ManagingHereditary Angioedema: A Guide
Hereditary Angioedema (HAE) is a rare genetic disorder that affects an estimated 1 in 50,000-150,000 people worldwide. This condition can cause recurrent attacks involving painful swelling of the skin and mucous membranes.
Living with HAE doesn't have to mean a life of constant discomfort. Understanding the factors that trigger HAE attacks can help individuals better manage their condition. Common triggers include stress, infections, and trauma. Making lifestyle changes, such as keeping a journal of attacks, adjusting the diet, managing stress, prioritizing sleep, and taking care to avoid strenuous exercise, can help people manage HAE triggers.
When it comes to treatment, a combination of lifestyle modifications and pharmacologic options is often employed. Acute attacks are treated primarily with C1 esterase inhibitor (C1-INH) replacement therapy. Other medications used for acute management include bradykinin receptor antagonists and kallikrein inhibitors.
For long-term prevention, prophylactic treatments include regular C1-INH infusions, androgens, and newer oral prophylactic agents like berotralstat. Berotralstat is the only prophylactic option that can be taken orally. In cases where first-line treatments are unavailable, fresh frozen plasma (FFP) can be used, but it is less preferred due to infection risk and limited availability.
Emotional stress, anxiety, and low mood can also trigger HAE attacks in some individuals. Speaking with a mental health professional can be beneficial for those experiencing symptoms of anxiety or depression.
People with HAE can live a close-to-normal life with treatment. 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.
The COVID-19 pandemic has been linked to an increase in HAE attacks in children due to anxiety, depression, stress, and fear of the virus.
Online or in-person support groups, such as DiscoverHAE on Facebook and the HAEA Cafe, can help people with HAE feel more connected and less alone. The HAEA also runs a youth program for children and adolescents living with HAE.
In cases of 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.
Planning can help reduce the risks of traveling with HAE. This includes ensuring enough medication, carrying medication in an accessible carry-on bag, requesting a doctor's letter for carrying medication on a flight, asking for a written prescription, recording phone numbers for doctors and emergency contacts, and knowing the location of a local hospital.
People with HAE who are working may find it beneficial to talk with human resources 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.
In summary, managing HAE involves a combination of lifestyle modifications to avoid triggers and a spectrum of pharmacologic options including C1-INH replacement, bradykinin pathway inhibitors, and prophylactic agents tailored to attack frequency and severity. With the right approach, people with HAE can lead fulfilling lives.
- It's essential for individuals with HAE to acknowledge that understanding the 'triggers' such as stress, infections, and trauma can aid significantly in managing their condition.
- For people living with HAE, seeking assistance from mental health professionals may help alleviate emotional stress, anxiety, and low mood that could potentially trigger attacks.
- People with HAE should ensure they are prepared for travel by planning ahead, such as carrying enough medication, obtaining a doctor's letter, and knowing the location of a local hospital in case of an emergency.
- In cases where the medication is unavailable or inappropriate during pregnancy, healthcare providers closely monitor the health of both the individual and developing baby to determine the most suitable treatment.
