What causes HAE?
HAE is caused by a mutation in the gene that makes a protein called C1 inhibitor. This protein normally helps control inflammation in your body. In the majority of HAE cases, you either don’t have enough C1 inhibitor, or it doesn’t work properly. You can inherit the gene defect from one of your parents or develop it at conception.
How common is HAE?
HAE is rare, impacting between 1 in 10,000 to 1 in 50,000 people worldwide. Both men and women seem to be affected in equal numbers.
How do I know if I have HAE?
If you have a family history of HAE or experience unusual swelling attacks, talk to your doctor. HAE is diagnosed with a simple blood test that measures C1 inhibitor levels. Sometimes, doctors also test for a blood protein called C4.
What are the most common symptoms of an HAE attack?
Swelling is the most notable symptom of an HAE attack, and it can affect different areas of the body, depending on the type of episode. In a subcutaneous attack, areas under the skin swell, usually on the face, hands, feet, or genitals. Your skin might turn red, but it typically doesn’t itch. If you have an abdominal attack, you may have pain, nausea, or vomiting. The most dangerous type of HAE event, called a laryngeal attack, involves swelling of the throat. If this happens, you may experience hoarseness; a tightening in the throat; trouble breathing or swallowing; or difficulty talking. About 25 percent of people with HAE say they notice a non-itchy rash that happens before or during an attack. Other general symptoms may include:
PainFatigueTingling skinMuscle achesMood changesHeadache
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Does HAE affect children?
Yes, HAE affects children as well as adults. Most experts suggest waiting until children are at least 1 year old before testing them for HAE. If your child has the disease, it’s important to come up with an emergency plan that caregivers and family members can follow if an attack occurs.
Are there warning signs that I’m going to have an attack?
Attacks can be unpredictable, but some people say they notice warning signs that an attack is about to happen. These indicators may include:
A rash or tingling sensation where the swelling startsMood changesAnxietyIrritability or aggressivenessNauseaExtreme fatigue
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What can trigger an HAE attack?
Certain triggers may prompt an HAE attack. These may include:
StressIllnesses, such as a cold or the fluInjuriesPregnancy, menstruation, or breastfeedingMedicines, such as angiotensin-converting enzyme (ACE) inhibitors or oral contraceptivesMedical proceduresActivities, such as typing, writing, shoveling, hammering, or pushing a lawnmower
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If I have HAE, can I continue to work, exercise, and live normally?
Once you’ve established a treatment plan with your doctor, you should be able to live a normal life, which includes working and exercising. But, you may have to take some time off if you experience an episode, as attacks can last two to five days. Taking preventive medicines regularly and managing stress can help reduce the frequency of your attacks.
What treatments are available for HAE?
While there’s no cure for HAE, there are treatments that can help improve your symptoms and quality of life. Some therapies are given to prevent attacks from occurring, and others are taken “on-demand,” when an attack happens. Everyone with HAE should have access to an on-demand medicine. There are currently seven therapies approved by the U.S. Food and Drug Administration (FDA) to prevent or treat HAE. Your doctor will recommend a treatment approach based on the frequency and severity of your attacks, as well as other factors.
How do I travel with my medications?
If you’re traveling by air, experts suggest that you keep your medicines in your carry-on bag in case you have an attack. The Transportation Security Administration allows passengers to travel with medically needed liquids. It’s a good idea to call your local airport to verify their policy. Additionally, you may want to bring an HAE diagnosis letter from your doctor as well as an ID card. If you can’t transport your treatments, ask your physician to write a prescription that you can fill when you arrive at your destination.
What side effects do treatments cause and how can I manage them?
Like most medicines, HAE treatments may cause side effects. The symptoms you experience will depend on the treatment you use, the dosage, and how it’s given. Talk to your doctor about the potential side effects and how you can effectively manage them.
Can it be difficult to get a diagnosis?
HAE is often misdiagnosed, and some people go years without a diagnosis. A misdiagnosis can be dangerous because you won’t get the proper treatment you need. It’s important to find a doctor who can accurately identify HAE. RELATED: Think You’re Allergic to Penicillin? Think Again
If I have HAE, will I pass it on to my children?
If you have HAE, your child has a 50 percent chance of inheriting the abnormal gene for the disease. But, it can also occur when there’s no family history. As many as 25 percent of HAE cases are due to a spontaneous gene mutation.
How can I find a doctor who specializes in treating HAE?
Because the disease is so rare, finding a doctor who has experience in treating HAE can be difficult. You’ll probably want to see an immunologist or hematologist. The US Hereditary Angioedema Association (HAEA) offers a tool to help you locate doctors.
