HAE Attack Management | Connect. Learn. Act.

Image of a hand of a person living with HAE holding a letter about their next HAE attack while playing tennis.

Hereditary Angioedema (HAE) is a rare condition characterised by unpredictable swelling¹

HAE is a chronic condition that is highly variable in symptom location, frequency, and severity. The hallmarks of all forms of HAE are recurrent cutaneous angioedema, abdominal symptoms from gastrointestinal angioedema, and airway symptoms due to laryngeal swelling. Symptom variability is observed between patients but also within the same individual over time.¹

Attacks may be triggered by physical trauma or emotional stress; however, swelling often occurs without a known trigger, which may lead to additional layers of anxiety for those living with HAE.^1,2

EVERY PATIENT WITH HAE, INCLUDING THOSE TAKING PROPHYLAXIS, SHOULD CARRY ON-DEMAND TREATMENT AND TREAT AT THE ONSET OF AN HAE ATTACK³

Although treatment advances such as prophylaxis can reduce frequency of attacks, studies show that patients continue to experience attacks in all anatomic locations, including potentially life-threatening laryngeal attacks. In fact, all patients with HAE are at risk for having an attack at any time, including those who take prophylaxis for attack prevention.^4,5 HAE Treatment Guidelines emphasize on-demand treatment as the essential component of HAE management for all patients. Further, guidelines reinforce that every attack should be considered for treatment as soon as possible once recognised.³

On-demand treatment is the essential component of HAE management

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1
Availability of on-demand treatmentEvery person living with HAE should consistently have access to sufficient on-demand treatment for at least 2 attacks and carry on-demand medication at all times, even if they're taking prophylaxis^2,3
2
Early treatment to prevent attack progressionOn-demand medication should be considered for every attack and taken at
attack onset³
3
Treatment regardless of location of swelling ALL HAE attacks are eligible for treatment, regardless of the location of swelling or severity of the attack³
4
Have a personalised action plan in place A treatment plan should be carefully developed through a shared decision-making process and based on the individual needs of a patient²

Infographics about 90 percent of patients experienced attacks despite prophylaxis

Infographic about people living with HAE, highlighting that 47 percent of people taking prophylaxis may not be filling on-demand prescriptions.

Patients waited 3.8 hours to treat on average after onset of an attack

*Data were derived from a US-based retrospective, real-world survey of 144 patients using the Adelphi HAE Disease Specific Program database (July-November 2021).⁶
^†According to a 2023 US HAE Association survey of 94 patients taking either on-demand treatment or prophylaxis + on-demand treatment.⁸

Prompt use of on-demand treatment can improve attack outcomes⁹

Infographic of a 10-year outcome survey highlighting how treating at the onset of an attack can prevent attacks from worsening and shorten their duration.

Despite recognising the onset of an attack, patients go through a complex decision-making process and rationalise delaying treatment¹⁰

While attacks vary and are highly individualised, the majority of patients recognise and can describe what they feel during the onset of an attack

Infographic of the reasons people living with HAE delay using on-demand treatment.

*According to a 2022 US HAE Association survey of 107 patients taking either on-demand treatment or prophylaxis + on-demand treatment.¹⁰

Delayed treatment may increase attack duration and recovery time^9,10

Infographic of recovery after delaying treatment highlighting 2 days to full recovery.

Infographic showing that attacks return for people living with HAE who do not treat at attack onset, highlighting 38%

Infographic highlighting 2 out of 3 people living with HAE who experience attack recurrance take a second dose of on-demand treatment.

People with HAE say they try to conserve treatment for their "worst" attacks.¹²

Anxiety remains a hallmark of HAE and is often the cause of on-demand treatment delay^12,13

Infographic showing that people living with HAE reported moderate to severe anxiety and depression.

Infographic of 1 in 4 people living with HAE having high anxiety with the use of on-demand treatment.

Infographic of the socioeconomic cost associated with HAE attacks.

How are your patients preparing for and deciding to treat HAE attacks?
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References: 1. US Hereditary Angioedema Association. What is Hereditary Angioedema (HAE)? Accessed July 25, 2023. https://www.haea.org/pages/p/what_is_hae 2. Maurer M, Magerl M, Betschel S, et al. The international WAO/EAACI guideline for the management of hereditary angioedema—the 2021 revision and update. Allergy. 2022;77:1961-1990. doi:10.1111/all.15214 3. Busse P, Christiansen SC, Riedl MA, et al. US HAEA Medical Advisory Board 2020 guidelines for the management of hereditary angioedema. J Allergy Clin Immunol Pract. 2021;1:132-150.e3. doi:10.1016/j.jaip.2020.08.046 4. Betschel S, van Kooten S, Heckmann M, Danese S, Goga L, Guilarte M. HAE patients decision to carry on-demand treatment when away from home. Abstract presented at: 13th C1-inhibitor Deficiency & Angioedema Workshop; May 4-7, 2023; Budapest, Hungary. Allergy Asthma Clin Immunol. In Press. 5. Longhurst H, Bouillet L, Cancian M, et al. Hereditary angioedema attacks in patients receiving long-term prophylaxis: a systemic review. Presented at: APAAACI; October 23-26, 2023; Singapore. 6. Anderson J, Soteres D, Tachdjian R, et al. Real-world outcomes in patients with hereditary angioedema prescribed lanadelumab versus other prophylaxis. Allergy Asthma Proc. Published online September 18, 2024. doi:10.2500/aap.2024.45.240046 7. Riedl M, Banerji A, Manning M, et al. Treatment patterns and healthcare resource utilization among patients with hereditary angioedema in the United States. Orphanet J Rare Dis. 2018;13(1):180. doi:10.1186/s13023-018 8. Christiansen S, O’Connor M, Ulloa J, et al. Delayed on-demand treatment of hereditary angioedema attacks: patient perceptions and associated barriers. J Allergy Clin Immunol. 2024;153(2)Supplement:AB89. doi:10.1016/j.jaci.2023.11.297 9. Maurer M, Aberer W, Caballero T, et al. The Icatibant Outcome Survey: 10 years of experience with icatibant for patients with hereditary angioedema. Clin Exp Allergy. 2022;52:1048-1058. doi:10.1111/cea.14206 10. Betschel SD, Caballero T, Jones DH, et al. The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks. Allergy Asthma Clin Immunol. 2024;20(1):43. doi:10.1186/s13223-024-00903-w 11. Data on File. KalVista Pharmaceuticals, Inc. 2023. 12. Mendivil J, Murphy R, de la Cruz M, et al. Clinical characteristics and burden of illness in patients with hereditary angioedema: findings from a multinational patient survey. Orphanet J Rare Dis. 2021;16:94. 13. Valerieva A, Van Kooten S, Heckmann M, et al. Patients delay treating hereditary angioedema (HAE) attacks with currently available, injectable, on-demand therapies. Abstract presented at: EAACI 2023 Hybrid Congress; June 9-11, 2023; Hamburg, Germany. Allergy. In Press. 14. Maurer M, Aberer W, Bouillet L, et al. Hereditary angioedema attacks resolve faster and are shorter after early icatibant treatment. PLoS ONE. 2013;8(2):e53773. doi:10.1371/journal.pone.0053773 15. Longhurst HJ, Dempster J, Lorenzo L, et al. Real-world outcomes in hereditary angioedema: first experience from the Icatibant Outcome Survey in the United Kingdom. Allergy Asthma Clin Immunol. 2018;14:28. doi:10.1186/s13223-018-0253-x

Hereditary Angioedema (HAE) is a rare condition characterised by unpredictable swelling1

EVERY PATIENT WITH HAE, INCLUDING THOSE TAKING PROPHYLAXIS, SHOULD CARRY ON-DEMAND TREATMENT AND TREAT AT THE ONSET OF AN HAE ATTACK3

On-demand treatment is the essential component of HAE management

Prompt use of on-demand treatment can improve attack outcomes9

Despite recognising the onset of an attack, patients go through a complex decision-making process and rationalise delaying treatment10