Table of ContentsView AllTable of ContentsPrescription MedicationsHow to TakeMedications to AvoidOTC Medications IneffectiveCosts

Table of ContentsView All

View All

Table of Contents

Prescription Medications

How to Take

Medications to Avoid

OTC Medications Ineffective

Costs

Hereditaryangioedema(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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An injector is prepared

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:

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 theseverity of symptoms.

Medications used for long-term prevention of symptoms include:

Medications used for short-term prevention include:

These treatments are sometimes prescribed before surgery, dental procedures, or any other anticipated event that could trigger symptoms of HAE.

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.

How to Administer a Subcutaneous Injection

Medications to Avoid That Can Trigger Symptoms

Many different medications cantrigger 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:

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 ofother types of angioedemaor 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 DeficiencyAcquired 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.

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.

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.

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.

7 SourcesVerywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am. 2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009Busse PJ, Christiansen SC, Riedl MA, et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract. 2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046American Academy of Allergy, Asthma, and Immunology.Treatment of hereditary angioedema.Betschel SD, Banerji A, Busse PJ, Cohn DM, Magerl M.Hereditary angioedema: a review of the current and evolving treatment landscape.J Allergy Clin Immunol Pract. 2023;11(8):2315-2325. doi:10.1016/j.jaip.2023.04.017Betschel SD, Caballero T, Jones DH, et ak.The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks.Allergy Asthma Clin Immunol.2024;20(1):43. doi:10.1186/s13223-024-00903-wLochbaum R, Hoffmann TK, Greve J, Hahn J.Concomitant medication in patients with bradykinin-mediated angioedema - there’s more than ACE inhibitors.J Dtsch Dermatol Ges.2023 Nov;21(11):1283-1289. doi:10.1111/ddg.15154Trainotti S, Johnson F, Hahn J, et al.Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH)-a bicenter retrospective study on diagnosis, course, and therapy.J Allergy Clin Immunol Pract. 2023;11(12):3772-3779. doi:10.1016/j.jaip.2023.09.003

7 Sources

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am. 2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009Busse PJ, Christiansen SC, Riedl MA, et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract. 2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046American Academy of Allergy, Asthma, and Immunology.Treatment of hereditary angioedema.Betschel SD, Banerji A, Busse PJ, Cohn DM, Magerl M.Hereditary angioedema: a review of the current and evolving treatment landscape.J Allergy Clin Immunol Pract. 2023;11(8):2315-2325. doi:10.1016/j.jaip.2023.04.017Betschel SD, Caballero T, Jones DH, et ak.The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks.Allergy Asthma Clin Immunol.2024;20(1):43. doi:10.1186/s13223-024-00903-wLochbaum R, Hoffmann TK, Greve J, Hahn J.Concomitant medication in patients with bradykinin-mediated angioedema - there’s more than ACE inhibitors.J Dtsch Dermatol Ges.2023 Nov;21(11):1283-1289. doi:10.1111/ddg.15154Trainotti S, Johnson F, Hahn J, et al.Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH)-a bicenter retrospective study on diagnosis, course, and therapy.J Allergy Clin Immunol Pract. 2023;11(12):3772-3779. doi:10.1016/j.jaip.2023.09.003

Verywell Health uses only high-quality sources, including peer-reviewed studies, to support the facts within our articles. Read oureditorial processto learn more about how we fact-check and keep our content accurate, reliable, and trustworthy.

Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am. 2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009Busse PJ, Christiansen SC, Riedl MA, et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract. 2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046American Academy of Allergy, Asthma, and Immunology.Treatment of hereditary angioedema.Betschel SD, Banerji A, Busse PJ, Cohn DM, Magerl M.Hereditary angioedema: a review of the current and evolving treatment landscape.J Allergy Clin Immunol Pract. 2023;11(8):2315-2325. doi:10.1016/j.jaip.2023.04.017Betschel SD, Caballero T, Jones DH, et ak.The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks.Allergy Asthma Clin Immunol.2024;20(1):43. doi:10.1186/s13223-024-00903-wLochbaum R, Hoffmann TK, Greve J, Hahn J.Concomitant medication in patients with bradykinin-mediated angioedema - there’s more than ACE inhibitors.J Dtsch Dermatol Ges.2023 Nov;21(11):1283-1289. doi:10.1111/ddg.15154Trainotti S, Johnson F, Hahn J, et al.Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH)-a bicenter retrospective study on diagnosis, course, and therapy.J Allergy Clin Immunol Pract. 2023;11(12):3772-3779. doi:10.1016/j.jaip.2023.09.003

Do T, Riedl MA.Current and emerging therapeutics in hereditary angioedema.Immunol Allergy Clin North Am. 2024;44(3):561-576. doi:10.1016/j.iac.2024.03.009

Busse PJ, Christiansen SC, Riedl MA, et al.US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema.J Allergy Clin Immunol Pract. 2021;9(1):132-150.e3. doi:10.1016/j.jaip.2020.08.046

American Academy of Allergy, Asthma, and Immunology.Treatment of hereditary angioedema.

Betschel SD, Banerji A, Busse PJ, Cohn DM, Magerl M.Hereditary angioedema: a review of the current and evolving treatment landscape.J Allergy Clin Immunol Pract. 2023;11(8):2315-2325. doi:10.1016/j.jaip.2023.04.017

Betschel SD, Caballero T, Jones DH, et ak.The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks.Allergy Asthma Clin Immunol.2024;20(1):43. doi:10.1186/s13223-024-00903-w

Lochbaum R, Hoffmann TK, Greve J, Hahn J.Concomitant medication in patients with bradykinin-mediated angioedema - there’s more than ACE inhibitors.J Dtsch Dermatol Ges.2023 Nov;21(11):1283-1289. doi:10.1111/ddg.15154

Trainotti S, Johnson F, Hahn J, et al.Acquired angioedema due to C1-inhibitor deficiency (AAE-C1-INH)-a bicenter retrospective study on diagnosis, course, and therapy.J Allergy Clin Immunol Pract. 2023;11(12):3772-3779. doi:10.1016/j.jaip.2023.09.003

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