The burden of HAE

HAE places a substantial burden on patient Quality of Life (QoL)

HAE imposes a substantial burden on patients, through its impact on physical, psychological/emotional, and social factors.¹

The unpredictability of HAE means that patients are impacted not only during attacks, but also between attacks.²

Impact during attacks

The physical burden of HAE

HAE attacks may be life-threatening if affecting the larynx.³ In a retrospective study of 242 HAE patients across 8 countries, 6.6% of patients reported that their latest attack affected the larynx.⁴

Attacks can also be painful and distressing for patients. One study reported 52% of patients experienced severe abdominal pain and 43% experienced severe nausea during their most recent attack.⁴Additionally, 38% of patients required at least one visit to the Emergency Department and 19% of patients were hospitalised due to HAE in the previous year.⁴

Break

In one study, 87% of patients described HAE-related abdominal pain as “severe to excruciating”, with some saying that they could not imagine more severe pain.⁵

Impact on work life and education

It is common that when attacks occur, patients need to take time off from work/school, which can lead to a loss of educational and career opportunities.⁴In a study of patients with HAE in the European Union, both patients and their caregivers reported losing an average of 20 days of work or school per year due to HAE.⁶

Additionally, 51% of patients reported that HAE impacted their career and/or educational progression. Patients believed HAE prevented them from applying to certain jobs (40%), caused them to switch positions at work (9%) and in some cases caused them to leave positions completely (10%).⁶

Break

In a multinational survey, patients with HAE reported:⁴

Absence from work for 8% of the prior week

A 24% loss in work productivity

Impact between attacks

Effect on emotional wellbeing

The unpredictability of HAE attacks can have a significant impact on patients’ quality of life.²For example, they might make lifestyle changes to avoid attack triggers.²Due to the constant fear of attacks, patients are also more likely to suffer higher rates of anxiety and depression than the general population, further adding to the disease burden.^2,4,7 Therefore, the burden of disease patients experience is not restricted to HAE attacks, but also includes the disruption to their quality of life between attacks.²

break

In a multinational survey of 242 patients with HAE:⁴

44.3% of patients report being ‘much’ or ‘very much’ bothered by the unpredictability of HAE attacks

38.0% of patients report moderate to severe anxiety

17.4% of patients report moderate to severe depression

Due to the genetic nature of HAE, patients may have ancestors who have died due to asphyxiation, which could worsen their worries about attacks.¹One study found that almost three quarters of patients with HAE worried about asphyxiating, greatly impacting their psychological wellbeing. This percentage was reduced to 38% after patients were taught to self-administer medication.⁸

The vicious cycle of HAE attacks:

Stress associated with unpredictable attacks can itself be a potential trigger for HAE attacks, which in turn can lead to a vicious cycle of further anxiety.⁹

Increased severity of anxiety and depression has been linked with worsening impairment across multiple measures of quality of life.⁹

The social burden of HAE

Besides the physical and psychological burden of HAE that patients experience, the disease can impact patients’ social activities and relationships. Patients can experience guilt about passing HAE onto their children, which might impact their decision on whether to have their own children.¹HAE attacks can also affect patients’ social wellbeing, as their ability to attend social/family events, participate in sports and travel may be hindered.¹⁰

It is important to discuss with your patients about how HAE may affect their work, education and social lives, and on the importance of treatment and disease management plans to help prevent their attacks and allow them to live a more normal life.

HAE imposes a burden on family members and caregivers

Patients with HAE may seek assistance from caregivers, primarily family members; 44% of patients reported seeking assistance from someone other than an HCP during their last HAE attack.⁹In addition to patients, caregivers experienced work or school absenteeism, which increased with greater pain severity and higher attack frequency.⁶A recent survey reported burdens for caregivers such as dealing with unpleasant or inconvenient infusions or injections, and watching a loved one suffer.¹¹

The burden of treatment

In addition to disease burden, patients with HAE experience a burden associated with treatment administration – whether it be the route of administration or due to poor tolerability of treatment.
In recent years, effective treatments for HAE have been developed which offer convenient administration methods such as subcutaneous injection and oral administration. It is important to regularly discuss the different treatment options available for HAE during consultations with patients in case their preferences change or new treatment options become available.

The Burden of HAE

QoL, quality of life
Bork K, et al. 2012;³ Mendivil J, et al. 2021;⁴ Banerji A, et al. 2020;⁹ Lumry W, Settipane R. 2020⁷

Unmet need in HAE treatment

In a 2019 multinational survey of patients with HAE, despite 62% of patients receiving LTP, patients reported a mean of 12.5 attacks in the past 6 months.⁴

As long as patients continue to experience attacks, there is still a need to develop new, more effective and more convenient treatments.

References

Bygum A, Busse P, Caballero T, Maurer M. Disease Severity, Activity, Impact, and Control and How to Assess Them in Patients with Hereditary Angioedema. Front Med (Lausanne). 2017;4:212.
Bork K, Anderson JT, Caballero T, Craig T, Johnston DT, Li HH, et al. Assessment and management of disease burden and quality of life in patients with hereditary angioedema: a consensus report. Allergy Asthma Clin Immunol. 2021;17(1):40.
Bork K, Hardt J, Witzke G. Fatal laryngeal attacks and mortality in hereditary angioedema due to C1-INH deficiency. J Allergy Clin Immunol. 2012;130(3):692-7.
Mendivil J, Murphy R, de la Cruz M, Janssen E, Boysen HB, Jain G, 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(1):94.
Bork K, Staubach P, Eckardt AJ, Hardt J. Symptoms, Course, and Complications of Abdominal Attacks in Hereditary Angioedema Due to C1 Inhibitor Deficiency. Am J Gastroenterol. 2006;101(3):619-27.
Aygören-Pürsün E, Bygum A, Beusterien K, Hautamaki E, Sisic Z, Wait S, 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.
Lumry WR, Settipane RA. Hereditary angioedema: Epidemiology and burden of disease. Allergy Asthma Proc. 2020;41(Suppl 1):S08-s13.
Bygum A. Hereditary angioedema - consequences of a new treatment paradigm in Denmark. Acta Derm Venereol. 2014;94(4):436-41.
Banerji A, Davis KH, Brown TM, Hollis K, Hunter SM, Long J, et al. Patient-reported burden of hereditary angioedema: findings from a patient survey in the United States. Ann Allergy Asthma Immunol. 2020;124(6):600-7.
Longhurst HJ, Bork K. Hereditary angioedema: an update on causes, manifestations and treatment. Br J Hosp Med (Lond). 2019;80(7):391-8.
Craig TJ, Banerji A, Riedl MA, Best JM, Rosselli J, Hahn R, et al. Caregivers' role in managing hereditary angioedema and perceptions of treatment-related burden. Allergy Asthma Proc. 2021;42(3):S11-s6.