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.
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
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.