Hereditary Angioedema

Your Guide to Understanding Hereditary Angioedema (HAE) Inhibitors

Hereditary angioedema (HAE) is a rare, genetic disease that causes episodes of swelling in different areas of the body. Medication can help prevent attacks.

Julie Marks
By Medically Reviewed by
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Medically Reviewed
illustration syringe and vials
When taken preventively HAE inhibitors can help ward off attacks. bob体育在线

There’s no cure forhereditary angioedema(HAE), but there is medication available to prevent and treat attacks.

Preventive, also called “prophylactic,” drugs are taken on a regular basis to reduce the frequency of HAE events. On-demand, or “acute,” medicines are used to address symptoms once an attack starts.

The U.S. Food and Drug Administration (FDA) has approved seven therapies for HAE. These medicines boost, regulate, or inhibit certain processes in the body that trigger an HAE attack.

C1 Inhibitor Boosting Medication

C1 inhibitor medicines raise levels of a protein in your body calledC1 inhibitor. When C1 inhibitor levels are too low or the protein doesn’t function like it should, an HAE attack can occur. There are several types of this medication.

Cinryze

Cinryze is a prescription medicine that’s given as an intravenous (IV) infusion every three to four days toprevent an HAE attackfrom happening.

It works by boosting plasma levels of C1 inhibitor in the body.

Side effects may include:

  • Nausea
  • Headache
  • Rash
  • Vomiting

Taking Cinryze could also increase your risk for blood clots.

2008年FDA批准这种药。

Haegarda

Haegarda is a plasma-derived concentrate of C1 esterase inhibitor that raises the body’s working C1 inhibitor levels.

It’s an injectable drug that’s used as a preventive therapy. You can give yourself the injection at home, usually twice a week.

Some side effects may include:

  • Injection reactions
  • Itching
  • Rash
  • Dizziness
  • Stuffy nose

The FDA approved Haegarda in 2017.

Berinert

Berinert is a C1 inhibitor booster that’s an acute treatment, which means it’s used when you feel an attack starting. It works by adding functioning C1 inhibitor to your body.

The medicine is delivered through an IV infusion. Usually, it’s given at home, but you can also receive it in a healthcare facility.

Some people experience a bad taste in their mouth when taking this drug. Other risks include:

The FDA approved Berinert in 2009.

Ruconest

Ruconestincreases the functional C1 inhibitor level in your body. It’s given as an acute treatment through an IV, when you feel an attack starting.

Some side effects of Ruconest are:

  • Headache
  • Nausea
  • Diarrhea
  • An allergic reaction

The FDA approved this treatment in 2014.

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Kallikrein Inhibiting Medication

Kallikrein inhibitors act on an enzyme in the blood known as kallikrein. When C1 inhibitor levels are too low, kallikrein becomes uncontrolled, which sets off a cascade of events that prompt an HAE attack.

Kalbitor

Kalbitor (ecallantide)is an on-demand injection that’s used to treat HAE attacks. It works by controlling kallikrein in the body.

Because it poses a serious risk for an allergic reaction, Kalibitor is administered in a healthcare setting.

Some common side effects are:

  • Headache
  • Nausea
  • Fever
  • Diarrhea
  • Stuffy nose
  • Injection site reactions

The FDA approved the medicine in 2009.

Takhzyro

Takhzyro is a preventive treatment that helps regulate the activity of kallikrein in the body.

It’s an injectable drug that you can give to yourself once every two weeks.

The most common side effects are:

  • Injection site reactions
  • Headache
  • Upper respiratory infections

Takhzyro was approved by the FDA in 2018.

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Bradykinin Antagonists

Bradykinin antagonists help reduce the activity of a peptide called bradykinin. Too much bradykinin can lead to fluid buildup in the body’s tissues, which causes HAE swelling attacks.

Firazyr

Firazyris an injectable, acute medicine that’s given when you feel an attack starting.

It works by reducing the activity of bradykinin in your body.

Side effects might include:

  • Injection site reactions
  • Fever
  • Dizziness
  • Nausea
  • Headache
  • Rash
  • Two much of an enzyme known as transaminase in your blood

The FDA approved Firazyr in 2011.

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Medicines That Don’t Work

Drugs that are used to treat an allergic reaction, such asantihistaminesorepinephrine, won’t helpsymptoms of HAE.

Additionally, some medicines, such as angiotensin-converting enzyme (ACE) inhibitors and oral contraceptive pills, may trigger an HAE event. You’ll want to avoid these medicines if you have HAE.

What You Should Know About Treatments for HAE

The severity and frequency of HAE symptoms vary from person to person. Some people only have a couple of attacks a year, while others have one or more a week.

Your treatment plan will depend on how often and how severe your symptoms are. Your doctor might recommend both a preventive and an on-demand therapy.

这是小孩ortant to work with your physician to come up with an approach that’s right for you.

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