There are several different medications that can help to prevent the frequency of HAE attacks, and treat symptoms should they occur.
Hereditary angioedema (HAE) causes swelling and discomfort of the skin, gastrointestinal tract, and upper airway. Swelling that occurs in your upper airway can be life threatening.
Some may mistake allergic swelling for HAE swelling. These conditions have distinct symptoms, treatments, and risks. As a result, it is important to clarify the diagnosis as a first step before getting treatment.
Doctors can differentiate HAE attacks from other causes of swelling through careful review of symptoms and their timing as well as blood sample analysis.
In this article, we detail the different treatment options for HAE.
While there’s no cure for HAE, there are many ways to prevent or subdue swelling attacks. In recent years, eight new prescription medications have become available in the United States to help you manage HAE.
These medications treat HAE differently, and their administration and dosing recommendations vary. Four of the medications prevent symptoms, and four treat acute HAE symptoms. Children can use some of these medications, but a few are only for adolescents and adults.
Management plans for HAE vary from person to person. Your doctor can help you determine the best course of treatment for you. Your plan may involve taking preventive medications or taking medications at the first sign of an HAE attack.
The following medications can prevent symptoms from occurring or reduce their severity.
There are four preventive treatments for HAE. These types of medications are also called prophylactics. Doctors administer them in various ways, and some may work for children.
C1 esterase inhibitor intravenous, human (Cinryze)
This prophylactic medication is available for adults and children over 6 years of age. It is known as a C1 esterase inhibitor. You can administer this medication intravenously in your arm at home after training or at your doctor’s office.
Your dose will range from 500 to 2,500 units. You must receive a dose every three or four days to prevent HAE attacks. It takes between 5 and 10 minutes to receive the dose.
C1 esterase inhibitor subcutaneous, human (Haegarda)
This is also a C1 esterase inhibitor and is suitable for people over 6 years of age. People may inject this medication under your skin in several areas of your body, including your abdomen, upper arms, and thighs.
You can inject it yourself at home after receiving instruction from your doctor. You’ll need the medication every 3-4 days. The dose varies based on your body weight and treatment plan.
This is a kallikrein inhibitor to prevent HAE attacks in adults and children over age 12. After training from a healthcare professional, you can inject it at home under your skin.
The beginning dose is typically 300 milligrams (mg) every 2 weeks. You may be able to take the medication every four weeks if you don’t experience an HAE attack for six months or longer.
This prophylactic medication is safe for use in people over the age of 12 at a dosage of 150 mg per day.
Research shows that taking this medication once daily can
There are four medications you can use during an HAE attack. You can use some at home, while a healthcare professional must administer others.
Remember that swelling in your throat can be life threatening. Seek immediate medical attention in addition to using these medications if you experience this.
C1 esterase inhibitor, human (Berinert)
This is a C1 inhibitor that you administer intravenously at home or in a doctor’s office. You take it when you experience the first symptoms of an HAE attack. Adults and children can use the medication, and your dose depends on your body weight.
Icatibant injection (Firazyr)
This B2 bradykinin receptor antagonist is for use in adults only. You can inject the dose at home under the skin of your abdomen when you experience signs of an acute HAE attack.
Sometimes, you may need to take more than one dose of the medication during an attack. You should wait 6 hours between doses and administer the medication no more than three times within a 24-hour window.
Doctors may use this medication when you experience signs of an HAE attack. It is a kallikrein inhibitor that is only safe for healthcare professionals to administer. They will typically inject a solution under the skin of your abdomen, upper arm, or thigh. Your doctor can monitor any possible allergic reactions to the medication after administration.
C1 esterase inhibitor, recombinant (Ruconest)
This is a C1 inhibitor that you receive intravenously. Adults and adolescents can self-administer this medication at home when experiencing signs of an HAE attack. You can also receive it under the supervision of a healthcare professional.
The average dose is 50 U/kg, and it takes about 5 minutes to receive the injection.
It’s not always possible to prevent an HAE attack, but by knowing common triggers, your can work with your doctor to create a prophylaxis plan to reduce the chances of an attack during exposure to triggers.
Triggers for HAE attacks may include:
- stress, either emotional or physical
- dental work and other surgeries
- illnesses, such as the common cold and flu
- menstruation and pregnancy
- repetitive physical activities like typing, writing and doing yard work
- certain medications like oral contraceptives, hormone replacement therapy, and ACE inhibitors
Talk with your doctor about managing these triggers and seeking short-term treatments for situations like dental work, surgery, and pregnancy.
Below are frequently asked questions about HAE attacks.
What drugs treat HAE?
The FDA approves the following drugs for the treatment of HAE.
- Berinert (C1 esterase inhibitor, human)
- Cinryze (C1 esterase inhibitor intravenous, human)
- Haegarda (C1 esterase inhibitor subcutaneous, human)
- Firazyr (Icatibant)
- Kalbitor (Ecallantide)
- Orladeyo (Berotralstat)
- Ruconest (C1 esterase inhibitor, recombinant)
- Takhzyro (Lanadelumab-flyo)
What is the fastest way to stop angioedema?
Doctors can administer several medications to stop acute attacks. These include C1 esterase inhibitors (Berinert and Ruconest), Ecallantide (Kalbitor), and Icatibant injections (Firazyr).
How long do HAE attacks last?
HAE attacks can last for 2-5 days. The frequency of these attacks may reduce with preventive therapy.
What triggers HAE?
Specific HAE triggers can vary between cases. However, the most common triggers are emotional stress, physical stress, and dental procedures.
There are several treatment options available to prevent or reduce HAE attacks. Talk with your doctor about the best HAE treatment plan for you. Your doctor may also recommend implementing certain lifestyle modifications to help you avoid HAE triggers. Together, medications and avoiding triggers can help you effectively manage your condition.