Hereditary angioedema (HAE) is a congenital disorder involving severe swelling, typically on the face, intestinal tract, limbs, and air passageways. Intestinal tract swelling may cause extreme abdominal pain, vomiting, and nausea. Since this condition is hereditary, it may occur without any apparent trigger. HAE symptoms may show up in childhood and aggravate during adolescence. Individuals not under treatment for the condition experience its relapse every 1-2 weeks. Here’s everything to know about HAE:

Cause of hereditary angioedema
Hereditary angioedema, or HAE, is caused by low levels or inadequate functioning of the C1 inhibitor, an essential anti-inflammatory protein. This deficiency of the C1 inhibitor causes the muscles in the body to swell up. Genetic studies have shown that every child of an individual grappling with HAE has a 50% probability of inheriting the condition. However, some persons without a family history of HAE may also develop the disease. About 25% of patients with the condition develop it due to de novo genetic mutations.

Signs and symptoms of hereditary angioedema

Swelling
Swelling may occur in different body parts, such as the mouth, throat, hands, feet, face, lung airways, intestinal tract, and genitals. Swelling in the throat is among the riskiest symptoms of this condition.

Rashes
About one-third of individuals with HAE develop rashes called erythema marginatum. These rashes are usually non-itchy.

Prickling sensation
Persons with HAE may experience a prickly sensation in the affected areas before swelling develops. One should consult a doctor to begin early treatment and prevent the condition from escalating.

Voice hoarseness
Swelling of the throat and air passageways may cause voice hoarseness, especially just before an HAE episode.

Extreme fatigue
Some patients with HAE may experience extreme tiredness and fatigue before the onset of an HAE episode.

Treatment options and remedies
Doctors may typically prescribe oral interventions to treat HAE. In addition, certain remedies and lifestyle tips can help manage its symptoms better:

Identify food triggers
Certain foods or food groups may increase one’s susceptibility to HAE. As per a study, some suspected food triggers include tomatoes, bread, kiwis, strawberries, dairy, cheese, citrus fruits, and garlic. Hence, one should be attuned to the body’s reaction to such foods and avoid them actively to control the frequency of HAE episodes.

Avoid spicy and processed foods
Spicy and processed foods can irritate the stomach’s lining and aggravate abdominal pain among patients suffering from intestinal tract swelling associated with HAE. Thus, it is advisable to avoid foods like confectionaries, fried foods, bacon, hotdogs, etc., to the best extent possible.

Identify and eliminate stressors
Some studies have shown that mental distress and trauma may contribute to HAE flare-ups and worsening symptoms. Thus, patients with this condition should try to identify and eliminate stressors. For example, if work is a significant stressor, one should consider switching to a part-time role or changing one’s job to ensure better peace of mind. In contrast, if household chores take up one’s time and effort, delegating these tasks to professionals and seeking assistance from family members and friends to heal better is advisable.

Maintain a nutrient-rich meal plan
A meal plan rich in all essential nutrients, including carbohydrates, healthy fats, proteins, vitamins, and minerals, can help control the symptoms of HAE and provide the body with sufficient immunity to deal with the condition. Therefore, one should have healthy foods like lean meats, eggs, whole grains, fruits, and vegetables (eliminating trigger foods) to accelerate the healing process.

Download a symptom-tracking app or maintain a journal
Many patients with HAE have found it beneficial to maintain a journal to track their symptoms and treatment progress. These days, apps that allow one to monitor their HAE symptoms, treatment courses, and improvements have made the process easier. Thus, one may maintain a journal or download one such app to monitor one’s progress while getting treated for HAE.

Stay active
While intense physical activity may trigger or worsen HAE symptoms, a sedentary lifestyle can inhibit progress. Thus, it is important to avoid being sedentary, including walking, stretching, and other low-intensity activities in one’s routine while avoiding strenuous ones.

Join a support group
Today, there are several support groups available for patients with HAE. Interacting with others grappling with the same condition can provide a sense of support and empathy, helping with recovery. One may look up patient organizations or discussion boards online to gain support from other patients with this condition.

Communicate with the doctor
Dealing with HAE episodes may be challenging and distressing; addressing its symptoms promptly can help reduce its effects. Therefore, patients with HAE should speak with their doctors openly about any new developments, worsening of conditions, improvements, triggers, and treatment side effects. It is also important to consult a doctor who understands this condition thoroughly and has proven experience helping patients with HAE.

Learning as much as possible about HAE is essential to make informed decisions about the treatment plan. Ensure the caregivers and close ones know about the condition and have a plan of action ready in an emergency.