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Living with HAE.

Having hereditary angioedema (HAE) doesn’t mean you can’t live a full, healthy life. It just means you need to:

  • Learn about your condition and treatment
  • Know the symptoms of HAE attacks
  • Know your triggers and watch for symptoms
  • Work with your doctor to find the best treatment plan for you

HAE basics.

HAE is a rare condition that causes repeated swelling (edema) in different parts of the body. About 6,000 people in the United States have HAE and it affects both men and women equally.[1] It’s usually passed down from parents to children, but can happen when there’s no family history.

HAE occurs when there’s a change in the gene that controls the complement-1 esterase inhibitor (C1-INH) protein found in the blood. The C1-INH protein usually helps:

  • Regulate swelling
  • Form blood clots
  • Fight off bacteria and viruses

Your blood vessels can become leaky when there isn’t enough C1-INH or when it doesn’t work the right way. Leaky blood vessels can cause fluid to build up in the tissue, which leads to swelling.

There are two main types of HAE:

  • Type I – The body doesn’t make enough C1-INH. Most people with HAE have this type
  • Type II – The body makes enough C1-INH, but it doesn’t work the right way

About 85 percent of patients with HAE have type I and about 15 percent have type II.[2] A small group of people have a rare type called HAE with normal C1-INH. Unlike type I and II, people with this type of HAE have normal C1-INH. HAE experts are working to learn more about HAE with normal C1-INH.

If you have children, you have a 50 percent chance of passing HAE on to them. Because of this, your doctor may run tests to see if your children also have HAE, even if they don’t have any symptoms. These tests are usually done when children are at least one year old.


The most common symptom of HAE is swelling that doesn’t feel itchy and HAE symptoms don’t respond to medicines that are used to treat allergic reactions or other types of swelling, including:

  • Antihistamines (such as diphenhydramine)
  • Corticosteroids (such as prednisone)
  • Epinephrine

HAE swelling usually lasts for two to five days, but can last longer. The swelling happens most commonly in the:

  • Arms, legs, hands and feet – swelling may be painful enough to affect daily activities
  • Stomach – swelling may cause severe pain, nausea, vomiting, diarrhea and dehydration
  • Throat – swelling in the throat can close the airway, which may cut off the oxygen supply. It can be serious and lead to death.
  • Swelling is also common in the face and genitals


HAE attacks are when symptoms go away and come back many times. A person’s first attack can happen at any point, but for most people it happens before they turn 20 years old. The number and severity of attacks can vary from patient to patient.

You may have early warning signs that an attack is coming. Common warning signs include:

  • A rash that isn’t painful or itchy
  • Change in mood
  • Anxiety
  • Tiredness
  • Tingling or feeling numb in an area of skin where the swelling will start
  • Triggers

Many HAE attacks are sudden (also known as acute attacks) and happen quickly with no known cause. But some things are known to cause HAE attacks. These are called triggers. Triggers can be different from patient to patient and may change throughout your life. Common triggers include:

  • Trauma or injury
  • Stress
  • Surgery
  • Dental procedures
  • Infections
  • Puberty and other hormone changes
  • Certain medications, such as ACE inhibitors (a treatment for high blood pressure)

Managing your HAE can be a challenge but you don’t have to do it alone. Your CVS Specialty HAE CareTeam is here to help. Your CareTeam provides specialized support so you can focus more on living your life, not your HAE. Call your HAE CareTeam at the phone number listed on your prescription label.

This information is not a substitute for medical advice or treatment. Talk to your doctor or health care provider about your medical condition and prior to starting any new treatment. CVS Specialty assumes no liability whatsoever for the information provided or for any diagnosis or treatment made as a result, nor is it responsible for the reliability of the content.

CVS Specialty does not operate all the websites/organizations listed here, nor is it responsible for the availability or reliability of their content. These listings do not imply or constitute an endorsement, sponsorship, or recommendation by CVS Specialty.

Your privacy is important to us. Our employees are trained regarding the appropriate way to handle your private health information.

1. DiscoverHAE Website. Accessed October 3, 2018.

2. DiscoverHAE Website. Accessed October 3, 2018.