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. 2024 Jul 25;20(1):43.
doi: 10.1186/s13223-024-00903-w.

The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks

Stephen D Betschel  1 , Teresa Caballero  2 , Douglas H Jones  3 , Hilary J Longhurst  4 , Michael Manning  5 , Sally van Kooten  6 , Markus Heckmann  6 , Sherry Danese  7 , Ledia Goga  8 , Autumn Ford Burnette  9
Affiliations

The complexities of decision-making associated with on-demand treatment of hereditary angioedema (HAE) attacks

Stephen D Betschel et al. Allergy Asthma Clin Immunol. .

Erratum in

Abstract

Background: Hereditary angioedema (HAE) is characterized by debilitating attacks of tissue swelling in various locations. While guidelines recommend the importance of early on-demand treatment, recent data indicate that many patients delay or do not treat their attacks.

Objective: This survey aimed to investigate patient behavior and evaluate the key factors that drive on-demand treatment decision-making, as reported by those living with HAE.

Methods: People living with HAE were recruited by the US Hereditary Angioedema Association (HAEA) to complete a 20-minute online survey between September 6, and October 19, 2022.

Results: Respondents included 107 people with HAE, 80% female, 98% adults (≥ 18 years). Attack management included on-demand therapy only (50%, n = 53) or prophylaxis with on-demand therapy (50%, n = 54). Most patients (63.6%) reported that they did not carry on-demand treatment at all times when away from home. The most common reason for not carrying on-demand treatment when away from home was 'prefer to treat at home' (72.1%). Overall, 86% of respondents reported delaying on-demand treatment, despite recognizing the initial onset of an HAE attack and despite 97% of patients agreeing that it is important to recover quickly from an HAE attack. Reasons for non-treatment or treatment delay included 'the attack is not severe enough to treat' (91.9% and 88.0%, respectively), 'cost of treatment' (31.1% and 40.2%, respectively), anxiety about refilling the prescription for on-demand treatment quickly (31.1% and 37.0%, respectively), the pain (injection or burning) associated with their on-demand treatment (18.9% and 28.3%, respectively), the lack of a suitable/private area to administer on-demand treatment (17.6% and 27.2%, respectively), lack of time to prepare on-demand treatment (16.2% and 16.3%, respectively), and a 'fear of needles' (13% and 12.2%, respectively). Survey findings from the patient perspective revealed that when on-demand treatment was delayed, 75% experienced HAE attacks that progressed in severity, and 80% reported longer attack recovery.

Conclusions: Survey results highlight that decision-making regarding on-demand treatment in HAE is more complicated than expected. The burden associated with current parenteral on-demand therapies is often the cause of treatment delay, despite acknowledgment that delays may result in progression of HAE attacks and longer time to recovery.

Keywords: Attacks; Decision-making, Burden of treatment, Survey, HAE attack journey; Hereditary angioedema (HAE); On-demand treatment.

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Conflict of interest statement

The authors declare no competing interests.

Figures

Fig. 1
Fig. 1
Duration of delay in administration of on-demand treatment after initial recognition of HAE attack onset
Fig. 2
Fig. 2
A Reasons provided for not treating an HAE attack (n = 74, excluding those who treat all of their attacks, n = 33) – respondents could select as many as they liked B Reasons provided for waiting to treat an HAE attack at recognition of attack onset (n = 92, excluding those that immediately treat their HAE attacks, n = 15) – respondents could select as many as they liked
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References

    1. Sinnathamby ES, Issa PP, Roberts L, Norwood H, Malone K, Vemulapalli H, et al. Hereditary Angioedema: diagnosis, clinical implications, and pathophysiology. Adv Ther. 2023;40(3):814–27. 10.1007/s12325-022-02401-0. - PMC - PubMed
    1. Busse PJ, Christiansen SC, Riedl MA, Banerjo A, Bernstein JA, Castaldo AJ, et al. US HAEA medical advisory board 2020 guidelines for the management of hereditary angioedema. J Allergy Clin Immunol Pract. 2021;9(1):132–e503. 10.1016/j.jaip.2020.08.046. - PubMed
    1. Cicardi M, Agostoni A. Hereditary Angioedema. N Engl J Med. 1996;334(25):1666–7. 10.1056/NEJM199606203342510. - PubMed
    1. Riedl MA, Danese M, Danese S, Ulloa J, Maetzel A, Audhya PK. Hereditary Angioedema with normal C1 inhibitor: US survey of prevalence and provider practice patterns. J Allergy Clin Immunol Pract. 2023;11(8):2450–e66. 10.1016/j.jaip.2023.01.023. - PubMed
    1. Dreskin S. Urticaria and Angioedema. In: Goldman L, Schafer AI, editors. Cecil Medicine. 24th ed. Philadelphia, PA: Saunders Elsevier; 2011. Chapter 260.

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