to help "normalize" patients' lives so that they may fully engage in work, school, family, and leisure activities. Aim:The main objective of this narrative review is to provide an overview of updated guideline recommendations specific to LTP of HAE and discuss clinical considerations and pharmacologic management options, with a focus on C1INH. Materials and Methods: The authors reviewed relevant HAE literature for current recommendations regarding LTP and the role of C1NH. Results: Acute HAE attacks are treated with on-demand medication; however, there is a consensus that LTP should routinely be considered for risk reduction and prevention of future episodes. The 2017 World Allergy Organization/European Academy of Allergy and Clinical Immunology guidelines recommend that all patients with HAE be evaluated for LTP routinely and the 2020 HAE Association (HAEA) guidelines emphasize that the decision to use LTP should not be based on rigid criteria, but rather should be based on individual patient needs. Both guidelines recommend C1INH as first-line/preferred therapy for LTP in a range of patient types including adults, children/adolescents, and pregnant/lactating patients. The HAEA also recommends the kallikrein inhibitor, lanadelumab, as a first-line option for LTP. HAE pathway-specific agents for LTP have not been associated with notable safety concerns. Discussion: Plasma-derived C1INH has been available for 40+ years in Europe and impacts multiple targets within the HAE pathway. C1INH has been used for ondemand treatment and LTP. A subcutaneous formulation of plasma-derived C1INH is approved for LTP and produces functional C1INH activity levels consistently above the threshold needed for protection from HAE attacks. Other This is an open access article under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in any medium, provided the original work is properly cited.