8 Hereditary Angioedema Treatments: Berinert, Orladeyo, and More
Key takeaways:
Hereditary angioedema is a genetic health condition that causes swelling in certain parts of the body. There are several medications available to treat and prevent angioedema attacks.
Berinert and Ruconest, which both contain a C1 esterase inhibitor, are intravenous (IV) injectables that treat hereditary angioedema attacks. Kalbitor (ecallantide) and Firazyr (icatibant) are also used to treat attacks, but they’re injected under the skin.
Cinryze is an IV C1 esterase inhibitor used to prevent attacks. Haegarda (C1 esterase inhibitor) and Takhzyro (lanadelumab) are also used for prevention, but they’re injected under the skin. Orladeyo (berotralstat) is an oral capsule meant to prevent attacks.
Table of contents
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. Luckily, airway-related swelling is rare and only makes up 1% to 3% of cases.
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 eight 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 provider 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 provider 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.
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 years 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 provider 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 years 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 provider 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 provider 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. This medication is the only drug on this list that’s 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 years 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. 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.
How do you find out which hereditary angioedema treatment is right for you?
Your healthcare provider 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.
Treatment of angioedema attacks | |||
Name | Class | Route | Approved ages |
Berinert | Human C1 inhibitor | IV injection | Kids and adults |
Ruconest | Recombinant C1 inhibitor | IV injection | 13 and older |
Kalbitor | Plasma kallikrein inhibitor | Under-the-skin injection | 12 and older |
Firazyr | Bradykinin receptor antagonist | Under-the-skin injection | 18 and older |
Prevention of angioedema attacks | |||
Name | Class | Route | Approved ages |
Cinryze | Human C1 inhibitor | IV injection | 6 years and older |
Haegarda | Human C1 inhibitor | Under-the-skin injection | 6 years and older |
Takhzyro | Plasma kallikrein inhibitor | Under-the-skin injection | 12 years and older |
Orladeyo | Plasma kallikrein inhibitor | Oral capsule | 12 years and older |
If you’re living with hereditary angioedema, talk to your healthcare provider 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.
The bottom line
Hereditary angioedema can cause swelling in the hands and feet, intestinal tract, and airways. It’s important to have a hereditary angioedema treatment — such as Berinert, Ruconest, or Firazyr — on-hand to treat sudden attacks. If needed, your healthcare provider may also suggest taking a maintenance medication — such as Cinryze, Haegarda, or Takhzyro — to prevent attacks over time. Talk to your healthcare provider about the best option(s) for you.
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References
Abdulkarim, A., et al. (2023). Hereditary angioedema. StatPearls.
American Academy of Allergy, Asthma & Immunology. (2022). Treatment of hereditary angioedema.
BioCryst Pharmaceuticals Inc. (2022). Orladeyo- berotralstat hydrochloride capsule [package insert].
Caballero, T. (2021). Treatment of hereditary angioedema. Journal of Investigational Allergology and Clinical Immunology.
CSL Behring GmbH. (2021). Berinert- human c1-esterase inhibitor [package insert].
CSL Behring GmbH. (2022). Haegarda c1 esterase inhibitor subcutaneous (human)- Human c1-esterase inhibitor [package insert].
National Organization for Rare Disorders. (2021). Hereditary angioedema.
Takeda Pharmaceuticals America, Inc. (2022). Firazyr- icatibant acetate injection, solution [package insert].
Takeda Pharmaceuticals America, Inc. (2022). Kalbitor- ecallantide injection, solution [package insert].
Takeda Pharmaceuticals America, Inc. (2023). Cinryze- human c1-esterase inhibitor injection, powder, lyophilized, for solution; Cinryze- human c1-esterase inhibitor [package insert].
Takeda Pharmaceuticals America, Inc. (2023). Doctor discussion guide.
Takeda Pharmaceuticals America, Inc. (2023). Takhzyro- lanadelumab-flyo solution; Takhzyro- lanadelumab-flyo injection, solution [package insert].
Tjoapack Netherlands BV. (2022). Ruconest- c1 esterase inhibitor recombinant injection, powder, for solution [package insert].