How to Manage Flare-ups
We've put together a patient-friendly review of treatment options for hereditary angioedema (HAE) so you can understand how they work, how to incorporate lifestyle and natural supports and understand warning signs. Always work closely with your immunologist or allergist before making changes to your treatment plan.
1. Haegarda (C1-Esterase Inhibitor, Subcutaneous)
This is often one of the first choices for long-term prevention. Haegarda is a form of C1-esterase inhibitor (C1-INH) delivered by subcutaneous injection (under the skin) twice weekly (every 3–4 days) in most patients.
In HAE (types I or II), the body either doesn’t make enough C1-INH or makes a dysfunctional version. That deficiency allows the cascade of proteins (involving kallikrein, factor XII, bradykinin) to run unchecked, leading to high levels of bradykinin, which causes blood vessels to leak, leading to swelling. HAEGARDA provides an external supply of C1-INH. That helps regulate the cascade, slowing or preventing the overproduction of bradykinin, thereby lowering the chance of swelling attacks.
Because it is for prophylaxis (prevention), it is not recommended for treating an active swelling attack once it starts.
Limitations and side-effects may include:
- Injection site reactions (pain, redness) are common.
- Dizziness, nasal stuffiness or other mild symptoms may occur.
- Rare hypersensitivity reactions.
- It requires regular injections and training in self-administration.
2. Preventive Therapies
If Haegarda is not sufficient, not available or not tolerated, several other preventative options exist:
- Lanadelumab (Takhzyro). A monoclonal antibody that inhibits plasma kallikrein, blocking part of the cascade that leads to bradykinin production. It’s given by subcutaneous injection, typically every two weeks.
- Garadacimab (Andembry). A monoclonal antibody targeting activated factor XIIa, it reduces activation of kallikrein. Approved more recently in some jurisdictions.
- Donidalorsen (Dawnzera). An antisense oligonucleotide (a newer “gene-targeted” drug) that reduces the amount of prekallikrein made, lowering the cascade’s ability to generate bradykinin.
- Attenuated androgens (e.g. low-dose danazol or stanozolol). An older approach, effective in many but with more side effects; used when newer therapies aren’t available or as adjuncts.
- Plasma-derived C1-INH concentrates (intravenous). Used prophylactically in some settings (e.g. Cinryze). These are infusions rather than injections.
The choice among these depends on severity, frequency of attacks, side-effect profiles, cost/insurance coverage and patient preference. The global guidelines emphasize combining prophylaxis with on-demand (acute) therapy.
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3. On-demand and Acute Attack Therapies
Even with preventive therapy, breakthrough swelling attacks can happen. The goal is to treat as early as possible.
The main on-demand options include:
- C1-INH concentrates (plasma-derived or recombinant). Infusions that “replace” the missing inhibitor temporarily, quickly helping to halt swelling.
- Icatibant (Firazyr). A bradykinin receptor antagonist (blocks the receptor that bradykinin acts on). Given by subcutaneous injection.
- Ecallantide (Kalbitor). An inhibitor of plasma kallikrein (the enzyme that helps generate bradykinin). It’s given by subcutaneous injection (three injections) during an acute attack.
- Fresh frozen plasma (FFP). Sometimes used if specific therapies aren’t available; it contains natural C1-INH and can help stop attacks in emergencies.
- Newer oral options. For example, sebetralstat (Ekterly) was approved in 2025 as an oral on-demand therapy. It inhibits plasma kallikrein, reducing production of bradykinin when an attack begins.
Again, prompt administration is key — delays allow the swelling to worsen and become harder to reverse.
Natural, Supportive and Lifestyle Measures
Though medications are the backbone of HAE care, several supportive strategies can help reduce attack frequency or severity, and improve overall health. These are not substitutes for medical therapy but are complementary.
Trigger Avoidance
- Try to identify and avoid known personal triggers: minor trauma, dental or surgical procedures, emotional stress, infections, hormonal changes, certain medications (like ACE inhibitors, estrogens) and even high-intensity exertion.
- Avoid drugs known to worsen angioedema (ACE inhibitors are a common culprit).
Infection and Inflammation Control
- Promptly treat infections (viral, dental, etc.) because they often precipitate attacks.
- Maintain good dental hygiene, as dental work or infections can trigger or worsen swelling in the face or airway.
Stress Management
- Techniques like relaxation, mindfulness, biofeedback, meditation or gentle yoga may reduce the frequency or intensity of stress-triggered attacks.
- Adequate sleep is also helpful.
Healthy Lifestyle
- A nutrient-dense diet, maintaining normal weight, avoiding excessive alcohol and smoking can support vascular and immune health.
- Stay well hydrated; dehydration can stress circulation.
Monitoring and Planning
- Keep a symptom diary (dates, severity, potential triggers, treatment response).
- Have a “go bag” with your emergency on-demand medication, medical alert card and contact info for your HAE specialist.
- Wear medical alert jewelry indicating you have HAE and carry instructions for acute care (e.g. “this is bradykinin-mediated swelling, do not treat as allergic angioedema”).
Physical Protection
- Be gentle with invasive procedures (dental, surgery) and pre-treat if recommended by your physician.
- In certain cases, short-term prophylaxis may be used before procedures to reduce risk.
When to See a Doctor
Common Signs and Symptoms
- Sudden swelling (non-itchy) of face, lips, tongue, throat, hands, feet and/or genitals.
- Abdominal pain, cramping, nausea, vomiting, diarrhea and/or bloating.
- Difficulty swallowing or breathing (throat or airway swelling).
- Voice changes, hoarseness, tightness in throat.
- Fatigue or weakness during or following attack.
When to Seek Urgent Medical Care
- Any signs of throat or laryngeal swelling, breathing difficulty, hoarseness and/or a feeling of tightness in your throat.
- Rapid progression of swelling.
- Severe abdominal pain you cannot relieve.
- Persistent swelling that doesn’t respond to your on-demand treatment.
- New or unfamiliar swelling pattern.
- Lightheadedness, low blood pressure or signs of severe systemic involvement.
- If you suspect airway involvement, call emergency services immediately.
HAE treatment involves preventive therapy to reduce the frequency of attacks, plus on-demand treatments to abort swelling once it starts. Complementary lifestyle measures (trigger avoidance, stress control, prompt infection treatment) can support medical care. Always carry emergency medication, monitor symptoms and seek prompt help for throat swelling or breathing trouble.