“HAE prevention plan: Tips for stopping an attack before it happens”

“Hereditary angioedema (HAE) is a rare genetic disorder that can cause swelling attacks in the limbs, face, upper airways, abdomen, and genitalia.

HAE can occur anywhere on the body. The most common areas are the lips, hands, feet, and the skin around the eyes. It also may occur in the mucosal linings of the respiratory and digestive tracts.

Swelling attacks can happen regularly, often without a known HAE trigger. HAE attacks can be prompted by trauma; viral infections; stress; medical and dental procedures, including surgery and having a tooth pulled; hormonal fluctuations; allergens; and physical activity.

HAE attack symptoms include swollen areas of skin that may be smooth or raised. Symptoms can vary from person to person or even from attack to attack in the same person, and can include non-swelling symptoms.

HAE attack treatment includes learning what the triggers are, which are individual to each person, and short- and long-term preventive treatment with medications. With the right medical care, a person may be symptom-free for years.

In some cases, severe swelling may affect the intestines, causing nausea and vomiting, along with intense pain. Some people may have swelling in the mouth and throat, making it difficult to swallow, speak, and breathe. Breathing problems can quickly become life-threatening emergencies and require immediate medical treatment.

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HAE prevention plan: Tips for stopping an attack before it happens
Last updated Oct. 2, 2023, by Susie Strachan
[emoji736] Fact-checked by Patricia Silva, PhD
What causes an HAE attack?Attack triggersTips for avoiding triggersPreventive treatments

Hereditary angioedema (HAE) is a rare genetic disorder that can cause swelling attacks in the limbs, face, upper airways, abdomen, and genitalia.

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HAE can occur anywhere on the body. The most common areas are the lips, hands, feet, and the skin around the eyes. It also may occur in the mucosal linings of the respiratory and digestive tracts.

Swelling attacks can happen regularly, often without a known HAE trigger. HAE attacks can be prompted by trauma; viral infections; stress; medical and dental procedures, including surgery and having a tooth pulled; hormonal fluctuations; allergens; and physical activity.

HAE attack symptoms include swollen areas of skin that may be smooth or raised. Symptoms can vary from person to person or even from attack to attack in the same person, and can include non-swelling symptoms.

HAE attack treatment includes learning what the triggers are, which are individual to each person, and short- and long-term preventive treatment with medications. With the right medical care, a person may be symptom-free for years.

In some cases, severe swelling may affect the intestines, causing nausea and vomiting, along with intense pain. Some people may have swelling in the mouth and throat, making it difficult to swallow, speak, and breathe. Breathing problems can quickly become life-threatening emergencies and require immediate medical treatment.

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What causes an HAE attack?
For people living with HAE, swelling can partly be caused by an inherited genetic mutation.

HAE attacks are triggered by an overproduction of a protein called bradykinin, which increases blood vessel leakiness and causes edema. Swelling occurs when bradykinin is activated in the deep layers of the skin, causing fluid to flow from the bloodstream into nearby tissues.

There are three types of HAE:

Types 1 and 2 are caused by mutations in the SERPING1 gene, which is involved in making a type of protein called C1-inhibitor. When there isn’t enough of this protein, bradykinin levels can get too high and cause an attack.

Type 3 HAE is caused by mutations in genes other than SERPING1, including the F12 gene. These mutations can trigger an inflammatory response and subsequently make bradykinin levels get too high and lead to an attack.

HAE attack triggers
Hereditary angioedema triggers include trauma, viral infections, stress, and medical or dental procedures. Other triggers are allergens, hormonal fluctuations, and repetitive physical activities.

Allergens
Exposure to common foods or environmental factors can trigger HAE attacks. A small study reported foods including milk, apple, pineapple, onion, garlic, fish, citrus fruits, strawberries, and cheese could be possible triggers. HAE attacks can seem similar to other kinds of allergic reactions.

Hormonal fluctuations
Changes in hormone levels also can trigger hereditary angioedema attacks.

Puberty can cause HAE symptoms to become more frequent and severe, particularly for girls.

Hormonal fluctuations during menstruation, menopause, pregnancy, and breastfeeding can lead to more frequent attacks, according to the International WAO-EAACI guideline for the management of HAE.

Birth control pills, which contain artificial female hormones, can trigger attacks.

Pregnant women should be monitored by an HAE expert, in addition to their obstetrician-gynecologist. With good management of the disease, most women will have a successful pregnancy and delivery.

Physical activities
Doing repetitive activities that put pressure on one part of the body for a long period of time may trigger swelling attacks in people with HAE. This could include gripping a tennis racket, typing, or tightly holding a tool such as a hammer.

Physical trauma, illness, and medication
Physical trauma, including from surgery or an accident, can cause an HAE attack in the affected area of the body.

Infections are a common trigger for HAE attacks in children and adults, so steps should be taken to limit exposure to viruses and to stay up to date with recommended vaccinations.

High blood pressure medications containing ACE inhibitors may increase the frequency or start of HAE attacks and should be avoided.

Emotional stress
A work-life imbalance, family problems, and the loss of a loved one can be some sources of emotional stress. People living with HAE also may find having unexpected, disfiguring, and painful attacks stressful.

Stress can lead to anxiety and depression, and affect quality of life.

Surgery
Procedures associated with the upper digestive tract, such as surgery or dental surgery, can cause swelling at the site of the surgery, usually within 48 hours.

For example, having a tooth removed causes swelling in more than one-third of people with HAE if preventive treatment is not provided before the procedure.”

Hereditary angioedema triggers: Tips for stopping an HAE attack | Angioedema News

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