Hereditary angioedema is a rare genetic disease characterized by recurrent episodes of severe swelling. The swellings usually appear on the lips and around the eyes, but also can affect the mouth, tongue, throat, and intestines, causing serious, sometimes life-threatening, problems.
Episodes of swelling in hereditary angioedema happen regularly and over the long-term, usually without an external trigger. This can happen with varying frequency, but may be as frequent as once or twice a week if left untreated.
Acute attacks of swelling also can be triggered by stress. This includes physical stress, such as injury, pain, viral infections, and some medical and dental procedures including surgery, mental stress, including stress from life events and school or work, clinical depression, or anxiety, or sometimes, stress originating from the disease itself.
What causes hereditary angioedema?
The swellings in hereditary angioedema occur due to the overproduction of a substance called bradykinin. Bradykinin regulates blood pressure and inflammation by causing small blood vessels to dilate (widen). When the levels of bradykinin are too high, the blood vessels become “leaky,” allowing fluid to accumulate in the surrounding tissue and causing swelling. The periodic swelling episodes in hereditary angioedema occur because bradykinin levels get too high over time.
There are a few ways this usually happens:
Types 1 and 2 hereditary angioedema are caused by mutations in the SERPING1 gene, which carries the instructions for making the C1-inhibitor protein. These mutations make the protein unable to prevent two other proteins, plasma kallikrein and coagulation factor 12, from promoting the production of bradykinin.
Type 3 hereditary angioedema is caused by mutations in the F12 gene, which encodes for coagulation factor 12. The protein becomes overactive and stimulates the inflammatory response more than usual. This results in higher bradykinin production.
How is stress connected to swelling episodes?
Stress can be caused by life events such as problems with family, work, or school. The emotional and physical pressures of hereditary angioedema itself, such as concern for the future, the unpredictability of attacks, or the fear of passing on the disease to one’s children, also can be a source of constant stress. Constant stress can manifest as excessive worrying, as in the case of clinical anxiety and fatalism and depression, which can, in turn, lead to more frequent attacks.
This is because stress changes the chemistry of the body drastically, increasing the risk of many diseases. Many of the hormones released during stress, including cortisol, contribute to inflammation in the body. Because of these hormones, allergies can worsen, overall health and behavior patterns can be affected and, very relevant to angioedema, excess bradykinin can be produced.
Individuals with hereditary angioedema already have high levels of bradykinin, so even a small increase in the production of bradykinin from stress can easily put them over the threshold for a swelling attack.
How can stress-related angioedema attacks be reduced?
Stress can be difficult to avoid outright, especially when it is triggered by the disease itself. However, there are ways to manage it.
Overall health is important to keeping stress under control. Eating a complete, nutritious diet and getting a healthy amount of exercise can reduce inflammation and keep away other potential triggers of angioedema attacks.
Taking care of one’s mental health is also vital. Experiencing swelling episodes can be terrifying and traumatic, and the size and location of even harmless swellings can be embarrassing or upsetting. Especially since the attacks can be unpredictable in both frequency and severity, there is a very high risk of developing anxiety and depression.
Anti-anxiety medications and antidepressants can help support mental health when prescribed, and so can talk therapy or other methods. Spending time with friends or taking the time to relax can help reduce stress. When not relaxing, reducing procrastination and managing work time also is important.
Finding the balance between school, work, and life can help improve one’s mental health, reduce stress, and therefore reduce the frequency of angioedema attacks.
Getting appropriate medical treatment
Hereditary angioedema is a highly treatable condition. With the right medical care, a person can be symptom-free for years.
Medications should be discussed with a medical professional, but may include C1 inhibitor-boosting medicines such as Haegarda, Cinryze, and Kalbitor. These may be taken to prevent episodes of swelling, or during the episode to reduce swelling.
Planning ahead and being prepared
The more one prepares and plans for what to do during a possible angioedema attack, the easier it will be to get medical care.
Once prescribed, hereditary angioedema medications always should be kept filled and up-to-date, especially when traveling. In addition, keeping Firazyr nearby and easily accessible for immediate, self-treatment of a life-threatening swelling attack is a must.
A plan for contacting medical services in case of a severe, life-threatening attack also should be put in place. Patients are advised to be aware of hospital locations, and make sure they and the people around them know what to do in case of an angioedema attack.
Not only can such planning save one’s life, or make swelling episodes easier to deal with, but it also can reduce the stress the disease causes and, in turn, reduce the frequency of attacks.
Angioedema News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.