Hereditary angioedema (HAE) is a rare genetic condition that a person is born with. It impacts different parts of your body. It causes swelling in the lining of the gut and lungs and can also cause swelling under the skin. These swelling attacks can impact different parts of your body and vary in severity and length.
It is a condition that you will always have if you are born with it. HAE can be managed and treated but not cured. It is a chronic condition.
If you have HEA, you are born with a problem in a gene that makes the C1 inhibitor, which is a blood protein. Due to the issue with the gene that creates the C1 inhibitor, you lack the proper amount of this protein in your body. Or you may have normal levels of the C1 inhibitor, but it doesn’t work as it should in your body.
The result of the C1 inhibitor protein being at low levels or not working correctly in your body is that tiny blood vessels push fluids to nearby parts of your body, resulting in random and sudden swelling.
With HAE, the main symptom you will experience is swelling. You will not get hives or itching like an allergic reaction. Instead, some part of your body will swell. This swelling is referred to by those with the condition as a “bout” or “episode.” HAE swelling generally lasts between two to five days.
The swelling can happen in different parts of your body. There are six commonly affected areas where swelling occurs in individuals with HAE:
- Mouth or throat
The most dangerous swelling is in your throat, as it can impact your ability to breathe. Swelling in your hands and feet can make it difficult to conduct daily activities, while swelling in the belly can result in diarrhea, vomiting, nausea, and extreme pain.
Many people notice warning signs that their body is about to have a bout of swelling. These warning signs may include the following symptoms:
- Muscle ache
- Extreme fatigue
- Belly pain
- Mood changes
Some people also have triggers that can result in a bout of HAE swelling in the body. These triggers can vary from person to person. It is important to track what is happening in your life when you have a bout of HAE, so you can learn what your triggers are. Possible triggers include:
- Engaging in physical activity
- Minor injury
- Minor surgery
- Use of certain medications
For some women, using birth control with estrogen and getting one’s period can be a trigger.
Diagnosing HAE can be tricky. Sometimes it may be incorrectly diagnosed as allergies. However, common allergy medications, such as corticosteroids and antihistamines, don’t work for HAE. The stomach issues associated with HAE can also be misdiagnosed.
That is why it is important to share detailed information about your swelling, what happens, how it happens, and when it happens with your doctor. Be sure to let them know if someone else in your family has this condition.
A blood test is needed to confirm an HAE diagnosis. If you are diagnosed with HAE, you should have your family get blood tests to see if anyone else has it.
With HAE, treatment involves preventing attacks and treating them when they occur.
It is essential to learn about your particular triggers. Knowing your triggers can help you avoid them and help reduce the frequency of your bouts.
In addition to tracking your triggers, a range of medications and treatments can be used to treat and prevent attacks. The exact medications you need will depend on your health history and the type of attacks you suffer. Most often used medications recommended for HAE include:
- Conestat alfa (Ruconest)
- Berotralstat (Orladeyo)
- C1 esterase inhibitor (Berinert, Cinryze, Haegarda)
- Ecallantide (Kalbitor)
- Icatibant (Firazyr)
- IV Fluids
It is also important to work with your doctor before taking any new medications or undergoing any medical procedure, even if it is something as simple as dental work.
If you ever experience swelling in your throat that impacts your ability to breathe, take the prescribed medication and call 911.
Call West Hills Allergy & Asthma Associates if you suspect you have HAE. We can get you a diagnosis and start you on a treatment path.
For a consultation with Dr. Rene Anderson-Cowell, you can call us at (503) 297-4779 or request an appointment online.