11 Hereditary Angioedema Treatments: Berinert, Orladeyo, and More

Hereditary angioedema is a rare, genetic health condition. It often occurs when a protein (called a “C1 inhibitor”) is dysfunctional or missing. Normally, this protein helps regulate the flow of fluids through your blood vessels. When it’s missing or malfunctioning, the flow is disrupted and fluid builds up.

This process can lead to severe swelling in the skin, hands and feet, intestinal tract, and upper airway tissues. Intestinal tract swelling can lead to cramping or an obstruction. If the airway swells, breathing can be affected.

Several medications are available to manage angioedema attacks like these. Some are useful to treat attacks, while others are designed to prevent them. Here, we’ll review 11 hereditary angioedema treatments and when they can be used.

1. Berinert

Berinert (C1 esterase inhibitor, human) is a medication that’s injected intravenously (IV) to treat hereditary angioedema attacks. It treats swelling in the face, stomach, or throat in children and adults. It replaces the C1 inhibitor protein that might be missing or malfunctioning. It also works quickly — typically within an hour. 

If you need Berinert, your healthcare professional will initially be in charge of giving it to you. You have the option to administer it to yourself after some training.

You shouldn’t receive Berinert to prevent angioedema attacks, though. It hasn’t been studied for this purpose.

2. Cinryze

Cinryze is another human C1 inhibitor that’s injected into a vein. Similar to Berinert, it replaces the C1 inhibitor protein that might be deficient in the body. But unlike Berinert, it’s not used to treat attacks. It’s a maintenance, preventative medication. It’s given every 3 or 4 days to prevent angioedema attacks in people ages 6 years and older.

Talk to your healthcare professional to see if you qualify for a prevention medication such as Cinryze. You might be a good candidate based on how often you get attacks and how severe they are.

3. Haegarda

Haegarda is another injectable human C1 inhibitor that prevents hereditary angioedema attacks. It works by replacing the missing C1 inhibitor protein. 

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What makes Haegarda unique is that it’s injected under the skin. This is easier to administer than an IV injection — especially when you’re giving it to yourself. 

Haegarda is typically prescribed to people ages 6 and older at a dose that’s calculated based on your body weight. It’s administered every 3 to 4 days — similar to Cinryze — to prevent attacks. In clinical trials, Haegarda proved to be very effective at reducing swelling.

4. Ruconest

Ruconest is a recombinant C1 inhibitor. It’s an IV injection given over 5 minutes to treat sudden angioedema attacks in adults and teens ages 13 and older. Your healthcare professional can inject it for you, but in many cases, you can also receive training to learn how to inject it yourself.

However, self-injecting an IV medication can be daunting. A Ruconest vein finder is one helpful resource you can use to make the administration process easier. It’s a special light that you can shine on your skin to identify where you should place the needle.

One weight-based dose of Ruconest was found to treat roughly 97% of swelling attacks in clinical studies. But if one dose doesn’t improve symptoms, you can administer a second dose shortly after. You shouldn’t administer more than two doses in a 24-hour period.

Good to know: Human C1 inhibitors — such as Berinert, Cinryze, and Haegarda — are made and purified from human blood. But Ruconest is made from rabbit milk. You shouldn’t use it if you’re allergic to rabbits.

5. Kalbitor

Kalbitor (ecallantide) is an under-the-skin injectable that treats angioedema attacks in people ages 12 and older. It belongs to a class of medications called plasma kallikrein inhibitors. These medications block a protein, called plasma kallikrein, to make sure it doesn’t make too much bradykinin. Bradykinin is thought to be responsible for the swelling in an angioedema attack.

Kalbitor should provide relief within 4 hours of an injection. But if you continue to experience symptoms, your healthcare professional may ask you to receive a second dose within 24 hours.

Keep in mind: Some people have reported anaphylaxis after Kalbitor injections, which is a life-threatening allergic reaction. That’s why Kalbitor can only be given by a trained healthcare professional at a clinic or hospital. This is to ensure you’re closely monitored in a healthcare setting after receiving the injection and given the support you may need.

6. Orladeyo

Orladeyo (berotralstat) is another plasma kallikrein inhibitor. It prevents angioedema attacks in people ages 12 and older. An oral capsule taken once daily, it’s a good option for those who don’t want to receive injections. 

Since it’s taken by mouth, Orladeyo can cause stomach pain, vomiting, or diarrhea. It should be taken with food to prevent stomach upset as much as possible. And you shouldn’t take more than one capsule per day, as extra doses could affect your heart rhythm.

7. Takhzyro

Takhzyro (lanadelumab) is also a plasma kallikrein inhibitor. It prevents angioedema attacks in people ages 12 and older.

This medication may be easier on your schedule, too. It’s not administered every day or every few days. It’s injected under the skin every 2 to 4 weeks, depending on how well controlled your attacks are.

What’s more, Takhzyro is also available as a single-dose prefilled syringe. Compared to many other medications on this list, Takhzyro may be easier and more convenient to administer yourself.

8. Ekterly

Ekterly (sebetralstat) is the first pill available to treat hereditary angioedema attacks. Until now, all on‑demand options required an injection. Approved for people ages 12 and older, it works by blocking plasma kallikrein to stop swelling from getting worse.

In clinical studies, people who took Ekterly at the first sign of an attack experienced faster relief and less severe symptoms compared to placebo. The most common side effect was headache. 

Experts recommend taking 2 tablets (600 mg) as soon as you notice symptoms of an attack. A second dose can be taken 3 hours later if symptoms persist.

9. Firazyr

Firazyr (icatibant) belongs to a different class of medications. It’s a bradykinin receptor antagonist. It works by blocking the activity of bradykinin, a common culprit behind swelling attacks. Firazyr can treat angioedema attacks in adults.

Normally, one dose of Firazyr should improve at least half of your symptoms after 2 hours. But it can take up to 8 hours for symptoms to completely resolve. You can give up to 3 additional injections in 24 hours if symptoms continue. The additional doses should be spaced apart at least 6 hours.

If you receive a prescription for Firazyr, you should inject it under the skin in the stomach area. It also comes in a prefilled syringe for convenient, at-home injections.

10. Andembry

Andembry (garadacimab-gxii) is a preventive hereditary angioedema treatment for adults and kids ages 12 and older. It targets a protein called factor XIIa, which plays a key role in triggering hereditary angioedema attacks. It’s the first medication of its kind, and it’s administered as a once‑monthly injection under the skin.

Clinical trials showed that Andembry substantially cut down the number of attacks people experienced. In fact, many people stayed completely attack‑free while using it. The most common side effects were injection site pain and cold‑like symptoms, but these were relatively mild overall.

Because it only requires a monthly shot, Andembry offers a simple option for long‑term prevention. You can administer the medication to yourself using an autoinjector or prefilled syringe.

11. Dawnzera

Dawnzera (donidalorsen) is the first and only RNA-targeted therapy approved to prevent hereditary angioedema attacks in people ages 12 and older. It works by lowering levels of plasma prekallikrein (PKK), another protein involved in swelling.

Dawnzera is administered using an autoinjector every 4 or 8 weeks — making it one of the least frequent dosage options for hereditary angioedema prevention. Most people can self‑inject their doses under the skin after some basic training.

In placebo-controlled studies, Dawnzera cut monthly attack rates by more than 80% and reduced moderate-to-severe attacks by about 90%. The most common side effects are injection site reactions, mild infections, and stomach discomfort.

How do you find out which hereditary angioedema treatment is right for you?

Your healthcare professional will help you identify one or more treatments that are appropriate for you based on your needs and preferences. 

If you need a medication to treat angioedema attacks, you’ll receive an IV or under-the-skin injectable product. You should also be trained on how to use it. If you qualify to take preventative medication, you may receive an injectable or oral medication.

If you’re living with hereditary angioedema, talk to your healthcare professional about which medication is best for you.

Can hereditary angioedema be cured?

There’s currently no cure for hereditary angioedema. But there are multiple options to prevent and treat swelling attacks and other symptoms. With the right preventative treatment, people can be free of attacks for many years. 

What’s more, there are additional newer treatments being investigated in clinical trials as longer-term prevention strategies.

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Written by:

Hindu Rao, PharmD, APh, BCACP

Hindu Rao, PharmD, APh, BCACP is a clinical assistant professor of pharmacy practice at Chapman University School of Pharmacy and a clinical pharmacist at Providence Medical Foundation. She specializes in chronic disease management and has her board certification in ambulatory care.

Edited by:

Joshua Murdock, PharmD, BCBBS

Joshua Murdock, PharmD, BCBBS, is a licensed pharmacist in Arizona, Colorado, and Rhode Island. He has worked in the pharmacy industry for more than 10 years and currently serves as a pharmacy editor for GoodRx.

Reviewed by:

Patricia Pinto-Garcia, MD, MPH

Patricia Pinto-Garcia, MD, MPH, is a medical editor at GoodRx. She is a licensed, board-certified pediatrician with more than a decade of experience in academic medicine.

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