Most of us know the word swelling as being synonymous with allergy. Sometimes, because of a new food, medicine or insect bite, the face or lips swell. However, there’s another situation where life-threatening swelling may occur without the characteristic allergy response.
It’s known as hereditary angioedema (HAE).
HAE is a rare genetic disorder characterised by unpredictable and recurrent episodes of swelling. The swelling may involve the hands, feet, face and abdomen, but the most dangerous attacks are those affecting the throat and upper airway.
HAE is primarily bradykinin-mediated and not histamine-mediated like ordinary allergic angioedema. The difference is important because the typical treatments for allergic reactions (e.g., antihistamines and corticosteroids) are not helpful for an HAE attack.
Attacks can happen at any time and may be caused by trauma, procedures, infections or hormonal changes. Abdominal attacks can be particularly perplexing because they can be very painful, with nausea and vomiting sometimes occurring, which may lead to unnecessary investigations or even surgery if HAE is not recognised.
Fortunately, there has been a huge change in treatment. Acute attacks can be treated with modern therapies and in suitable patients, future attacks can be prevented.
A diagnosis is more than just a label for someone affected by HAE. It can mean being able to identify an attack early, as well as to be able to have the right treatment available and know when an attack is a medical emergency due to airway swelling.
It may appear as swelling, but it can be quite serious.
Had you heard about this condition before?
MBH/PS