Today, many healthcare providers opt for nonsteroidal HAE treatments. There are two kinds of hereditary angioedema (HAE) treatments: preventive and on demand. Preventive therapies Also known as prophylactic medication, this type of medication is taken regularly to help ward off an HAE attack. These drugs might also reduce the frequency and severity of your symptoms. On-demand Also known as acute treatments, this type of therapy is given to stop an attack in its tracks. All people with HAE should have access to an on-demand therapy. (1) Currently, the U.S. Food and Drug Administration (FDA) has approved seven medicines to prevent or treat HAE attacks. (2)
Treatments to Prevent HAE Attacks
Treatments used to prevent HAE symptoms include these medications:
Cinryze
Cinryze (C1 esterase inhibitor) is a plasma-derived concentrate of C1 esterase inhibitor that works by increasing the plasma levels of C1 inhibitor activity in your body. Cinryze is given as an intravenous (IV) infusion every three to four days to prevent an HAE attack in children ages 6 and older, teens, and adults. A healthcare provider can give you Cinryze, or you can administer it at home. In a clinical trial, most people who took Cinryze had fewer HAE attacks. Specifically, 20 out of 22 adults and teens reported a reduced number of episodes, including four who had no attacks. (3) The FDA approved Cinryze in 2008. The most common side effects include headache, nausea, rash, and vomiting. Blood clots are another serious risk that can occur with this medicine. (2,4)
Haegarda
In a clinical study, Haegarda reduced the number of HAE attacks by 95 percent. (5) Common side effects of Haegarda include injection-site reactions, itching, rash, dizziness, and stuffy nose. (6) The FDA approved this medicine in 2017. (2)
Takhzyro
This drug works by regulating the activity of an enzyme called plasma kallikrein in your body. In a clinical study that lasted six and a half months, 44 percent of participants who took Takhzyro every two weeks had no HAE attacks compared with 2 percent of those who took a placebo. (7) The FDA approved Takhzyro in 2018. The most common side effects are injection site reactions, headache, and upper respiratory infection. (2,8)
Berinert
Berinert (C1 esterase inhibitor) is used to treat acute facial, stomach, or throat HAE episodes in children and adults. It is given as an IV infusion, usually when you feel an attack coming on. Most people give themselves the Berinert infusion, but you can also get it in a healthcare setting. This medicine is a C1 inhibitor concentrate. It works by adding functioning C1 inhibitor to your body. In a clinical trial, nearly 70 percent of those treated with Berinert saw symptom relief within four hours compared with about 43 percent of those who took a placebo. (9) The FDA approved Berinert in 2009. The most common side effect of Berinert is that it can give you a bad taste in your mouth. Risks include blood clots and allergic reactions. (2,9)
Firazyr
Firazyr (icatibant) is an injectable medicine that you can give yourself when you feel an HAE attack coming on. It’s intended for people 18 and older to treat attacks that affect the skin, stomach, or throat. Firazyr is a bradykinin receptor antagonist. It works by reducing the activity of a protein peptide called bradykinin in your body. In a clinical trial, the median time it took people to achieve a 50 percent reduction in HAE symptoms was two hours for those treated with Firazyr versus nearly 20 hours for those given a placebo. (10) Side effects of Firazyr might include injection site reactions, fever, dizziness, nausea, headache, rash, or too much of an enzyme called transaminase in your blood. The FDA approved Firazyr in 2011. (2,10) Serious allergic reactions have happened in some people who took this medicine. For this reason, Kalbitor is given by a doctor or nurse in a healthcare setting. Other common side effects might include headache, nausea, fever, diarrhea, stuffy nose, and injection site reactions. Kalbitor is a plasma kallikrein inhibitor that works by controlling the protein kallikrein in your body. It’s been studied in people with skin, abdominal, and throat HAE attacks. Those treated with Kalbitor have shown significant improvement in symptoms at four hours. (11) The FDA approved Kalbitor in 2009. (2,11)
Ruconest
Ruconest (conestat alfa) used to treat HAE attacks in adults and children 13 years of age and older. You can give yourself the medicine intravenously when you feel an attack coming on. The drug is a plasma-free C1 esterase inhibitor. It works by increasing the functional C1 inhibitor level in your body. Ruconest may cause side effects like headache, nausea, or diarrhea. A severe allergic reaction is also possible with this medicine. This medicine might not be effective for helping HAE throat attacks. In a clinical trial, people treated with Ruconest saw symptom relief in 90 minutes compared with 152 minutes for those given a placebo. Later, an extension of the study showed that Ruconest helped relieve symptoms in just 75 minutes. (12) The FDA approved Ruconest in 2014. (2,12)
New HAE Treatments Under Study
Researchers are continuing to study new treatment options for HAE that could be more effective or easier to use. For example, in March 2020, scientists reported on positive findings from a phase 3 clinical trial that tested the oral medicine berotralstat (BCX7353). Results showed the drug significantly lowered the frequency and lessened the severity of swelling attacks in people with HAE. Berotralstat is a plasma kallikrein inhibitor. It’s currently under review for approval in the United States as a once-daily oral capsule to prevent HAE attacks. (13) Another oral treatment, known by its clinical name, PHA121, has shown promise in animals by reversing bradykinin-induced changes in blood pressure. (14) Another group of scientists is looking at how a one-time antibody-based gene therapy might help people with HAE. (15)
HAE Treatments That Don’t Work
It’s important to know that medicines used to treat swelling from allergies, such as corticosteroids, antihistamines, and epinephrine, won’t help HAE. Although an HAE episode may cause symptoms that mimic those of allergies, it’s not an allergic attack. (2)
Diet and Lifestyle Approaches for HAE
Adopting a healthy lifestyle won’t cure HAE, but it may help improve your symptoms.
HAE Food Triggers
Recent research has suggested that certain foods may trigger HAE attacks. (16) You might want to keep a daily diary to determine what foods are prompting your symptoms. Some common culprits include: (16)
SaladsFishFruits, such as tomatoes, apples, bananas, strawberries, kiwi, pineapples, and citrus fruitsOnionsGarlicMilk and cheeseChickenTree nutsShrimpLeeksAlcoholChiliBread
One way of approaching potential triggers is to try an anti-inflammatory diet, which excludes foods that promote swelling in the body. This type of eating plan typically emphasizes consuming whole, unprocessed foods that don’t contain additives or preservatives. (17)
Stress as an HAE Trigger
Emotional stress is another common trigger for HAE attacks. You can help control your stress levels by: (16,18)
Getting enough restExercising dailyPracticing meditation, deep breathing techniques, or yogaGetting a massage
Determining Which Treatment Is Right for You
Talk to your doctor about which medicines are most appropriate for treating your HAE attacks. Guidelines suggest that everyone with HAE should have an on-demand treatment plan. (1) If you have frequent episodes, your physician might suggest both a preventive and an on-demand therapy. The severity of your attacks and your general health will also factor into your doctor’s recommendations. The FDA-approved therapies are typically used to treat the most common kinds of HAE: type 1 and type 2. Scientists are still researching medicines that can help type 3 HAE, an extremely rare form of the disease. But some studies have shown that people with type 3 may also respond to the current FDA-approved HAE therapies. (2) Clinical trials are underway to uncover new medicines that could help HAE in the future.
