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If you live with hereditary angioedema (HAE), you know attacks don’t exactly stick to a schedule. Swelling can show up without warning, derail your day, and in some cases become life-threatening. That’s why health experts stress the importance of having an on-demand treatment plan.
On-demand medication helps stop HAE attacks that have already started. Even if you take medicine to prevent attacks, you may still have one, so it’s important to know when and how to use your on-demand treatment.
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On-demand treatment, sometimes called rescue treatment, refers to medications used to treat an HAE attack after symptoms begin. Unlike preventive medications, which are taken regularly to reduce the number of attacks, on-demand therapies are meant to stop an attack that’s already happening.
On-demand therapies are used for attacks involving:
According to international HAE guidelines and the US Hereditary Angioedema Association (HAEA), everyone with HAE should have access to effective on-demand treatment, even if they’re also taking preventive medication.
Health experts recommend treating attacks as early as possible. In general, the sooner treatment is given, the faster symptoms improve and the shorter the attack tends to be. Waiting to see whether symptoms worsen may delay relief and allow swelling to become more severe.
Not every attack requires a trip to the emergency room, but some symptoms should always be treated as medical emergencies.
Seek immediate medical attention and administer your rescue medication if you experience:
Even after using your medication, airway symptoms warrant emergency evaluation. Swelling in these areas can progress quickly and become life-threatening.
Severe abdominal attacks may require medical attention, especially if pain, vomiting, or dehydration don’t improve after treatment.
Several therapies approved by the U.S. Food and Drug Administration (FDA) are available to treat hereditary angioedema (HAE) attacks. These treatments are primarily used for type 1 and type 2 HAE, which involve problems with C1 inhibitor (C1INH).
Your allergist or HAE specialist can help determine which option best fits your needs, lifestyle, and medical history.
Berinert is a plasma-derived C1INH replacement treatment, also called a complement C1 esterase inhibitor. It’s given through an intravenous (IV) infusion. People with HAE and caregivers can be trained to administer Berinert at home.
Because it replaces C1INH, a protein that is missing or does not work properly in people with type 1 or type 2 HAE, Berinert can be used in children and adults. Plasma-derived C1INH also can be used during pregnancy and breastfeeding.
Many people notice symptoms beginning to improve within 30 minutes to a few hours after treatment.
Side effects are generally mild and may include headache, nausea, changes in taste, and infusion-site reactions.
Ruconest is another C1INH replacement treatment, but it’s made using recombinant technology rather than donated plasma. Like Berinert, it’s administered intravenously and is designed to restore working C1INH, which helps control the processes that cause swelling. Symptoms generally begin to improve within a few hours.
The most commonly reported side effects include headache, nausea, and diarrhea. Ruconest is produced using milk from genetically modified rabbits. People with a known or suspected rabbit allergy should not use it. This makes allergy history an important part of the discussion with your doctor.
Icatibant (Firazyr) works differently from C1 inhibitor replacement therapies. It It blocks the bradykinin B2 receptor. Bradykinin is a chemical that contributes to swelling during HAE attacks.
The medication is injected just beneath the skin, and adults can learn to administer it themselves after receiving training. This may make it easier to treat an attack at home or while traveling.
Symptoms often begin improving within a few hours, and the convenience of self-infection may make icatibant a practical option for some adults.
The most common side effect is temporary discoloration, swelling, or pain at the injection site. Fever, nausea, and dizziness can also occur.
Ecallantide (Kalbitor) also targets the pathway that leads to HAE swelling by blocking a protein called plasma kallikrein (an enzyme involved in producing bradykinin).
Unlike icatibant, however, it must be administered by a healthcare professional because of the small but serious risk of allergic reactions, including anaphylaxis (a severe, potentially life-threatening allergic reaction).
Most people begin experiencing symptom relief within several hours.
Common side effects include headache, nausea, fever, and injection-site reactions. Because treatment must be given by a qualified healthcare professional, ecallantide may not offer the same self-treatment flexibility as some other options.
Sebetralstat (Ekterly) is an oral on-demand treatment for HAE attacks. It also works by blocking plasma kallikrein. Because it’s taken by mouth, it may be easier for some people to use quickly compared with an injection or IV treatment.
Sebetralstat is approved for acute attacks in adults and children ages 12 and older.
The most commonly reported side effect in clinical trials was headache.
Your doctor can help you decide whether an oral option makes sense based on your age, symptoms, and other medications, as well as whether you can take the treatment soon after recognizing an attack.
No single treatment is right for everyone. Your doctor can help you weigh convenience, pregnancy considerations, and other health factors when choosing a therapy.
Before you head home, make sure you and your doctor have covered the details you’ll need when an attack happens.
Not every on-demand treatment is right for every person. Ask your doctor why a particular options fits your situation, especially if you:
Understanding the reasoning behind your treatment can make it easier to feel confident using it when symptoms appear.
Health experts recommend keeping enough on-demand treatment available for at least two attacks. Discuss:
Some people also keep medication at work, in a travel bag, or with a trusted family member to ensure access when they’re away from home.
Don’t assume you’ll figure it out during an emergency. Ask your healthcare team to provide:
Reviewing administration techniques periodically can help ensure you’re comfortable with the process, especially if attacks are infrequent.
Talk through what to do if your first dose doesn’t provide enough relief. A written action plan or instructions stored on your phone can be especially helpful during stressful situations. Carrying a medical ID card can also help in times where speaking may be difficult.
HAE medications are expensive and often require specialty pharmacies and prior authorization. Before leaving the office, make sure you understand:
Because approvals and shipping can take time, it’s best not to wait until your last dose to start the refill process.
HAE attacks are unpredictable, but your response doesn’t have to be. Having rescue medication is important, but understanding when to use it, knowing what symptoms require emergency care, and keeping enough doses readily available are just as essential.
By working with your doctor to create a clear action plan, you’ll be better prepared to respond quickly and confidently when an attack occurs.
On MyHAETeam, people share their experiences with hereditary angioedema, get advice, and find support from others who understand.
Do you know exactly what you’d do if an HAE attack started right now? Let others know in the comments below.
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