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Hereditary Angioedema

Hereditary Angioedema

What is Hereditary Angioedema?

Hereditary Angioedema (HAE) is a genetic condition that happens when the body does not properly make an inhibitor protein called C1 esterase inhibitor. This rare and potentially life-threatening condition leads to episodes of swelling (edema), since the body cannot properly make this inhibitor protein to stop the swelling process. This condition can be very dangerous when the airways swell and prevent breathing. Angioedema is similar to hives, but the swelling is under the skin rather than at the surface of the skin.

How do I get Hereditary Angioedema?

HAE is hereditary, meaning that it is genetic and tends to run in families. While HAE is genetic, it still may take time for a patient to be diagnosed with HAE. It is not unusual for patients to be adults when they receive the HAE diagnosis.

Symptoms

Swelling of face, airway, hands, and feet

Sharp abdominal pain, nausea, and vomiting from intestinal swelling

Triggers

Dental Work

Illness

Surgery

Cause

Hereditary

Genetic

Runs in Families

Testing

Testing

If Hereditary Angioedema is suspected, a C1 Esterase Inhibitor Test will be conducted. In this test, your blood will be drawn and the levels of the inhibitor will be measured.

Treatment

Treatment

After taking a full medical history and conducting the proper testing, your physician will select the best treatment plan for you.

C1 Esterase Inhibitor Replacement

Since this condition happens because the C1 Esterase Inhibitor protein is too low, one of the treatments is to receive infusions of the C1 Esterase Inhibitor protein so that the protein level will become normal.

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