The clinical features of HAE include (7):

• Swelling without urticaria (wheals)

• Onset usually hours (vs minutes to hours for mast cell/allergic reactions)

• Unresponsive to epinephrine, antihistamines, and corticosteroids

Mental stress is a leading cause of HAE acute attacks (1).

Management of HAE with C1 inhibitor deficiency should include integrated management of on-demand, short-term prophylaxis, and long-term prophylaxis (4).

Recommend family testing (6).  Jessica’s first attack may have been prevented by testing when her sister was diagnosed 2 years earlier.

Recognize that the burden of illness with HAE often extends well beyond the physical manifestations and can restrict educational, social, and career opportunities (3).

Recognize and ameliorate factors contributing to the burden of illness in patients with HAE (3).

Use the Angioedema Quality of Life Questionnaire (AE-QoL) to assess symptom-specific QoL impairment in patients with recurrent angioedema (8).

• Other QoL instruments include the generic health-related QoL (Short Form Survey, 12- [SF-12] or 36- item [SF-36]) (9),  Hereditary Angioedema Quality of Life Questionnaire (HAE QoL) (10), and the United States Hereditary Angioedema Association Quality of Life Questionnaire (HAEA QoL) (5,10).

The burden of living with HAE is complex; the health care provider should recognize factors related to disease course, the nature of attacks themselves, treatment challenges, and quality of life (4).

Newer HAE-specific treatments have decreased the burden of disease and improved QoL in patients with HAE (4).