Hereditary angioedema (HAE) is an inherited disease characterized by recurrent episodes of severe swelling (angioedema).
Hereditary angioedema is estimated to affect 1 in 50,000 people.
The most common cause of HAE is a decrease in C1 esterase inhibitor (C1 INH), a protein that is part of our body’s normal immune system. Patients with HAE do not experience hives. Allergic reactions to foods or stinging insects, which may cause hives, may also cause angioedema.
This condition is inherited in an autosomal dominant pattern, which means one copy of the altered gene in each cell is sufficient to cause the disorder. In some cases, an affected person inherits the mutation from one affected parent. Other cases result from new mutations in the gene and occur in people with no history of the disorder in their family.
Symptoms of hereditary angioedema typically begin in childhood and worsen during puberty. On average, untreated individuals have an attack every 1 to 2 weeks, and most episodes last for about 3 to 4 days. The frequency and duration of attacks vary greatly among people with HAE, even among people in the same family.
Pressure, trauma, or emotional stress can trigger an attack; however, the trigger of most attacks is unknown. Typical attacks last for about 1-3 days. If your child develops an acute attack, please contact your physician or go to the emergency room.
Treatment for an attack includes:
Attacks can be prevented with: