Your Step-by-Step Guide to Managing a Hereditary Angioedema Attack

Your Step-by-Step Guide to Managing a Hereditary Angioedema Attack

Your Step-by-Step Guide to Managing a Hereditary Angioedema Attack
Andrey Popov/iStock

Even if you’re doing everything you can to avoid an episode of hereditary angioedema (HAE) — dodging injuries, preventing respiratory illnesses, taking medication — it’s not always possible to ward off an attack.

That fact alone can induce anxiety. Research shows that many people with HAE worry about when an attack will come on — and, once it starts, how extreme the swelling will be.

There are ways to limit the severity of an attack, though, if you know which signs to look out for and how to take action right away. Check out these tips on what to do before, during, and after an HAE attack.

Understand the Warning Signs of an HAE Attack

There’s a wide range of symptoms that can signal an incoming HAE attack. Apart from swelling, symptoms may include include:

  • Abdominal pain and cramping
  • Diarrhea
  • Difficulty breathing
  • Fatigue
  • Red rash that doesn’t itch

“Some people notice warning signs like skin tingling, fatigue, or stomach discomfort before the swelling begins,” says Inderpal Randhawa, MD, a board-certified allergist and immunologist and the founder of the Food Allergy Institute in Long Beach, California.

These symptoms can sometimes appear quickly, but they more often build up gradually, which gives you time to take action, says Dr. Randhawa.

Take Steps to Manage an HAE Attack

Once you know which signs to watch out for, you can take steps to limit the severity of an HAE attack.

Use Rescue Medication

At the first sign of an attack, it’s important to use prescribed HAE medication right away, says Randhawa. Taking these drugs as soon as symptoms appear can ease the severity of an attack and prevent life-threatening complications. Remember to keep these medications on hand always, where you or a caregiver can access them quickly.

Practice Calming Techniques

Anxiety can worsen the symptoms of an HAE episode, so try your best to stay calm during an attack, says Randhawa. (Admittedly, that can be easier said than done, but it’s still important to try.) To tame the tension, use relaxation techniques, such as deep breathing, meditation, and grounding exercises (for example, focusing on what you can see and touch).

Put Your Feet Up

The swelling from HAE can be painful. Try to keep pressure off the swollen areas to the best of your ability, says Randhawa. Elevating your feet or legs may help.

Rest

An HAE attack can trigger a number of uncomfortable symptoms, so try to rest as best you can, says Randhawa. Most attacks last about two to five days, with symptoms increasing in severity before subsiding.

Know When to Seek Medical Care for an HAE Attack

Although only 1 to 3 percent of HAE attacks involve swelling in the upper airway, it’s important to have a plan in place in case it happens to you.

Seek emergency medical care right away if you start to experience:
  • Breathing difficulties
  • Swallowing issues
  • Swelling in the throat or tongue

“Call 911 immediately, because this can be life threatening and needs urgent care,” says Randhawa.

Something worth noting: An HAE attack that affects the upper airway is not the same as anaphylaxis, a severe allergic reaction that can cause the airway to close. Anaphylaxis can be treated with medications such as antihistamines and corticosteroids, whereas throat swelling due an HAE attack requires HAE-specific treatment.

Another reason to seek medical care is if you experience HAE symptoms that are unusual for you or if treatment isn’t helping you find relief.

Follow Up After an HAE Attack

Once you are through the most uncomfortable or painful parts of an HAE episode and have the energy, there are a few follow-up steps that may help you better manage — or prevent — a future episode.

Keep Track of Symptoms

After the swelling goes down, continue to rest and track symptoms, says Randhawa. Use a journal or notes app to record where the swelling occurred, how long the swelling lasted, and which treatments and self-care strategies you used to manage the attack, along with how well they worked.

If symptoms are severe or flaring up frequently and interfering with your quality of life, your doctor may want to either adjust the medication dosage or switch you to a new maintenance medication (to prevent episodes) or on-demand medication (to treat an acute episode).

Stay in Touch With Your Care Team

Everyone with HAE should be in regular contact with their doctor. If symptoms are, for the most part, under control and don’t interfere very much with your daily life, you may only need to see your doctor every 6 to 12 months. If symptoms flare up a little more often or are more severe, you’ll likely want to see your doctor more frequently.

Consider a Clinical Trial

You may also be a candidate for an HAE clinical trial. These test the safety and effectiveness of new potential treatments before they’re fully approved by the U.S. Food and Drug Administration. The US Hereditary Angioedema Association keeps tabs on active clinical trials on their website. Talk with your doctor to get a better understanding of the risks and benefits, and determine if a clinical trial could be a good option for you.

“HAE medications have made significant advancements in the last decade,” says Randhawa. Once you find the right one for you, it’ll be easier to keep symptoms under control.

The Takeaway

  • The warning signs of an HAE attack can vary from person to person, but they tend to include abdominal pain, fatigue, and rashes. These lead up to the hallmark symptom of swelling, particularly in the arms, feet, and legs.
  • Once an attack occurs, take rescue medication as soon as possible. If you have any trouble breathing or swallowing, call 911.
  • Be sure to follow up with your doctor after an attack to discuss whether you may need a treatment adjustment, particularly if you’re experiencing more frequent or severe symptoms.
EDITORIAL SOURCES
Everyday Health follows strict sourcing guidelines to ensure the accuracy of its content, outlined in our editorial policy. We use only trustworthy sources, including peer-reviewed studies, board-certified medical experts, patients with lived experience, and information from top institutions.
Resources
  1. Jean-Baptiste M et al. The Symptom Experience of Hereditary Angioedema (HAE) Patients Beyond HAE Attacks: Literature Review and Clinician Interviews. Orphanet Journal of Rare Diseases. June 16, 2022.
  2. Pedersen D. Hereditary Angioedema. MedlinePlus. March 31, 2024.
  3. Soteres DF. The Elephant in the Room: HAE & Mental Health. US Hereditary Angioedema Association. December 9, 2024.
  4. Abdulkarim A et al. Hereditary Angioedema. StatPearls. May 1, 2023.
  5. What Is Hereditary Angioedema (HAE)? US Hereditary Angioedema Association.
  6. Busse PJ et al. US HAEA Medical Advisory Board 2020 Guidelines for the Management of Hereditary Angioedema. The Journal of Allergy and Clinical Immunology: In Practice. January 2021.

Stephen H. Kimura, MD

Medical Reviewer

Stephen Kimura, MD, is a board-certified allergist and immunologist. He's been in private practice in Pensacola, Florida, for the past 25 years with the Medical Center Clinic, a multi-specialty practice. He enjoys working with people who were seen as patients as children and now are bringing their children to him for care.

Dr. Kimura received his medical degree from the University of Kansas School of Medicine. He went on to complete his residency at Butterworth Hospital in Grand Rapids, Michigan, and later received additional training in allergy and immunology during his fellowship at the University of Kansas.

Kimura grew up in Hawaii, and says he has many happy memories of coming home from school to go surfing, snorkeling, and swimming at the beaches there.

Maria Masters

Maria Masters

Author

Maria Masters is a contributing editor and writer for Everyday Health and What to Expect, and she has held positions at Men's Health and Family Circle. Her work has appeared in Health, on Prevention.com, on MensJournal.com, and in HGTV Magazine, among numerous other print and digital publications.