NORFOLK -- You may not have heard of it, but H-A-E is something parents should know about.
Hereditary angioedema, or H-A-E, is a potentially-fatal genetic disorder that causes recurrent swelling of the extremities, face, trunk, abdomen and upper airway. CHKD physicians say it's often mistaken for allergic reactions.
What is HAE – and why can it be deadly?
HAE is a genetic disorder that causes recurrent swelling of the extremities, face, trunk, abdomen and upper airway.
Parents and even doctors may believe that HAE is a result of an allergic condition and treat it with medications that do not work – and that actually may worsen an episode.
Abdominal swelling associated with HAE can cause severe pain and disfigurement and result in unnecessary surgeries for conditions such as appendicitis – again, a mistake even doctors can make. When HAE strikes the throat, it can be fatal and will not respond to medications for allergic reactions. Until recently, the only treatment was intubation.
How can parents recognize HAE attacks?
HAE is extremely rare, but its attacks are distinctive.
The swelling of acute attacks usually develops over 12 to 36 hours.
The swelling that occurs with HAE is never itchy and often is accompanied by a flat, reddish rash.
Parents should suspect HAE if their child presents with non-itchy swelling that does not respond to antihistamines – or if there’s a family history.
What are the treatments for HAE?
Until recently, treatments were ineffective.
Today, there are treatments that can stop most attacks.
It’s important to consult with a specialist for a definitive diagnosis and for medications, some of which can be self-administered.
For a child with HAE, a specialist can prescribe new treatments that can stop or reduce attacks as they occur.
To contact a CHKD allergist for evaluation, call 757-668-8255.