Although hereditary angioedema (HAE) attacks are often unpredictable, factors such as stress, minor illnesses, physical trauma, or food intolerance can sometimes trigger them. For this reason, certain lifestyle adjustments can help a person manage and live with HAE.
HAE is a rare genetic disorder that can cause recurrent attacks involving painful swelling of the skin and mucous membranes. These attacks can affect the face, feet, hands, and gastrointestinal tract. Along with skin swelling, a person with HAE can experience extreme fatigue, abdominal pain, nausea, vomiting, and diarrhea.
HAE is most commonly due to mutations in a gene called C1NH. This gene provides instructions for producing a protein called C1 inhibitor (C1INH). HAE usually runs in families, but it can sometimes occur spontaneously. The disorder affects an estimated 1 in 50,000–150,000 people worldwide.
As HAE symptoms can look very similar to the symptoms of other common conditions, such as contact dermatitis, appendicitis, or irritable bowel syndrome, doctors sometimes misdiagnose it.
Making specific lifestyle adjustments and possibly taking medication can help a person reduce the frequency and severity of HAE attacks.
Understanding the factors that trigger HAE attacks can help people with HAE better manage their condition. HAE triggers vary from person to person. They may include:
- intense physical activity
- stress or anxiety
- 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
Although it is not possible to avoid all triggers, a few lifestyle changes can help people manage them. Examples include:
- 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
Surgeries and dental work can trigger HAE episodes. A doctor may prescribe a short-acting prophylactic — a preventive treatment — that a person can take before certain medical procedures to reduce the likelihood of HAE attacks.
Currently available prophylactic options include:
- intravenous C1 esterase inhibitor (Cinryze)
- subcutaneous C1 esterase inhibitor (Haegarda)
- lanadelumab (Takhzyro)
- berotralstat (Orladeyo)
Other than berotralstat, which a person takes orally, these medications require injecting. A doctor or pharmacist can provide training for a person with HAE on how to self-inject these medications at home. Alternatively, they can show a person’s household member or caregiver how to deliver the drug.
It is also essential that people with HAE manage anxiety and stress prior to surgery, as emotional stress can also trigger attacks. A doctor or dentist may sometimes prescribe mild sedatives to help control anxiety ahead of surgery.
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 make traveling difficult. Planning can help reduce the risks.
People with HAE can plan for a trip 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 on board a flight, if necessary
- 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
Emotional stress can trigger HAE attacks. Research suggests that stress, anxiety, and low 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.
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
It is a good idea for people experiencing symptoms of anxiety or depression to talk with a mental health professional, such as a psychologist or psychiatrist.
As HAE is a rare disease, people with the condition may find it difficult to find others who can relate to their situation. Online or in-person support groups can help people with HAE feel more connected and less alone.
Social media is a good place to find others with HAE. For example, the Facebook page for DiscoverHAE 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.
For some people with HAE, pregnancy and breastfeeding can trigger attacks or worsen their severity. When trying to conceive, a person with HAE should speak with their doctor in advance to discuss the risks.
A doctor — ideally a genetic counselor — can also discuss the risk of passing HAE to children. Children have a 50% chance of inheriting HAE if one of their parents has the condition.
During a pregnancy, a doctor will closely monitor the health of the person and the developing baby so that they can determine which medication is most appropriate.
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.
People with HAE who are working may find it beneficial to talk with human resources (HR). A person with HAE can ask HR about company policies or accommodations for their situation. 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.
HAE attacks can be painful and sometimes life threatening. However, people can often manage the condition through lifestyle adjustments and medications.
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.