According to the US HAE Association Medical Advisory Board recommendations, overall Hereditary angioedema (HAE) treatment goals are to reduce morbidity and mortality and to restore a normal quality of life. Because there is significant intra- and interindividual clinical variability, a comprehensive, individualized HAE treatment plan is crucial.1
For all patients with HAE, guidelines recommend they have access to at least two doses of acute, on-demand therapy for administration as early as possible after recognizing the symptoms of an attack beginning. For some patients, on-demand therapy alone is adequate.
Long-term preventative treatment reduces the likelihood of attacks. Patients who are more likely to need long-term treatment have frequent and/or severe attacks or complicating comorbid conditions. Certain patients who live in rural areas and don’t have convenient access to emergency care could also benefit from long-term treatment.
Short-term prophylactic treatment is for patients who know they will encounter a trigger (stressor or procedure) and want to reduce the chance of an attack. The US HAE Association states it is critically important that patients have access to effective on-demand treatment whether the patient is given short-term prophylaxis or not.
Patients with HAE must be seen at least annually—more often if attacks are severe, frequent or laryngeal in nature. Continuous monitoring is critical to evaluate treatment efficacy, assess treatment-associated side effects, and understand the clinical course of each patient’s disease. HAE severity can wax and wane, and treatment may require refinement periodically.
Patients should keep an attack diary, recording descriptions of attacks, how the attacks were treated, how symptoms responded to treatment (e.g., how long did it take for symptoms resolve), and any potential side effects.