With hereditary angioedema (HAE), swelling attacks can be hard to predict. One day, you may feel fine — the next, you may have swelling in your skin, stomach, or throat. Because attacks can come on quickly, having the right treatment plan is important.

Some HAE treatments are taken regularly to help prevent swelling attacks. Others are kept on hand to be used as soon as symptoms begin. Your doctor can help you choose a treatment plan based on how often your attacks happen, how severe they are, and what works best for you or your child.

Below, we’ll cover eight treatments for HAE — four for treating attacks as they happen and four for helping prevent attacks over the long term.

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On-Demand HAE Treatments

Keep your on-demand treatment with you at all times so you can use it as soon as HAE symptoms occur. Your care plan may include one or more of these on-demand treatments.

In type 1 HAE, the most common type of HAE, the body doesn’t make enough of a protein called C1 inhibitor (C1INH). In type 1 HAE, too little C1INH leads to excess bradykinin, which causes swelling attacks.

C1 inhibitor replacement treatments can quickly treat an HAE attack. Plasma-derived C1 inhibitor (Berinert) and recombinant C1 inhibitor (Ruconest) are given by IV infusion, which many people can learn to do at home. A second dose may be needed if symptoms continue.

The bradykinin B2 receptor antagonist icatibant (Firazyr) is injected under the skin to treat an HAE attack. Icatibant works by blocking the effects of bradykinin, the chemical that causes swelling in HAE.

Use icatibant as soon as you notice symptoms of an HAE attack. If symptoms continue or return, additional doses may be used as directed by your doctor.

Selective plasma kallikrein inhibitors such as ecallantide (Kalbitor) are used to treat HAE attacks in people ages 12 and older. Ecallantide blocks chemicals called kallikreins that contribute to HAE inflammation and pain.

Ecallantide is given as three injections under the skin during an HAE attack and may reduce swelling within two hours. Because there’s a 5 percent chance it could cause a severe allergic reaction, including anaphylaxis, ecallantide must be given by a healthcare professional in a medical setting.

Sebetralstat (Ekterly) is the first and only on-demand treatment for HAE. Like ecallantide, sebetralstat blocks plasma kallikrein to reduce the production of bradykinin.

Sebetralstat is approved for people ages 12 and older with HAE. Because it comes as a pill that’s taken by mouth, it may be easier for some people to use when symptoms begin.

During an HAE attack, sebetralstat may reduce symptoms in under two hours. If symptoms continue, a second dose may be taken as directed by your doctor. Sebetralstat is generally well tolerated, and most side effects are mild.

Long-Term HAE Prevention Treatments

Prophylactic (preventive) treatments for HAE help lower the risk of future swelling attacks. Using preventive treatment can reduce the need for on-demand medication and improve overall quality of life.

Some prophylactic treatments are taken on a regular schedule to help prevent attacks. Others are used only before a known trigger, such as surgery or a dental procedure.

Some C1INH treatments are used to help prevent HAE attacks rather than treat them after they begin. IV C1 inhibitor (Cinryze) and subcutaneous (under-the-skin) plasma-derived C1 inhibitor (Haegarda) replace the C1INH that your body doesn’t make or doesn’t make enough of.

Clinical trials have shown that regular treatment with preventive C1INH may reduce the number of HAE attacks by 95 percent. Some people may have few or no attacks while using preventive C1INH therapy.

Kallikrein inhibitors can also help prevent HAE attacks. Berotralstat (Orladeyo), an oral medication taken once a day, works by blocking plasma kallikrein. In research studies, berotralstat reduced the frequency of HAE attacks by 50 percent to 70 percent.

Donidalorsen (Dawnzera) belongs to a newer group of medicines called antisense oligonucleotides. It works by blocking the body’s instructions for making prekallikrein, a protein involved in producing bradykinin. With less prekallikrein available, the body makes less bradykinin, reducing the risk of swelling attacks.

Donidalorsen is approved for people ages 12 and older with HAE. It’s given as an injection every four to eight weeks and may prevent over 80 percent of HAE attacks. Some people may go long periods without having an attack.

Monoclonal antibodies are another option for preventing HAE attacks. Garadacimab-gxii (Andembry) and lanadelumab-flyo (Takhzyro) are given as injections under the skin and can be self-administered at home after proper training.

Monoclonal antibodies work by targeting proteins involved in the process that causes swelling and can significantly reduce the frequency of HAE attacks.

When To Seek Emergency Care for HAE

Always seek emergency care immediately if you or your child has signs of swelling in the tongue, throat, or airway during an HAE attack, even if you’ve used on-demand treatment. An HAE attack affecting the airway can be life-threatening. Call 911 right away if you think swelling is making it harder to breathe or swallow.

Don’t ignore or brush off symptoms of tongue or throat swelling, including:

• Gasping for air
• Wheezing
• Shortness of breath
• Trouble swallowing
• A swollen tongue

Talk to Your HAE Specialist

Your HAE specialist can help you build a treatment plan that fits your needs, goals, and daily life. Together, you may discuss:

• Which treatment you’re most comfortable using (for example, self-injection or an IV infusion)
• How often you have HAE attacks
• How often you may need to use the medication
• Whether other medications you take could interact with HAE treatment
• Whether you’re pregnant or planning to become pregnant

Even with an effective treatment plan, it’s important to keep your on-demand medication available and know when to seek emergency care.

Join the Conversation

On MyHAEteam, people share their experiences with hereditary angioedema, get advice, and find support from others who understand.

Which treatments do you use to manage hereditary angioedema? Let others know in the comments below.