Treatment for Hereditary Angioedema

Living with a rare condition can feel overwhelming for you and your family. Hereditary angioedema (HAE) is a genetic condition that causes unexpected swelling in the body. This swelling can cause pain and disrupt your daily activities. Fortunately, you can learn to manage your symptoms with a proper treatment plan.¹

Since HAE symptoms typically start to appear before age 13, parents and caregivers play an important role in helping to manage this condition. Learning about available treatment options will help your entire family feel prepared.¹

What is hereditary angioedema?

Hereditary angioedema is a rare genetic disorder that affects about 1 in 50,000 people. HAE causes severe swelling in the face, hands, feet, stomach, and throat. Swelling in the throat is dangerous and can block your breathing.¹

These swelling attacks are unpredictable and can happen at any time. HAE attacks are not allergic reactions. Standard allergy medicines like antihistamines will not work for HAE swelling. This unique condition requires specialized medical care and specific treatments.¹

Who treats hereditary angioedema?

You should work with a doctor who has experience treating HAE. These doctors are usually specialists in allergy and immunology. An expert doctor will help you build a personal management plan. They will help you find the right healthcare treatments for your needs.^2,3

Your specialist teaches you and your caregivers how to handle attacks. Caregivers can learn to give treatments at home or on the go. Regular follow-up visits with your doctor ensure your plan stays effective. HAE symptoms can change, so your treatment plan may need updates.

Types of medical treatment

Medical treatments for HAE fall into 2 main categories:^1,2

• On-demand (acute) treatment
• Prophylaxis (preventive) treatment

On-demand (acute) treatment

The first type of treatment is on-demand, or acute, therapy. You take on-demand medicines at the first sign of an attack. These medicines stop the swelling from getting worse.^2,3

On-demand medicines include:^2,3

• Berinert® (C1-esterase inhibitor - human) – intravenous injection
• Ekterly® (sebetralstat) – oral tablet
• Firazyr® (icatibant) – subcutaneous injection
• Icatibant (generic) – subcutaneous injection
• Kalbitor® (ecallantide) – subcutaneous injection
• Ruconest® (C1-esterase inhibitor - recombinant) – intravenous injection

You can inject these medicines (either intravenously or under the skin) or take them by mouth. Early treatment helps your symptoms resolve as quickly as possible. Experts advise people with HAE to have on-demand medicine with them at all times.^2,3

Prophylaxis (preventive) treatment

The second type of treatment is long-term prophylaxis. This means taking medicine regularly to prevent attacks before they start. Preventing attacks helps improve overall quality of life for those with HAE. Regular preventive care can greatly reduce how often swelling occurs.^2,3

Preventive medicines include:^2,3

• Andembry® (garadacimab-gxii) – subcutaneous injection
• Cinryze® (C1-esterase inhibitor - human) – intravenous injection
• Dawnzera™ (donidalorsen) – subcutaneous injection
• Haegarda® (C1-esterase inhibitor - human) – subcutaneous injection
• Orladeyo® (berotralstat) – oral tablet or sprinkles
• Takhzyro® (lanadelumab) – subcutaneous injection

These options include daily oral capsules or regular injections (intravenous or subcutaneous, under the skin). Your doctor can help you choose the best treatment option for you. Your doctor will recommend using short-term prophylaxis before certain surgical or dental procedures.^2,3

The history of anabolic steroids

In the past, doctors used anabolic steroids like danazol to prevent HAE attacks. However, these older drugs can cause liver damage, high cholesterol, and allow for dangerous breakthrough attacks. Modern treatment guidelines now prefer safer and more effective choices.³

The future of gene editing

The future of HAE treatment may include gene editing. A new study showed positive phase 3 clinical trial results for a drug called lonvo-z. This one-time treatment uses CRISPR technology to change a specific gene. According to the study, a single dose reduced swelling attacks by 87 percent compared to a placebo. Additionally, 62 percent of people with HAE became completely attack-free.⁵

This is not an FDA-approved treatment yet, and more research is needed to understand its safety and efficacy. But it provides hope for future management of the condition.⁵

Lifestyle changes and avoiding triggers

Medicines are vital, but lifestyle choices can also help you manage HAE. Many attacks are caused by specific everyday triggers. Knowing what triggers your swelling can help you prevent future flare-ups.¹

Common triggers of an HAE attack can include:^1,4

• Emotional stress and anxiety
• Physical injury or bodily trauma
• Common illnesses like the flu or a virus
• Surgical or dental procedures
• Activities like typing, hammering, or shoveling
• Hormonal changes like menstruation and pregnancy

While you cannot avoid all of these possible triggers, there are some things you can do to reduce the risk of an attack:⁴

• Reduce stress by practicing deep breathing, walking, or journaling.
• Choose gentle exercises like swimming or walking instead of contact sports.
• Wash your hands, eat a balanced diet, and get plenty of sleep to support a healthy immune system and reduce the risk of getting sick.

It can be helpful to track your personal triggers. Tracking symptoms helps you notice early signs of an attack. These early signs are called prodromal symptoms and can include fatigue or tingling.

Complementary and alternative therapies

Some people look for alternative options to support their health. Complementary therapies should never replace your prescribed HAE medicines. Relaxation activities like yoga and meditation can help manage stress. Lowering your stress levels may reduce the frequency of attacks.

Certain nutrients like vitamin D and omega-3 fatty acids may lower inflammation. You must always talk to your doctor before starting any new supplement. Supplements are not regulated the same way as prescription medicines. Your specialist can check your blood levels for any nutrient deficiencies.

Managing your health

Managing HAE requires a strong partnership with your healthcare specialist. By using on-demand and preventive medicines, you can control swelling. Avoiding personal triggers like stress and injury adds extra protection. A complete plan allows you and your loved ones to live with confidence.^1,3,4