Rapid swelling from hereditary angioedema can result from a variety of triggers. Doctors may use prophylactic medications to try and prevent these episodes, but managing stress and developing healthy lifestyle habits can also help.

Hereditary angioedema (HAE) is a rare genetic condition caused by a genetic variant passed down from parent to child.

It can include rapid, painful episodes of fluid buildup under the skin (angioedema), most often affecting the face, extremities, genitals, and buttocks. For some, HAE can also cause swelling in the gastrointestinal (GI) system, urinary tract, or upper airway.

HAE attacks can be painful and unpredictable. They can affect multiple areas of the body during a single episode. In rare cases, HAE can affect your ability to breathe and become life threatening.

It’s not always possible to predict an HAE attack, but there are steps you can take to help reduce the frequency and severity of HAE episodes.

HAE attacks are caused by a buildup of bradykinin in your body. Bradykinin is a small peptide and part of the kallikrein-kinin system, which works to regulate processes in your body related to inflammation, pain, and blood pressure.

Bradykinin plays a critical role in vasodilatation, or the widening of blood vessels. It also promotes vascular permeability, a state in which the cells of blood vessels separate and allow fluids to pass through, traveling into the surrounding tissue.

With HAE, a genetic variant causes your body to not make enough of a protein called C1 esterase inhibitor (C1-INH), or it may prevent C1-INH from functioning correctly.

When C1-INH levels aren’t sufficient, the kallikrein-kinin system can become unregulated and levels of bradykinin elevate and can lead to angioedema.

For many people, certain triggers can cause HAE attacks by further stimulating your kallikrein-kinin system and increasing bradykinin production.

As a part of your inflammatory response, the kallikrein-kinin system can become activated by stimuli like mental or physical stress, injury, hormonal changes, and some medications, including angiotensin-converting enzymes (ACE) inhibitors, that may be used to treat other conditions.

Triggers are different for everyone, but common examples include:

  • illnesses and infections like the cold or flu
  • anxiety
  • stress
  • injury
  • surgery
  • dental procedures
  • exercise
  • repetitive physical activities (even low impact like typing)
  • hormone changes during the menstrual cycle
  • pregnancy

It’s not always possible to prevent HAE attacks. Episodes of hereditary angioedema aren’t always linked to a discernable trigger and can develop naturally as bradykinin accumulates in your body.

When an attack occurs, medications that inhibit bradykinin or provide your body with functional C1-INH can deliver rapid relief. Some of these may be administered by your doctor while others can be self-administered.

Common medications for acute and immediate symptom relief include:

  • icatibant (Firazyr)
  • ecallantide (Kalbitor)
  • Ruconest
  • Berinert

To help reduce the frequency and severity of attacks, your doctor may discuss the following options as part of your comprehensive management plan:

Prophylactic medications

Prophylactic medications are those used as long-term, preventive therapies before you encounter a potential trigger, such as a dental cleaning.

Like acute treatments, these medications also work by increasing the body’s amount of C1-INH or by regulating pathways involved with bradykinin.

Common prophylactic medications include:

  • Cinryze
  • Haegarda
  • lanadelumab (Takhzyro)
  • berotralstat (Orladeyo)

Not everyone with HAE needs prophylactic therapies. How C1-INH deficiency or dysfunction affects you can range in severity. Some people may never develop symptoms or may experience mild symptoms that resolve on their own within a few days.

Identifying and tracking triggers

Understanding and identifying your personal HAE triggers is a big part of successful disease management. When you know what causes an HAE attack for you, you can take the steps necessary to avoid that trigger or receive prophylactic treatment.

Journaling is one way to look for patterns over the course of a life-long condition like HAE. By recording your symptoms and the circumstances around them, you can start to identify your triggers and the behaviors that may lead to an attack.

Building healthy lifestyle habits

Bradykinin is a natural part of inflammatory responses in your body. The more you can do to prevent unnecessary inflammation, the more effectively you may be able to manage symptoms of HAE.

Making healthy lifestyle changes can help reduce your chance of physical injury or chronic illness and can keep your body functioning at its peak performance.

Changes to consider include:

  • eating a balanced, nutrient-dense diet
  • maintaining an optimal weight
  • exercising regularly
  • staying hydrated
  • getting plenty of quality sleep
  • eliminating smoking and substance use

Developing health hygiene strategies

No matter how mindful you are about your general health, viral and bacterial exposure can happen. You can reduce your chance of infection by:

  • washing your hands regularly
  • using hand sanitizers
  • wearing a mask in high risk or densely populated areas
  • staying current with vaccinations
  • engaging in social distancing
  • using sterile practices for administration of at-home medical injections

Finding ways to reduce and cope with stress

Stress is one of the most common triggers reported by people with HAE. Learning to manage stress both long-term and in the moment can help reduce its impact on your kallikrein-kinin system.

Long-term stress management options could include:

  • regular physical activity
  • meditation
  • mind-body arts, like tai chi
  • working with a mental health professional

In-the-moment stress reduction options can include:

  • progressive muscle relaxation
  • deep breathing techniques
  • journaling
  • going for a walk
  • sensory grounding (using a stress ball, splashing your face with cold water, tapping your forehead)
  • repeating affirmations

Hereditary angioedema is a rare genetic condition that occurs when your body doesn’t make or use C1-INH effectively. It causes painful, rapid swelling under your skin, and attacks or episodes of swelling can be brought on by a variety of inflammatory triggers.

Prophylactic treatment of HAE can help reduce how often you experience attacks and how severe symptoms become, but making beneficial lifestyle changes and taking steps to prevent infection and reduce stress can also help.