In 2013, the US HAE partnered with its Medical Advisory Board (MAB) to write and publish a first of its kind set of guidelines for the treatment of HAE. Understanding these recommendations can be useful when speaking with your physician. This Q&A, provided by Dr. Marc Riedl, Professor of Medicine and Clinical Director of the US HAEA Angioedema Center at UC San Diego, and HAEA Medical Advisory Board member, offers a concise, patient-friendly overview of these guidelines and other recent study findings.
How has the treatment of HAE changed during the last few years?
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.
How have these changes affected the lives of patients?
A recent physician survey presented at the American Academy of Allergy, Asthma and Immunology Annual Meeting showed several encouraging signs of progress. Over the past few 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.
Recent guidelines from the US HAE Medical Advisory Board call for an HAE Management Plan for every HAE patient. What are the most important elements of this plan?
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 her/his 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 his or her 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 back-up 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.
Why is acute treatment 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-INH deficiency should have access to at least two (2) standard doses of a medication proven effective in treating HAE attacks.
Current HAE management guidelines recommend treating at the first sign of an attack. Why is this important for patients and physicians?
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.
Why is it important for a patient to have medication readily available for self and home administration?
Self and home administration can reduce the time to 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.
What are the benefits of prophylactic treatment?
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.
Why is ongoing monitoring of attack frequency and severity necessary?
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 its essential for the HAE physician to regularly monitor your condition. In addition, its 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.
What resources are available to better understand HAE and how to manage it??
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 an Angioedema Center at the University of California San Diego (UCSD) to provide expert patient care for all types of angioedema. Travel assistance is available based on need – contact your HAEA Patient Advocate to learn more about scheduling an appointment with one of the Center physician experts.