PAGE CONTENTS
  • Patient Education

Hereditary angioedema (HAE) is a rare autosomal dominant disease in which patients experience unpredictable and recurrent attacks of swelling in different body parts.1 The subcutaneous tissues, gastrointestinal tract, and upper airway are most commonly involved in HAE episodes, with about 50% of patients suffering at least 1 laryngeal attack during their lifetime.2,3

Living with HAE can be associated with a poor quality of life due to the unpredictable frequency pattern of attacks, the pain that occurs during abdominal episodes, and the risk of death from asphyxiation when a laryngeal attack occurs, which may require a tracheotomy.1 

Patients with HAE may have difficulties in navigating the disease. Making plans, either related to their career development or to personal activities, may pose challenges due to the fear of experiencing an attack.1 

Identifying and Avoiding Triggers 

Patients with HAE should be able to distinguish an HAE episode from allergic angioedema. HAE attacks present with a slow start, do not respond to medications used to control allergies (such as antihistamines), and may last for several days.1

The frequency at which HAE attacks occur is not the same for each patient. Frequent attacks may derive from factors or events, which include psychological and physical stress; however, most of the episodes are not related to any specific trigger.1 Patients who experience frequent attacks may also exhibit several warning signs, called prodromal symptoms, that may alert the patient to a future attack. These symptoms range from extreme fatigue to rashes.4

Other common triggers of HAE attacks include illnesses such as infections, trauma, surgical and dental procedures, hormonal changes (from menstruation or pregnancy), prescription and over-the-counter medications such as hormonal contraceptives and ibuprofen, strenuous exercise, and mechanical pressure from repetitive physical activities such as typing.4-7 

Avoiding some of these triggers may involve simple lifestyle changes such as writing down HAE attacks to help identify common triggers and preparing prophylactically for surgical or dental procedures.8

Traveling With Hereditary Angioedema

Patients with HAE should consider a few activities before traveling that may help them prepare for a successful trip. These include9:

– Carrying a card with identification as well as their primary doctor’s identification and emergency contacts;

– Carrying a prescription for HAE medication and a doctor’s note authorizing the transport of this medication on board in case of air traveling;

– Ensuring that their medication is always at hand in a carry-on bag;

– Ensuring enough medication is available for the traveling time;

– Knowing the medical options available at their destination.

Pregnancy in Hereditary Angioedema

Close monitoring is recommended for pregnant women with HAE since pregnancy and breastfeeding may either exacerbate or alleviate disease symptoms.7,8,10 Pregnant women should also be encouraged to detect early signs of HAE attacks during their pregnancy.10 

Several medications such as anabolic steroids (ie, danazol), ecallantide, lanadelumab, and berotralstat are not recommended during pregnancy or breastfeeding.7,10 Women should therefore discuss other HAE therapy alternatives with their medical team.10 Additionally, a discussion with a genetic counselor may help clarify doubts and questions about the risk of passing HAE to their children.8

Managing Emotional Stress and Seeking Support

Anxiety and depression can develop due to the fear of developing HAE attacks, and both anxiety and stress are frequent triggers of HAE.8,11,12 Patients with HAE may reduce stress by including stress-relieving activities such as yoga in their daily routines, getting enough sleep, and reducing their responsibilities at work or even taking time off.8

Different organizations can help patients with HAE and their caregivers or family members find emotional support and up-to-date information about the disease. These include the US Hereditary Angioedema Association (HAEA) and the nonprofit network of patient associations, HAE International (HAEi).13,14

In addition to providing access to quality sources of information, different organizations represent opportunities for patients to join a support group or share experiences with other HAE patients. Through these groups, patients may feel connected and more able to deal with loneliness. Social media platforms that may be used by patients with HAE include the Facebook page for Discover HAE and the HAEA Café, which provide opportunities for patients to connect with virtual support groups.15,16

References

1. Katelaris CH. Self-management plans in patients with hereditary angioedema: strategies, outcomes and integration into clinical care. J Asthma Allergy. 2020;13:153-158. doi:10.2147/JAA.S200900

2. Bork K, Anderson JT, Caballero T, 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. doi:10.1186/s13223-021-00537-2

3. Bork K, Siedlecki K, Bosch S, Schopf RE, Kreuz W. Asphyxiation by laryngeal edema in patients with hereditary angioedema. Mayo Clin Proc. 2000;75(4):349-354. doi:10.4065/75.4.349

4. Hereditary angioedema patient information. HAE UK. Accessed June 14, 2022.

5. Bork K, Fischer B, Dewald G. Recurrent episodes of skin angioedema and severe attacks of abdominal pain induced by oral contraceptives or hormone replacement therapy. Am J Med. 2003;114(4):294-298. doi:10.1016/s0002-9343(02)01526-7

6. Atkinson JC, Frank MM. Oral manifestations and dental management of patients with hereditary angioedema. J Oral Pathol Med. 1991;20(3):139-142. doi:10.1111/j.1600-0714.1991.tb00908.x

7. 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. Published online January 10, 2022. doi:10.1111/all.15214

8. Cafasso J. Living with hereditary angioedema (HAE). Medical News Today. August 30, 2021. Accessed June 14, 2022.

9. Travel. US Hereditary Angioedema Association (HAEA). Accessed June 14, 2022.

10. Pregnancy. US Hereditary Angioedema Association (HAEA). Accessed June 14, 2022.

11. Savarese L, Bova M, De Falco R, et al. Emotional processes and stress in children affected by hereditary angioedema with C1-inhibitor deficiency: a multicenter, prospective study. Orphanet J Rare Dis. 2018;13(1):115. doi:10.1186/s13023-018-0871-x

12. Fouche AS, Saunders EFH, Craig T. Depression and anxiety in patients with hereditary angioedema. Ann Allergy Asthma Immunol. 2014;112(4):371-375. doi:10.1016/j.anai.2013.05.028

13. About us. US Hereditary Angioedema Association (HAEA). Accessed June 14, 2022.

14. Who is HAEI? HAE International (HAEi). Accessed June 14, 2022.

15. Connect with others. Discover HAE. Accessed June 14, 2022.

16. HAEA café portal. US Hereditary Angioedema Association (HAEA). Accessed June 14, 2022.

Reviewed by Harshi Dhingra, MD, on 6/20/2022.

READ MORE ON