Are you prepared for a HAE emergency?

If you are living with hereditary angioedema (HAE), you need an emergency plan.

HAE causes recurring episodes of swelling. This swelling can affect various parts of the body, including the airways.

Swelling in the airways can make it difficult for a person to breathe. An attack that involves the airways can quickly become life-threatening. Though this only occurs in a small percentage of HAE attacks, HAE is unpredictable, and any attack has the potential to affect the airways.

Having a plan in place can help keep you safe if an emergency occurs. Below is a look at the key strategies you need to consider.

Know how to recognize and respond to an emergency

As with most aspects of HAE, your healthcare provider will be your best source of information. The potential for emergencies, the signs and symptoms of an emergency, and what to do in an emergency are important topics to cover during your appointments.

Your healthcare provider should prescribe an on-demand (rescue) medication to take during an attack. This medication can help reduce the severity and duration of the attack.

Your healthcare provider should also give you instructions on what to do if you experience an HAE attack. You should seek medical care if you experience an HAE attack, even if you’ve taken an on-demand medication at the first sign of symptoms. Always seek medical care if symptoms worsen or if a medication does not seem to be working.

You will want to have your healthcare provider’s contact information in a place that is easy to access for yourself and for someone who may be assisting you during an attack, such as family and friends.

When to seek emergency care

In some circumstances, you may need to seek emergency care, such as calling 911 or going to an emergency room. You should seek emergency medical care immediately if you experience:

• Swelling in the throat, tongue, or face that affects your ability to breathe or swallow
• Difficulty breathing, noisy breathing, voice changes (hoarseness), pain, or trouble swallowing
• Attacks that cause severe and rapid swelling and/or pain

If you feel something is wrong, or feel that a symptom could signal something serious, seek emergency medical care.

Know who to contact and where to go

While you can predict if or when an emergency will occur, you can still take steps to prepare ahead of time:

• Know how to get to the nearest 24-hour emergency room.
• Contact your local hospitals and medical centers in your area, inform them of your condition, and ask what HAE emergency treatment is available there.
• Contact your local emergency medical services and inform them of your condition. Ask what information they need to help provide you with treatment in the event of an emergency.

If you are participating in a sport, activity, or class, it’s important that coaches and instructors know about your condition and what to do in an emergency.

Wear a medical alert ID

People with HAE may also consider wearing a medical ID. Also called a medical identification tag, medical alert ID, or medical alert jewelry. This is often a piece of jewelry (like a bracelet or a necklace) that provides critical information about your condition and the type of care you need in an emergency. This can help if you are unable to communicate verbally during an emergency (which can occur if there is swelling in the throat).

Remember, this information can be lifesaving. HAE is fundamentally different than allergic reactions, but its symptoms can mimic those of allergic reactions. Treatment for severe allergic reactions (like epinephrine) will have no effect on an HAE attack.

Having this information readily available for your healthcare providers will save them time and enable them to provide you with the appropriate treatment in an emergency.

Preparing for emergencies

Everyone with HAE should have an emergency kit. This is another topic to review with your healthcare provider, but here are some general guidelines:

• Have at least two doses of your on-demand medication accessible at all times
• Have instructions for administering your on-demand medication
• Have an emergency information card explaining what HAE is and how it needs to be treated
• Have step-by-step instructions on what to do during an attack
• Have names and contact information for your emergency contacts

For on-demand medications (as well as other medications), pay attention to expiration dates and how the medications need to be stored. Some medications may be stored at room temperature while others may need to be refrigerated.

Reduce your risk of HAE attacks

Perhaps the best protection against emergencies is taking steps to prevent HAE attacks from occurring.

Identify and avoid triggers

Known triggers include stress, trauma, injury, infections, certain medications (such as ACE inhibitors, NSAIDs, and estrogen-containing medications), dental work, and surgery. Repetitive tasks that involve physical pressure and vibration (such as gripping a steering wheel, typing, or using tools) can also cause symptoms. You won’t always be able to identify a trigger for an HAE attack.

Preventive (prophylactic) therapies

These are therapies that are taken to prevent HAE attacks from occurring. These may be taken on an ongoing basis or taken ahead of circumstances that are likely to trigger an attack, such as dental work, surgery, or in some cases, stressful life events.

Also remember that HAE is a different experience for everyone. Think about what you need from treatment. Think about your concerns. Bring these up at your appointments.