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PubMed This is a summary of 23 peer-reviewed journal articles Updated
Immunology

Taking Control: Modern Strategies for Managing HAE Attacks

At a Glance

Modern Hereditary Angioedema (HAE) treatment uses a Treat-to-Target approach aiming for zero attacks. Management involves on-demand medications to stop acute attacks, short-term prophylaxis for high-risk events, and long-term preventative therapies like daily pills or monthly injections.

Treatment for Hereditary Angioedema (HAE) has evolved rapidly. Today, the medical community follows a “Treat-to-Target” (T2T) approach, where the “target” is complete disease control—meaning zero attacks and a life unburdened by the fear of swelling [1][2].

Management is divided into three distinct phases: stopping an attack that has already started (Acute), preventing an attack during a high-risk event (Short-Term Prophylaxis), and preventing attacks from happening at all (Long-Term Prophylaxis) [3][4].

Navigating Treatment Access

Modern HAE therapies are life-changing, but accessing them involves navigating specialty pharmacies, prior authorizations, and insurance hurdles. Do not be discouraged; pharmaceutical companies offer robust co-pay assistance programs, and HAE patient advocacy organizations can provide case managers to help you secure the medications you need [5].

1. Acute (On-Demand) Treatment

The goal of acute treatment is to stop an attack as soon as it begins. Guidelines recommend that every patient have access to enough rescue medication to treat at least two attacks at all times [6].

  • Subcutaneous Injection: Icatibant is a common on-demand therapy injected under the skin that blocks bradykinin from binding to its receptors [7].
  • Intravenous (IV) C1-inhibitors: Products like Berinert replace the missing protein [4]. A special note on Ruconest: Ruconest is a recombinant C1-inhibitor produced using transgenic rabbits. It carries an absolute contraindication for patients with known rabbit allergies and must not be used by those individuals, as it can trigger severe anaphylaxis [8].
  • New Oral Option: Approved in late 2025/early 2026, sebetralstat is an oral on-demand pill that inhibits plasma kallikrein and provides rapid symptom relief without needles [9][10].

2. Short-Term Prophylaxis (STP)

Certain events, especially dental work or surgeries involving the airway, can trigger an attack [11]. STP involves taking medication shortly before a procedure to provide a temporary “shield” [4]. The standard of care is usually a dose of IV C1-inhibitor concentrate administered shortly before the procedure [12]. Even with this protection, always have acute rescue medication ready [6].

3. Long-Term Prophylaxis (LTP)

LTP is used to prevent attacks from ever occurring [13][14].

  • Monthly Injections (Monoclonal Antibodies):
    • Lanadelumab: A subcutaneous injection usually given every 2 to 4 weeks that blocks kallikrein [13][15].
    • Garadacimab: A recently approved monthly subcutaneous injection that targets Factor XIIa, stopping the cascade earlier [16][17].
    • Donidalorsen: An emerging therapy using RNA technology to reduce prekallikrein production [18][19].
  • Daily Oral Pill:
    • Berotralstat: A once-daily capsule inhibiting plasma kallikrein, popular for avoiding injections [20][21].
  • Older Therapies: Historically, attenuated androgens (like Danazol) were used for prophylaxis. While some patients globally still rely on them, they carry risks of severe side effects, including liver toxicity (hepatotoxicity), and have largely been replaced by modern targeted biologics [22][23].

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Common questions in this guide

What is the Treat-to-Target approach for HAE?
The Treat-to-Target approach in HAE management means aiming for complete disease control. The ultimate goal is for patients to experience zero attacks and live without the constant fear of sudden swelling.
Is there an oral pill to treat an acute HAE attack?
Yes, sebetralstat is a newly approved oral on-demand pill designed to stop an acute HAE attack. It works by inhibiting plasma kallikrein to provide rapid symptom relief without the need for an injection.
How can I prevent an HAE attack before dental work?
Short-term prophylaxis can provide a temporary shield of protection before high-risk events like dental surgery. This is typically done by administering an IV C1-inhibitor concentrate shortly before your procedure, though you should always have rescue medication on hand just in case.
What is the difference between short-term and long-term prophylaxis for HAE?
Short-term prophylaxis is used temporarily before specific triggers like surgeries to prevent an immediate attack. Long-term prophylaxis involves taking regular medication, such as daily pills or monthly injections, to constantly prevent attacks from happening in your day-to-day life.
Are there risks with using older HAE treatments like Danazol?
Older therapies like Danazol carry the risk of severe side effects, including liver toxicity. Because of these safety concerns, they have largely been replaced by modern, targeted biologic therapies that offer better protection with fewer risks.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Given my current attack frequency, am I a candidate for a 'Treat-to-Target' approach aiming for zero attacks?
  2. 2.Is the newly approved oral on-demand medication (sebetralstat) appropriate for my specific type of HAE?
  3. 3.If I choose long-term prophylaxis, should I consider a daily oral pill (berotralstat) or a monthly injection (garadacimab/lanadelumab)?
  4. 4.What is the specific protocol for short-term prophylaxis if I need an emergency dental procedure?
  5. 5.Can you provide resources or connect me with a social worker to help navigate specialty pharmacy prior authorizations?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (23)
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    A Treat-to-Target approach in hereditary angioedema: expert consensus from a European committee.

    Cancian M, Caballero T, Boccon-Gibod I, et al.

    Frontiers in immunology 2026; (17()):1773279 doi:10.3389/fimmu.2026.1773279.

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    Patient Preferences for Long-Term Prophylactic Treatment in Hereditary Angioedema: A Discrete-Choice Experiment.

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    Treatment of Hereditary Angioedema With Plasma-Derived C1 Inhibitor: A Review.

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    Breakthrough attacks in patients with hereditary angioedema receiving long-term prophylaxis are responsive to icatibant: findings from the Icatibant Outcome Survey.

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This page provides educational information about modern treatment strategies for Hereditary Angioedema (HAE). Always consult your immunologist or healthcare provider to determine the safest and most effective treatment plan for your specific condition.

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