Hereditary angioedema (HAE) is a rare genetic disorder that causes recurring episodes of severe swelling throughout the body.
Also called attacks, episodes of HAE are often unpredictable, painful, and life-threatening. Episodes that affect the airways can impair a person’s ability to breathe. Episodes that cause inflammation in the abdomen can cause symptoms so severe that a person may need to be hospitalized.
When an attack occurs, people with HAE will need medications to reverse the attack. These are called on-demand or acute therapies. Everyone with HAE should work with a healthcare provider to prepare for attacks. They will need training on how to take a rescue medication (if it is self-administered) or where to seek treatment (if a rescue medication needs to be administered by a healthcare provider).
They should also have a plan on where to seek emergency medical care and how to recover once an attack is over.
In addition to rescue medications, there are also medications that can prevent HAE attacks. These are called preventive therapies or prophylactic therapies.
In the past, preventive therapies were reserved for very severe cases of HAE, or instances where a person was at a higher risk of an HAE episode—such as a surgical procedure, dental work, or a period of high stress, which are all known triggers.
Today, preventive therapies have become an important part of standard treatment for many people with HAE—many are taking medications on an ongoing schedule to prevent attacks.
This is helped by the fact that numerous new medications have become available, giving healthcare providers and people with HAE more options.
If you are living with HAE, it is worth talking to your healthcare providers about these treatment options. Preventive therapies can help reduce the number of attacks a person experiences and reduce the severity of symptoms during an attack.
In addition to fewer attacks and less severe symptoms, here are five possible benefits of preventive therapy.
Better mental health
Living with HAE can be a burden on a person’s mental health. Anyone living with HAE is living with some degree of uncertainty—When will the next attack occur? Will it be severe? Will I need to go to a hospital? What plans will it disrupt? How long will it take to get back to feeling normal?
Some research has found that people who are treated with long-term preventive therapy showed fewer symptoms of depression and anxiety than those who were only treated with acute therapies.
Less emergency care
People living with HAE want to avoid attacks that require urgent care or hospital visits. Beyond the fact that attacks that require emergency care are typically more severe (more painful, longer recovery time, more likely to be life threatening), urgent care and hospital visits are expensive.
Improved quality of life
One of the hardest aspects of living with a chronic and unpredictable health condition like HAE is the way it interferes with every other part of your life—your ability to maintain friendships and other relationships, work or go to school, pursue goals and interests, your moods and how you feel about yourself. Collectively, this is referred to as “quality of life.”
People with HAE who are treating with long-term preventive therapies report better quality of life, better health, and better performance in work and school.
Easier on caregivers
Many people with HAE rely on the help of a caregiver—oftentimes, a family member or other loved one. Caregivers may be directly involved with care by helping administer medications or taking care of a loved one while that person is recovering from an HAE attack.
They may also help with the practical aspects of living with HAE, such as taking on physically demanding errands or chores that could trigger an attack. And of course, caregivers can be an important source of emotional support.
HAE therapies that lead to fewer and less severe attacks will also benefit caregivers, who will need to spend less time helping a loved one address and cope with attacks—and hopefully less emotional energy, worrying about the chances of their loved one experiencing a severe attack.
Remember that HAE is a different experience for everyone. Your best source of information about your treatment options will be your healthcare provider.
Sources: MedlinePlus. "Hereditary angioedema." + 11
- MedlinePlus. "Hereditary angioedema."
- William R. Lumry. "Current and Emerging Therapies to Prevent Hereditary Angioedema Attacks."
- New Horizons in the Diagnosis and Treatment of Hereditary Angioedema: Overcoming Barriers to Management and Improving Patient Outcomes, 2018. Vol. 24, No. 14.
- HAE International. "How Can HAE Be Treated?"
- US Hereditary Angioedema Association. "Triggers: What Causes Hereditary Angioedema Attacks?"
- Huamin Henry Li. "Hereditary angioedema: Long-term prophylactic treatment." Allergy & Asthma Proceedings, 2020. Vol. 41, Suppl. 1.
- William Raymond Lumry. "Hereditary Angioedema: The Economics of Treatment of an Orphan Disease." Frontiers in Medicine. February 16, 2018.
- Julia Hews-Girard and Marilyn Dawn Goodyear. "Psychosocial burden of type 1 and 2 hereditary angioedema: a single-center Canadian cohort study." Allergy, Asthma & Clinical Immunology, 2021. Vol. 17.
- J. Zarnowski, M. Rabe, P. Kage, J.C. Simon, R. Treudler. "Prophylactic Treatment in Hereditary Angioedema Is Associated with Reduced Anxiety in Patients in Leipzig, Germany." International Archives of Allergy and Immunology, 2021. Vol. 182, No. 9.
- World Health Organization. "WHOQOL: Measuring Quality of Life."
- Timothy J. Craig, Aleena Banerji, et al. "Caregivers' role in managing hereditary angioedema and perceptions of treatment-related burden." Allergy and Asthma Proceedings, 2021. Vol. 42, No. 3.
- Jose Granero-Molina, Francisco Sanchez-Hernandez, et al. "The Diagnosis of Hereditary Angioedema: Family Caregivers’ Experiences." Clinical Nursing Research. June 4, 2018.
