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Approved HAE Treatments

With eight products currently approved by the FDA for preventing and treating Hereditary Angioedema (HAE) attacks, and two therapies approved for generic use, patients and their physicians have options for developing an HAE treatment plan tailored to meet each patient's unique needs.

BERINERT® is a plasma-derived C1 Esterase Inhibitor (Human) indicated for the treatment of HAE attacks in adult and pediatric patients. Berinert is delivered intravenously and is approved for self-administration. For more information visit: www.berinert.com.
Acute attacks
Adults and children
Self-administered
Intravenous
CINRYZE® is a C1 Esterase Inhibitor indicated for routine prevention against HAE attacks in adults, adolescents and pediatric patients (6 years of age and older). Cinryze is delivered intravenously and is approved for self-administration. For more information visit: www.cinryze.com.
Prevention
medicine
Adults and children
Self-administered
Intravenous
FIRAZYR® is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of HAE in adults 18 years of age and older. Firazyr is delivered by subcutaneous injection and is approved for self-administration. For more information visit: www.firazyr.com.
Acute attacks
Adults
Self-administered
Subcutaneous
injection
HAEGARDA® is a plasma-derived concentrate of C1 Esterase Inhibitor (Human) indicated for routine prevention of HAE attacks in patients 6 years of age and older. Heagarda is delivered by subcutaneous injection and is approved for self-administration. For more information visit: www.haegarda.com.
Prevention
medicine
Adults and children
Self-administered
Subcutaneous
injection
ICATIBANT (GENERIC) is a bradykinin B2 receptor antagonist indicated for treatment of acute attacks of Hereditary Angioedema (HAE) in adults 18 years of age and older. Generic Icatibant is delivered by subcutaneous injection and is approved for self-administration. Multiple manufacturers offer Generic Icatibant.
Acute attacks
Adults
Self-administered
Subcutaneous
injection
KALBITOR® is a plasma kallikrein inhibitor indicated for treatment of acute attacks of HAE in patients 12 years of age and older. Kalbitor is delivered by subcutaneous injection and must be administered by a healthcare professional. For more information go to: www.kalbitor.com.
Acute attacks
Adults and
adolescents
Administered by
healthcare professional
Subcutaneous
injection
ORLADEYO™ is a plasma kallikrein inhibitor indicated for routine prevention of HAE attacks in adults and pediatric patients 12 years and older. ORLADEYO is taken orally once daily. For more information go to: www.orladeyo.com.
Prevention
medicine
Adults and
adolescents
Self-administered
Oral capsule
RUCONEST® is a plasma free recombinant C1-Inhibitor concentrate for treating HAE attacks in adults and adolescents. Ruconest is delivered intravenously and is approved for self-administration. For more information go to: www.ruconest.com.
Acute attacks
Adults and
adolescents
Self-administered
Intravenous
TAKHZYRO™ is a plasma kallikrein inhibitor (monoclonal antibody) indicated for routine prevention of HAE attacks in patients 12 years and older. TAKHZYRO is administered by subcutaneous injection and is approved for self-administration. For more information go to: www.takhzyro.com.
Prevention
medicine
Adults and
adolescents
Self-administered
Subcutaneous
injection

Anabolic steroids

Before there were approved treatments for HAE, anabolic steroids (also known as androgens) such as danazol, oxandrolone and stanozolol were the most commonly prescribed preventive HAE therapies. While anabolic steroids have been shown to be useful, they are not well-tolerated by many women, directly linked to liver toxicity, and can cause an increase in cholesterol levels. In addition, these drugs should not be used to treat children. Research indicates that patients treated with anabolic steroids can experience breakthrough laryngeal or abdominal attacks that require hospitalization.

IMPORTANT: Patients with the slightest hint of throat swelling should seek immediate treatment to ensure that their airway is not compromised. Medicines used to treat swelling from allergies, such as corticosteroids (prednisone), antihistamines, and epinephrine are NOT effective treatments for HAE.

US HAEA Medical Advisory Board 2020 Guidelines for the
Management of Hereditary Angioedema

The treatment of HAE has undergone dramatic changes as newer medicines have become available in recent years. Optimal care of HAE patients requires a comprehensive management plan. The US HAEA Medical Advisory Board has developed evidence-based recommendations for the diagnosis and treatment of HAE. If your doctor is unsure how to treat you or your loved one, please contact an HAEA Patient Advocate, who can help you get in touch with an HAE expert physician in your area.

TREATMENT GUIDELINES

Recommendations regarding a comprehensive care plan for HAE, including development of an overall management plan, treatment of angioedema attacks, prophylactic treatment, and patient monitoring, have been published in the peer-reviewed Journal of Allergy and Clinical Immunology: In Practice.

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Considerations For Pregnant
Women With HAE

According to recent studies, pregnancy may aggravate or alleviate symptoms in equal proportion. It is important to create an individualized HAE treatment plan during pregnancy.

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PERSPECTIVES ON THE EVOLUTION OF HAE TREATMENTS

Prior to 2008, limited HAE treatment options were available in the United States. Now, people with HAE can choose from eight FDA-approved medications (and several generic icatibants) for managing their condition.
The availability of multiple on-demand and prophylaxis treatment options that can be administered at home has significantly increased the HAEA community’s quality of life while decreasing emergency room visits and hospitalizations. We are fortunate that many manufacturers continue to pursue innovative approaches to improving treatment efficacy and ease of administration.
Prophylactic treatment is an option for people who wish to try to reduce the frequency and/or severity of HAE attacks by taking medication on a regular schedule. This can be done on a long-term or short-term basis. Long-term prophylaxis is typically given regularly for weeks, months, or years in an effort to minimize the overall attack burden. Short-term prophylaxis is typically given just prior to a surgical or dental procedure to prevent an HAE attack during or following the procedure. People with HAE receiving prophylactic treatment still need an effective on-demand treatment plan in the event the prophylactic treatment is not 100% effective.
Acute attacks of HAE are unpredictable, disabling and can be life-threatening if the airway is affected. Quickly and effectively treating these attacks prevents complications and minimizes interference with everyday activities. The US HAEA Medical Advisory Board recommends that all patients with HAE due to C1-Inhibitor deficiency should have access to at least two standard doses of a medication proven effective in treating HAE attacks.
Self and home administration can reduce the time to treatment and allows medication to be given earlier in an attack. Delays could allow an attack to become more severe and take longer to resolve once medication is finally received. Self and home treatment allows for greater independence and more reliable management of HAE symptoms.
Yes, many insurance companies are requiring information on the use and experience of HAE medications. The US HAEA Medical Advisory Board suggests that everyone with HAE keep a record of their prophylaxis and/or on-demand treatments. The documentation should include any attacks that may have occurred, the treatment response, and any possible medication side effects. The US HAEA offers a free app that makes it easy to record this type of information.


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