Hereditary angioedema (HAE) is a rare genetic disorder that has unpredictable attacks, The recurrent attacks include painful swelling of the skin and mucous membrane, and they occur in the face, hands, feet, and gastrointestinal tract. In addition to skin swelling, a person with HAE may also experience abdominal pain, extreme fatigue, vomiting, and diarrhea.
While the attacks are unpredictable, certain factors may occasionally trigger them. These factors include minor illnesses, stress, physical trauma, or food intolerance. If you have HAE, making some lifestyle adjustments may help manage the condition.
HAE results from a mutation to the gene C1NH. This gene contains the instructions for producing a protein called C1 inhibitor. HAE typically runs in families, although it can occur spontaneously. HAE affects approximately 1 in 50,000-150,000 people globally.
Sometimes doctors misdiagnose HAE because the symptoms look similar to other common conditions like appendicitis, contact dermatitis, or irritable bowel syndrome.
Making lifestyle adjustments and possibly taking medication can help a person reduce the frequency and severity of HAE attacks. It also helps to recognize and learn to avoid triggers. Triggers will vary from person to person, but typically they include:
- intense physical activity
- stress or anxiety
- injuries
- surgery
- dental procedures
- illnesses, such as a cold or the flu
- intolerance to certain foods, such as fish, dairy, peanuts, onions, or garlic
- certain medications, such as blood pressure drugs or birth control pills
- hormonal changes, such as those that occur during pregnancy or menopause
While it is unlikely that you can avoid all triggers, you can make take some steps to help manage triggers, such as:
- keeping a journal of HAE attacks to identify potential triggers
- adjusting the diet to avoid foods that trigger attacks
- receiving vaccinations to prevent illnesses
- managing stress
- prioritizing sleep and self-care
- taking care to avoid exercise that is too strenuous and taking ample breaks during exercise
- prepare for surgery and dental work
Dental work and surgeries may occasionally trigger an HAE episode. Your doctor may prescribe a short-acting preventative treatment prior to a medical procedure to reduce the risk of an HAE attack.
These options include:
- intravenous C1 esterase inhibitor (Cinryze)
- subcutaneous C1 esterase inhibitor (Haegarda)
- lanadelumab (Takhzyro)
- berotralstat (Orladeyo)
Only berotralstat is taken orally. The other medications are delivered through injection. A person with HAE may be able to keep these medications at home and learn to self-inject when necessary. A doctor, nurse, or pharmacist can provide training on how and when to do this. On occasion, a member of the person’s family or a caregiver may be trained to deliver the medication.
It is crucial that a person with HAE manage their stress and anxiety before surgery. A doctor or dentist may sometimes prescribe mild sedatives to help control anxiety ahead of surgery.
Plan ahead when traveling
The Hereditary Angioedema Association (HAEA) recommends that people with HAE due to a deficiency in the protein C1INH should always have access to at least two standard doses of a medication that the Food and Drug Administration (FDA) has approved for HAE. They should also have a plan for accessing a healthcare facility during an attack, especially if the attack restricts breathing.
The need to have access to medications and healthcare can be an obstacle while traveling. Planning ahead helps to mitigate the risks.
People with HAE can plan for travel by:
- ensuring that they have enough medication for the duration of the trip and for a short time after the return
- carrying medication in an easily accessible carry-on bag that will be on hand at all times
- requesting a letter from a doctor that authorizes carrying this medication
- asking a doctor for a written prescription just in case more medication is necessary while traveling
- recording phone numbers for doctors, emergency contacts, and caregivers
- knowing the location of a local hospital that can provide treatment if necessary
Limit and alleviate emotional stress
Emotional stress can trigger HAE attacks. Research suggests that stress, anxiety, and depressed mood are among the most common triggers that people with HAE report. Fear of HAE attacks can also cause anxiety and depression, which may lead to a cycle of increased emotional stress and more attacks.
One study found that the restrictive measures put in place during the COVID-19 pandemic caused an increase in the number of HAE attacks in children due to anxiety, depression, stress, and fear of the virus.
It is not always possible to prevent stress completely, but there are a few ways in which people with HAE can work to limit or relieve stress. Examples include:
- trying not to commit to too many responsibilities at work or home
- prioritizing stress-relieving activities, such as yoga, listening to music, or reading
- finding time for a hobby
- spending quality time with loved ones
- having family members or friends help with chores, child care, or other responsibilities, as necessary
- taking steps to get adequate sleep
- taking time off work
Join a support group
Because HAE is rare, it may be difficult to find others who have the condition and can relate to the difficulties. Online or in-person support groups can help people with HAE feel less alone and more connected.
Social media can be a good place to find others with HAE. Facebook has a page for DiscoverHAE, which provides an opportunity to connect with other people living with HAE and join conversations with them.
The HAEA operates an online community known as the HAEA Café. Once a person with HAE has created an account, they can chat live with an HAEA patient advocate, listen to informational webinars, and engage with other people with HAE in virtual support groups.
The HAEA also runs a youth program, which organizes conferences, camps, and other events for children and adolescents living with HAE.
Consult with your doctor before trying to conceive
Unfortunately, for some moms with HAE, pregnancy and breastfeeding may trigger an attack or worsen the severity. If you have HAE, consult with your doctor before conceiving to talk about the risks.
You may also wish to consult with a genetic counselor who can talk about the risks of passing HAE to your children. Children have a 50% chance of inheriting HAE if one of their parents has the condition.
During pregnancy, a doctor will closely monitor the health of the mom and the developing baby so that they can determine which medication is most appropriate.
Prepare for attacks away from home
People with HAE may have to miss school or work due to an attack. They may also find it difficult to maintain a full-time job. However, HAE should not keep a person with HAE from pursuing their career of choice.
A person with HAE can live a close-to-normal life with treatment. The parents or caregivers of students living with HAE can inform the school of the condition and ensure that the school staff knows the actions to take in case of an attack. Carrying a medical ID card at work can give medical professionals crucial information in case of an emergency. The card should include specific instructions for treating an attack.
Final thoughts
HAE attacks can be painful. Sometimes they may be life-threatening. Still, making lifestyle adjustments and taking certain medications can help alleviate the frequency and severity of the attacks. By getting treatment, making lifestyle modifications, and planning carefully for travel, work, and school, a person with HAE can have a good quality of life.