Hereditary Angioedema (HAE) Medications to Better Manage Episodes
Medically reviewed by Kristie Reed, PharmDMedically reviewed by Kristie Reed, PharmD
Hereditary angioedema (HAE) is a lifelong disorder that requires ongoing therapy to prevent episodes. Acute treatment during an episode can also help the episode resolve and prevent symptoms from becoming severe.
Several medications are used for HAE treatment. Some are prescribed for long-term symptom prevention, some for short-term prophylaxis (preventing symptoms prior to surgery or another anticipated trigger), and some during an episode.
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Prescription Medications
You may need more than one prescription for treating hereditary angioedema, with a medication for acute treatment (during an episode) and another for long-term use. Generally, these medications would be prescribed by a medical specialist, such as an immunologist, who has experience in treating HAE.
C1 inhibitor protein mediates both the body’s immune and fluid responses. With HAE, this protein is low and does not function properly, leading to swelling. Many of the medications used to treat HAE affect the C1 inhibitor protein.
Medications used for the treatment of episodes include:
Berinert, a C1 Inhibitor (human), works by replacing C1 inhibitor, which is deficient in HAE.
Firazyr (icatibant) inhibits bradykinin, a protein that plays a role in fluid movement from blood vessels and swelling.
Kalbitor (ecallantide) binds to kallikrein, a protein that plays a role in inflammation.
Ruconest, a C1 esterase inhibitor (recombinant), works by preventing the breakdown of C1 inhibitor, which is deficient in HAE.
Fresh-frozen plasma (FFP) is a treatment sometimes used when the medications used to treat episodes of HAE are not available. This treatment can provide C1 inhibitor from donor plasma.
Starting treatment as soon as possible during the episode can shorten its length and reduce the severity of symptoms.
Medications used for long-term prevention of symptoms include:
Cinryze, a C1 Inhibitor (human), works by replacing C1 inhibitor, which is deficient in HAE.
Danocrine (danazol) reduces inflammation and swelling by modifying hormone levels.
Haegarda, a C1 esterase Inhibitor (human), works by preventing the breakdown of C1 inhibitor, which is deficient in HAE.
Orladeyo (berotralstat) decreases kallikrein activity to reduce inflammation.
Takhzyro (lanadelumab-flyo) binds to kallikrein, a protein that plays a role in inflammation.
Winstrol (stanozolol) reduces inflammation and swelling by modifying hormone levels.
Medications used for short-term prevention include:
Plasma-derived C1NH therapies (Berinert, Cinryze, Haegarda)
Anabolic androgen
These treatments are sometimes prescribed before surgery, dental procedures, or any other anticipated event that could trigger symptoms of HAE.
| Use | How to take | Dosing schedule | Potential side effects |
Ruconest | During an episode | Self-administered intravenous (IV) | Up to twp injections | Allergic reactions: rapid heartbeat, wheezing, trouble breathing |
Berinert | During an episode or short-term prevention | Self-administered intravenous (IV) | For prophylaxis, injection every three to four days. For acute, up to two doses | Rash, dizziness, swelling |
Kalbitor | During an episode | Healthcare provider–administered intravenous (IV) | Three injections | Stomach upset, fever |
Firazyr | During an episode | Self-administered subcutaneously (under the skin) | Up to three injections | Skin bruising, dizziness, nausea |
Orladeyo | Prevention | By mouth | Daily | Vomiting, fatigue, headaches |
Takhzyro | Prevention | Self-administered subcutaneously | Every two to four weeks | Skin bruising, rash, respiratory infection |
Cinryze | Long-term or short-term prevention | Self-administered intravenous (IV) | Every three or four days, depending on dose | Nausea, fever, headaches |
Haegarda | Long-term or short-term prevention | Self-administered subcutaneously | Two times a week | Skin reactions, rash, dizziness |
Danocrine | Prevention | By mouth | Two or three times a day to start. then may reduce dosage | Coughing blood, allergic reactions: wheezing, hives |
Winstrol | Prevention | By mouth | Daily | Acne, headaches, difficulty sleeping |
Anabolic androgen | Short-term prevention | By mouth | Daily | Excess hair growth |
How to Take HAE Medications
For some HAE therapies, you would need instructions and a demonstration of how to use them, especially if you are going to give yourself or your child an intravenous or subcutaneous injection.
If you have any questions or concerns about how to use your medication, don’t hesitate to ask. The healthcare providers managing your treatments are aware that you may have questions and need help in self-administering these treatments.
You may feel more comfortable having your providers watch you self-administer your first dose before you start doing it on your own at home.
Learn More: How to Administer a Subcutaneous Injection
Medications to Avoid That Can Trigger Symptoms
Many different medications can trigger symptoms of HAE. It’s important to avoid these medications. Be sure to tell all of your healthcare providers and your pharmacist about your condition.
Medications to avoid include:
Angiotensin converting enzyme (ACE) inhibitors: Such as Lotensin (benazepril), Capoten (captopril), Vasotec (enalapril), Zestril (lisinopril), and Prinivil (lisinopril)
Estrogen-containing therapies: Oral contraceptives, hormone replacement therapy
Angiotensin 2 receptor blockers: Such as Cozaar (losartan), Benicar (olmesartan), and Avapro (irbesartan)
Renin inhibitor: Tecturna (aliskiren)
Angiotensin receptor neprilysin inhibitor (ARNI): Entresto (sacubitril/valsartan)
Are OTC Medications Effective for HAE?
Over-the-counter (OTC) treatments are not effective for the treatment of ongoing HAE symptoms or for the prevention of HAE symptoms.
Sometimes, the symptoms of HAE can appear similar to symptoms of other types of angioedema or may look like allergic reactions. Allergic reactions and angioedema that are not hereditary are often effectively treated with OTC antihistamines and steroids.
However, the underlying physical cause of HAE differs from these other disorders, and these treatments would not be effective in preventing or alleviating an episode.
HAE Medication Costs and Affordability
The medications used for treating HAE can vary in price. If you are paying out of pocket (without the aid of health insurance coverage), the price of your treatment would vary depending on your dose and the region where you live. If you have health insurance coverage, your health insurance may pay the total cost of your treatment, or you may be expected to pay a portion of the cost.
Sometimes, health insurance coverage may require that certain medications are prescribed when more than one option is considered equivalent in treatment efficacy. Usually, they will make an exception if there’s a special reason why you and your healthcare provider decide that you should avoid the use of the other medications (such as a drug allergy or intolerance).
It’s important that you check with your health insurance provider to see what your out-of-pocket costs will be as you and your healthcare provider discuss your treatment options. Your healthcare provider’s office might already have a process in place for looking into this matter.
Acquired Angioedema Due to C1 Inhibitor Deficiency
Acquired angioedema due to C1 inhibitor deficiency (AAE-C1-INH) is a rare disorder associated with autoimmune diseases and disorders and malignancies of the lymphatic white blood cells. It is not hereditary and appears later in life. It shares the underlying cause of angioedema with HAE and the angioedema symptoms may be treated with some of the same medications as HAE.
In addition, a person with AAE-C1-INH would also be treated for the underlying condition that produced it. This may include medications to suppress the immune system or treat the malignant cells.
Summary
Hereditary angioedema (HAE) is a chronic disease, but treatments can help you manage and prevent the symptoms. You can learn how to take medications as needed and when it’s important for you to call for medical attention during an episode.
Some medications are prescribed for long-term symptom prevention, some for short-term prevention, and some for use during an episode. They may be self-administered intravenously, subcutaneously, or orally, or administered by healthcare providers.
Over-the-counter medications used for other types of angioedema, such as antihistamines, are not effective for hereditary angioedema, as it has a different underlying cause.
Additionally, lifestyle measures, such as preventing triggers and avoiding triggering medications are an important part of managing this condition.
Read the original article on Verywell Health.
