This website is intended for users outside of the US and UK. sign up to stay in the know
impact of HAE

the burden of hereditary angioedema may persist beyond the attack

You may already know the signs and symptoms of hereditary angioedema (HAE), but assessing its full impact on your patients may help inform treatment goals and planning.

hereditary angioedema limits the way patients live their lives

Even after the physical symptoms of an attack subside, a patient may still be affected by HAE. For many, the anticipation of an HAE attack dictates what patients can or cannot do.1

Due to the unpredictable nature of the disease, some patients reported high levels of distress and anxiety in everyday life. These feelings are attributed to1,2:
- Interrupted social life
- Disrupted educational aspirations
- Anxiety of future attacks
- Fear of passing HAE to their children

In addition to absences and lost productivity, patients reported that HAE has hindered their career or education by6:
- Impacting educational choices*
- Preventing them from applying to certain jobs
- Causing them to resign or switch positions within a company

good to know

38% and 14% of patients with HAE had clinically meaningful anxiety and depression, respectively, vs global population rates of 3.6% and 4.4%.3,4*

Because of this, patients often forgo1,2:
- Travel and vacation
- Certain hobbies
- Sports and other physical activities
- Social events

This goes beyond cancelling plans–some patients would not consider planning these activities in the first place.1

Watch and learn:

Hear one patient's experience in her own words

Reassessing the True Burden of Hereditary Angioedema

See how you and your patients can uncover the burden of HAE

*Based on the Work Productivity and Activity Impairment-General Health questionnaire (N=457), distributed in the United States from November 2007 to January 2008.6

Based on quantitative survey responses (N=186) in the 2011 Hereditary Angioedema Burden of Illness Study in Europe.3

talking to your patients

talking to your patients about the total impact of hereditary angioedema

You strive to limit its impact on your patients. But how forthcoming are your patients on all the ways HAE affects them? Many patients may not even realise just how pervasive the negative impact of HAE can become.1

To help assess the full impact of their HAE, consider asking your patients:

  • How often do you worry about or plan around the unpredictability of a future attack?
  • Have you limited your travel due to fear of an attack?
  • Have you avoided social plans and other activities?
  • Have you missed opportunities for educational or professional development?

patient questionnaire

Share these questions with your patient to help them identify areas in their life where they may be struggling due to HAE.

managing HAE

creating a treatment plan to manage their hereditary angioedema

All patients with HAE should have an individualised treatment plan that is carefully developed to fit their needs and lifestyle. Individualised treatment plans should address preventative measures, home care, and self-administration, as well as an effective emergency (on-demand) treatment plan with clear instructions on how best to use medications to treat HAE attacks.7

2017 World Allergy Organization Treatment Guideline

It is recommended that all patients with HAE should be educated about possible triggers which may induce HAE attacks.7

on-demand treatment

  • All attacks should be considered for on-demand treatment and treated as early as possible. Any attack potentially affecting the upper airway should be treated7
  • Attacks can be treated with C1-INH or icatibant (a bradykinin receptor antagonist). Ecallantide (kallikrein inhibitor) can be used to treat attacks but is only approved in the United States7
  • All patients should have on-demand treatment for 2 attacks, to be carried with them at all times7

preventative treatment

  • Short-term prophylaxis: Case reports and series suggest that despite prophylaxis, swellings may occur even after relatively minor procedures. However, several reports document a reduction in the incidence of swelling for both adults and children with preprocedural prophylaxis, and the response appears to be dose related. Preprocedural prophylaxis with C1-INH concentrate is therefore recommended for all medical, surgical, and dental procedures associated with any mechanical impact to the upper aerodigestive tract7
  • Long-term prophylaxis: Consider for patients who face events in life that are associated with increased disease activity; patients should be evaluated for long-term prophylaxis at every visit, taking into account disease burden and patient preference7
    • Dosage and treatment interval should be adapted as needed to minimise burden of disease7

good to know

Androgens are recommended as second-line treatment, with C1-INH as first line. Androgens must be regarded critically, especially in light of their adverse androgenic and anabolic effects, drug interactions, and contraindications.7

Availability of therapies differs by country.

Watch and learn:

New Views on Prevention & On-Demand Therapy: Highlights from the 2017 WAO/EAACI Guideline

Watch Dr Marcus Maurer review some updates to the treatment guideline

Please note that current treatment guidelines are for patients with Type 1 and Type 2 HAE only; the pathogenesis of other forms of HAE is not well-characterised, and therefore, therapeutic options may not be similarly effective.7

sign up to stay in the know

Sign up to be the first to know when new resources are made available, and to stay up to date on the latest in HAE.

First Nameplease enter your first name
Last Nameplease enter your last name
Specialtyplease select a specialty
Countryplease select your country
Emailplease enter a valid email address
Terms + Conditions
Click here for a refresher on identifying and diagnosing HAE
References: 1. Bygum A, Aygören-Pürsün E, Beusterien K, et al. Burden of illness in hereditary angioedema: a conceptual model. Acta Derm Venereol. 2015;95(6):706-710. 2. Longhurst H, Bygum A. The humanistic, societal, and pharmaco-economic burden of angioedema. Clin Rev Allerg Immunol. 2016;51(2):230-239. 3. Caballero T, Aygören-Pürsün E, Bygum A, et al. The humanistic burden of hereditary angioedema: results from the Burden of Illness Study in Europe. Allergy Asthma Proc. 2014;35(1):47-53. 4. World Health Organization. Depression and Other Common Mental Disorders: Global Health Estimates. Geneva, Switzerland: WHO Document Production Services; 2017. 5. Aygören-Pürsün E, Bygum A, Beusterien K, et al. Socioeconomic burden of hereditary angioedema: results from the hereditary angioedema burden of illness study in Europe. Orphanet J Rare Dis. 2014;9:99. doi: 10.1186/1750-1172-9-99. 6. Lumry W, Castaldo A, Vernon M, Blaustein MB, Wilson DA, Horn PT. The humanistic burden of hereditary angioedema: impact on health-related quality of life, productivity, and depression. Allergy Asthma Proc. 2010;31(5):407-414. 7. Maurer M, Magerl M, Ansotegui I, et al. The international WAO/EAACI guideline for the management of hereditary angioedema—the 2017 revision and update. Allergy. 2018. doi: 10.1111/all.13384.