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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 people with HAE 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 HAE Advocate who can help you get in touch with an HAE expert physician in your area.


The guidelines provide the medical community with a comprehensive scientific overview of HAE in all its forms (Type I, Type II, and HAE with Normal C1-Inhibitor) and offer best practices that emphasize the importance of the patient voice in determining an optimal treatment approach.

HAE Primer

The US HAEA joined forces with the prestigious medical journal, Allergy and Asthma Proceedings, and HAE physician-experts to develop a special supplementary Primer on HAE diagnosis and management. The Primer contains 14 peer-reviewed articles on a range of vitally important HAE topics.
To view the Primer, please click here.

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. Anabolic steroids, also known as androgens (such as danazol, oxandrolone, stanozolol, etc.), should be avoided during pregnancy. There are studies published on FDA-approved HAE therapies and pregnancy that should be read and considered when developing an HAE treatment plan during pregnancy. Importantly, people with HAE and their physicians should plan to have an acute treatment available upon delivery.


US HAEA Health Economics Study Demonstrates the Value of New HAE Preventitive Medicines

Published in a peer-reviewed medical journal, original HAEA research — based on 800 people with HAE — reveals that the new subcutaneous preventive therapies yield (1) substantial economic value in light of remarkable decreases in attack frequency and (2) statistically significant improvements in patient quality of life. To view the study, please click here.

Frequently Asked Questions

This Q&A, provided by Dr. Marc Riedl, Professor of Medicine and Clinical Director of the US HAEA Angioedema Center at UCSD and US HAEA Medical Advisory Board Member, offers a concise, patient-friendly overview of HAE treatment guidelines.

Prior to 2008, limited treatment options were available to HAE patients in the United States. Now, HAE patients can choose from several FDA-approved medications for managing their condition.
A recent physician survey presented at the American Academy of Allergy, Asthma, and Immunology Annual Meeting showed encouraging signs of progress. Over the past several years patient satisfaction with HAE treatment has increased (as reported by their physicians). In addition, more patients are receiving their HAE treatment at home and the number of hospitalizations and visits to the emergency room for HAE has decreased. It's believed the availability of more effective HAE medications is a major reason for these changes.
The first step is for individuals with HAE to consult with a physician experienced in treating HAE whenever possible. This expertise may allow for more comprehensive HAE care and maximal options in managing this complex and rare medical condition. Because HAE is so variable in how it affects people, each patient needs a treatment plan tailored to their symptoms and other personal factors. Secondly, all patients should have a clear plan for treating HAE attacks with an effective on-demand medication. The patient should know exactly where their medication is kept, how to use it, and who to contact if assistance is required for treatment. Acute treatment medications should be reliably and efficiently accessible to avoid treatment delays. Patients should also have a backup plan and be prepared to navigate the hospital or health care system if they need treatment for airway involvement or other severe angioedema symptoms. This may involve discussing their HAE condition with a local hospital, carrying a letter or electronic file summarizing HAE and the treatment, or flagging the medical record to expedite appropriate treatment during an HAE attack. For some HAE patients, preventative medication regimens are another important part of the management plan. All treatment plans should include some method of regular follow-up with the HAE specialist to track the efficacy and safety of the treatment as well as adjust the plan over time. HAE symptoms often change over time, so the treatment plan must sometimes change along with this. Communication between the patient, the HAE specialist, and other involved health care providers is extremely important.
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. This is the reason that an on-demand, acute treatment plan is essential for every patient. Based on the US HAEA Medical Advisory Board recommendations, all patients with HAE due to C1-Inhibitor deficiency should have access to at least two (2) standard doses of a medication proven effective in treating HAE attacks.
Studies have shown that treating HAE attacks earlier reduces the total time with swelling symptoms and improves quality of life. People are able to return to their normal activities more rapidly. Regardless of the location of the attack, it is important to treat as soon as the attack is recognized.
Self and home administration can reduce the time of treatment and allows medication to be given earlier in an attack. While some patients may prefer to go to the hospital or doctor's office to be treated for an HAE attack, this plan can be associated with delays (travel time, wait times at the emergency department or clinic). During these delays, the attack may progress to more severe symptoms, which take longer to resolve once medication is finally received. Reduction in the time-to-treatment can make a big difference in rapidly stopping an attack early in the course. Self and home treatment may also give the patient more independence to reliably manage their condition.
Prophylactic treatment is an option for patients 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. Patients receiving prophylactic treatment still need an effective on-demand treatment plan in the event the prophylactic treatment is not 100% effective.
HAE is a highly variable condition, which means that attack severity, frequency, and affected body regions can change over time. It may be necessary to adjust the HAE treatment plan along with these changes, so it's essential for the HAE physician to regularly monitor the patient's condition. In addition, it’s important to ensure the prescribed HAE medications remain safe and effective for patients over time. The US HAEA Medical Advisory Board suggests all patients keep a record of their attacks, noting which medications were used, their response to treatment, and any possible medication side effects.
One of the most important resources for any HAE patient is your expert physician. Given the highly variable nature of the condition and the complexity of managing HAE, your HAE expert physician can provide individualized information specific to you. The US HAEA has established the US HAEA Angioedema Center at the UCSD to provide expert patient care for all types of angioedema. Travel assistance is available based on need – contact an HAE Advocate at the US HAEA to learn more about scheduling an appointment with one of the Center physician experts.


There Are New Treatments On The Horizon

Wondering if you or someone you love might be living with undiagnosed HAE? Contact us to find a physician in your area with HAE experience who can make a diagnosis.