Hereditary Angioedema (HAE)
Angioedema or (an area of swelling) can have many causes. Allergic reactions on the skin can frequently have hives and swelling. Sometimes swelling can occur with no hives and can be localized to just one small part on your body. One hand, or one lip, or even your gut may swell, and this episode can last for a few days. It can be very painful. These types of episodes may be unrelated to an allergic reaction and may be something called Hereditary angioedema.
Hereditary Angioedema is a genetic condition where people do not have a break mechanism to stop swelling. They can have severe swelling episodes of different places on their body including extremeties, face, their gut, or even their airway. Frequently there will be patterns to their swelling episodes ie always having their left hand swell up when an attack comes on. However, even if you have a very consistent pattern of swelling of just your left hand, your next swelling episode could be swelling of your airway, which can be fatal.
HAE has a specific defect that causes this condition. Usually a protein is not made, or a defective protein is made and unable to do its job. This protein puts the "breaks" on a swelling episode and so patients with HAE can have severe swelling that can last for days.
Usually the onset of this condition is before or during the teenage years, but can develop later in life. The early onset is usually due to genetic mutations, and the later onset can be due to an underlying condition an therefore can be labled an accquired angioedema.
Because this condition has a very specific cause it has very specific treatments. HAE usually does not respond to allergy medicaitons like benadryl. They have special categories of medicine that are used. For many years fresh frozen plasma infusions, or anti-estrogen medications. Starting in the 2000's new medications for HAE started getting approval.
