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 called C1 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 to prevent an HAE attack from happening. It works by boosting plasma levels of C1 inhibitor in the body. Side effects may include:
NauseaHeadacheRashVomiting
Taking Cinryze could also increase your risk for blood clots. The FDA approved this medicine in 2008. 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 reactionsItchingRashDizzinessStuffy 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:
Blood clotsAllergic reactions
The FDA approved Berinert in 2009.
Ruconest
Ruconest increases 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:
HeadacheNauseaDiarrheaAn allergic reaction
The FDA approved this treatment in 2014. RELATED: 8 Common Types of Rashes
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:
HeadacheNauseaFeverDiarrheaStuffy noseInjection 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 reactionsHeadacheUpper respiratory infections
Takhzyro was approved by the FDA in 2018. RELATED: How to Get Rid of a Headache Fast
Firazyr
Firazyr is 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 reactionsFeverDizzinessNauseaHeadacheRashTwo much of an enzyme known as transaminase in your blood
The FDA approved Firazyr in 2011. RELATED: 11 Fascinating Facts About Body Temperature
Medicines That Don’t Work
Drugs that are used to treat an allergic reaction, such as antihistamines or epinephrine, won’t help symptoms 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. It’s important to work with your physician to come up with an approach that’s right for you.