HAE treatment classes: Understanding the differences
Fact-checked by Getting up to speed after being diagnosed with a rare condition like hereditary angioedema (HAE) can feel like a full-time job, with a whole new vocabulary to learn and important treatment decisions to make, often at the same time.
Your doctor may have already told you that there isn’t just one standard therapy for HAE. There are two different HAE treatment classes, each serving a unique purpose. Learning the basics can help you feel informed during treatment conversations with your healthcare team.
Types of HAE treatment classes
Today’s HAE treatments fall into two main groups. The first includes medications taken during an episode. The other includes prophylactic treatments, which help prevent HAE attacks. You may be prescribed medications from one group or both. Together, they form the foundation of HAE management.
On-demand medications
During an HAE attack, fluid leaks from blood vessels, causing sudden swelling. These HAE symptoms can affect the hands, feet, face, stomach, throat, and other parts of the body.
On-demand medications are to be taken as soon as symptoms appear. Their goal is to stop the swelling as quickly as possible. On-demand HAE medications include:
- bradykinin B2 receptor antagonists: These block the receptors for bradykinin, a natural molecule that triggers swelling in HAE.
- kallikrein inhibitors: These inhibit kallikrein, an enzyme involved in the production of bradykinin.
- C1-inhibitor replacement therapies: These replace the C1-inhibitor (C1-INH) protein, which is missing or dysfunctional in some types of HAE, and normally regulates bradykinin production.
Most on-demand medications take effect within an hour, so it’s important to administer them as soon as symptoms first appear.
Prophylactic HAE treatments
Some people have frequent or disruptive HAE attacks that interrupt work, school, sleep, or daily functioning. In these situations, doctors may recommend medications that are taken regularly to help prevent swelling before it begins.
Preventive therapy’s main goal is to reduce how often attacks happen and make them less severe if they do occur.
Your doctor will consider your quality of life, attack pattern, and personal needs when deciding whether to prescribe a preventive HAE treatment. Even if you’re on one of these medications, you’ll still likely need to have an on-demand medication available as well.
Prophylactic HAE treatments include:
- kallikrein inhibitors: These are taken regularly to prevent bradykinin levels from rising too high and triggering swelling attacks.
- C1-inhibitor replacement therapies: These are given on a routine schedule to help maintain C1-INH levels high enough to prevent bradykinin levels from rising too high.
In some cases, short-term prevention strategies may be used. This means taking a prophylactic medication for a brief period before known triggers, such as dental procedures, surgery, or other physical stressors.
Having the right prevention plan in place can also lower anxiety about when the next attack might happen.
How your HAE type can affect treatment choice
The three different types of HAEÂ include:
- HAE type 1: The body doesn’t make enough C1-INH due to mutations in a gene called SERPING1.
- HAE type 2: C1-INH levels are normal, but the protein doesn’t work properly due to SERPING1 mutations.
- HAE type 3, or HAE with normal C1-INH: C1-INH levels and activity are normal, but genetic mutations in genes other than SERPING1 can still trigger swelling episodes.
Before choosing a treatment, your provider will first determine which type of HAE you have. In type 1 and type 2, swelling is linked to dysregulation of bradykinin production driven by the lack or malfunction of C1-INH.
Because of this, treatments that replace the missing or dysfunctional C1-INH protein or block the bradykinin pathway are commonly used. These may include C1-inhibitor replacement therapies and medicines that block kallikrein or bradykinin receptors.
Treating HAE with normal C1 inhibitor can be different. Some people may still benefit from the same swelling-blocking medicines. Other prevention methods may include hormonal therapy or medications that prevent blood clots from breaking down too quickly.
Your doctor will also consider your symptoms, triggers, and daily routine when choosing a treatment plan.
Choosing the right treatment for you
Finding the right treatment plan can take time after an HAE diagnosis. Talk openly with your healthcare team about your symptoms, frequency of attacks, and biggest challenges related to living with HAE.
Your treatment plan may change over time, too. That’s normal. The goal is better control, fewer surprises, and more confidence in daily life. With regular follow-up and clear communication, you can build a routine that prioritizes safety and the activities that matter to you.
Angioedema News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.
