Hereditary angioedema (HAE) is a rare genetic disorder that causes episodes of swelling in various parts of the body. Though there is no known cure, those living with HAE can manage their condition through lifestyle changes such as implementing an individualized diet, as well as creating an active treatment plan. While managing symptoms through diet may seem daunting, the potential benefits make it worth exploring. They can help reduce or prevent flare-ups while also allowing them to live an active life.
Understanding Hereditary Angioedema and Its Impact
Hereditary Angioedema (HAE) is a rare and potentially life-threatening genetic disorder. Individuals with this disorder have a lower-than-normal level of proteins in the blood, leading to symptoms often mistaken for an allergic reaction. It presents through unpredictable, recurrent episodes of swelling. These angioedema attacks affect various body parts, including the face, extremities, and even the airways. Acute episodes often emerge without clear triggers and lead to significant discomfort.
The patient can experience discomfort and may have difficulty breathing during these episodes. Unlike other types of angioedema like allergic angioedema, which is often related to an allergic reaction, HAE is caused by a flaw in the immune system. Thus, the occurrence and severity of symptoms can vary greatly from person to person, depending heavily on their physical health and genetic predisposition.
HAE severely affects patients’ quality of life due to its nature of recurrent angioedema. Unpredictable bouts result in increased anxiety around the potential for sudden, severe attacks. Angioedema episodes often require hospitalization, thus influencing patients’ socioeconomic status. The impact extends to mental health, with many patients reporting significant psychological distress.
Interestingly, angioedema attacks in patients tend to become less severe with age. Yet, acute episodes in the elderly may increase the risk of fatal outcomes due to associated comorbidities. The unpredictable nature and severe consequences of attacks demand effective preventative measures.
Here is a list of life aspects impacted by hereditary angioedema:
- Physical health – frequent unpredictable swelling.
- Mental health – anxiety, stress.
- Socio-economic aspects – potential hospitalization costs, missed work.
- Personal life – fear of public appearance, socialization issues.
Causes of HAE
The Role of Inhibitors in HAE
Hereditary Angioedema is a genetic disease characterized by recurrent episodes of severe swelling. Central to this condition is the deficiency of the C1 inhibitor, a protein that controls inflammation. Deficiencies in thisinhibitor, result in excessive inflammation, largely contributing to the symptoms of HAE.
The management of HAE involves replacing the deficient C1 inhibitor with a plasma-derived C1 inhibitor. This is derived from the plasma of healthy donors and has been demonstrated for its efficacy in treating acute swelling attacks.
Angiotensin-converting enzyme inhibitors can potentially induce acute episodes of swelling in patients with Hereditary Angioedema due to their role in regulating inflammation. Accurate diagnosis of hereditary C1 inhibitor deficiency is essential for the safe and effective management of Hereditary Angioedema. Physicians must be made aware of the contraindications of this inhibitor in these patients.
Production of Bradykinin
HAE is a unique and severe condition, often linked to the overproduction of bradykinin. As a potent inflammatory mediator, bradykinin stimulates the sensation of pain and dilation of blood vessels. In the context of HAE, these inflammatory responses can lead to painful, dangerous swelling.
Overproduction of bradykinin in individuals with HAE is typically the result of a gene mutation. This can cause a series of sudden, unpredictable swelling episodes affecting various body parts. While these can be mild, such episodes can also be life-threatening when occurring in the airways.
In the field of HAE clinical research, the use of bradykinin B2 receptor antagonist agents has shown promising results. As illustrated in various studies, such antagonists act by blocking the activation of bradykinin receptors. This can help control the exaggerated inflammatory responses that characterize HAE.
Potential Triggers of a HAE Attack
Common triggers of an acute attack of HAE include
- Physical or emotional stress
- Dental procedures
- Trauma, infections, and medical procedures
- Insect bites
- Environmental factors such as changes in temperature
Symptoms of HAE
Excessive swelling is one of the primary symptoms of hereditary angioedema (HAE). This disorder causes episodes of recurrent and severe swelling in various parts of the body. In particular, laryngeal edema—or swelling in the throat—is a serious concern in HAE patients. This can lead to difficulty breathing, which could be potentially life-threatening without proper medical care. Controlling excessive inflammation is essential for managing HAE, as it reduces the risk of sudden airway obstruction.
Gastrointestinal Tract Symptoms
Hereditary angioedema often manifests as gastrointestinal tract issues and abdominal attacks. It can provoke swelling of the intestinal wall. A common symptom that patients frequently experience during an episode is abdominal or stomach pain. Moreover, swift response is crucial as untreated abdominal attacks can lead to complications. Acute treatment is the key to minimizing the severity and duration of attacks. In particular, attention to the gastrointestinal tract should be prioritized, as it’s commonly implicated in hereditary angioedema.
Other Potential Symptoms
In addition to the common symptoms of hereditary angioedema, patients may also experience other signs and symptoms. These include facial swelling, hoarseness in the voice, chest pain, difficulty breathing or swallowing, nausea and vomiting, itching skin rashes, hives, or welts. Skin discoloration may also occur as an episode progresses. Patients may also experience a decrease in urine output, wheezing, and fatigue.
When it comes to treating Hereditary Angioedema, acute therapy, and preventive treatment options are essential for managing symptoms and reducing the risk of dangerous episodes that can cause hypersensitivity reactions and thrombotic events.
The Importance of Diet in HAE
Certain food choices may worsen the symptoms of Hereditary Angioedema and lead to adverse reactions as well and others can help to live with HAE.
Avoiding Trigger Foods
When it comes to hereditary angioedema (HAE) attacks, many people automatically assume that these attacks are triggered by food allergies. However, a 2018 study published in the World Allergy Organization Journal about abdominal angioedema attacks, found something else. This study showed that allergic reactions were not the cause of symptoms resulting from trigger foods for those with HAE.
The research team determined that certain foods may still be more likely than others to spur HAE attacks due to their high levels of histamine. These include fish, shellfish, certain fruits and vegetables, processed meats, pickled foods, and fermented beverages such as wine or beer. Skin prick tests did not confirm allergies for any of these foods so this indicates that the inflammatory processes causing the episodes are not mediated by immunoglobulin E.
Food that can trigger HAE symptoms according to the study:
- Citrus fruits: kiwi, pineapple, and strawberries
- Dairy products: milk and cheese
- Seafood: fish and shellfish
- Other: chili, onion and garlic
Suspected foods to cause swelling:
- Alcohol: wine or beer
- Fruits: tomato, banana and kiwi
- Tree nuts
- Green salad
It should be noted that not all sufferers experienced an attack following any of these foods; however, the ones who did had similar experiences. If someone is prone to HAE episodes then it might be best to avoid consuming common food to reduce chances of triggering an attack. The healthcare provider might also advise them to keep a detailed log of their diet and symptom occurrence to keep track of what causes chronic or acute episodes to make better-informed decisions about dietary modifications.
Consuming a Healthy Diet
Eating a balanced and nutritious diet is one of the most important aspects of living a healthy lifestyle. For patients with HAE, a diet that is high in whole, unprocessed foods is recommended to reduce the risk of a flare-up. This means avoiding processed food products and anything containing flavor enhancers, additives, preservatives, or food coloring.
Medical experts suggest following a heart-healthy diet due to the link between HAE and a higher likelihood of heart disease. The Mediterranean diet is linked with a reduced risk of heart disease. This diet includes:
- Healthy fats from sources such as fish, olive oil, avocado, nuts, and seeds
- Whole grains
- Beans and lentils
- Nuts and seeds
- Fruits and vegetables
- Moderate amounts of dairy products
- Lean protein
Nutritionists can help patients with HAE develop an individualized dietary plan that takes into account their personal needs, including any potential food triggers they may have. Professional guidance can also help when following an elimination diet so different foods can be tested and understood before being reintroduced into regular eating habits.
Hydration is another focus of these guidelines. Drinking an ample amount of water every day is stressful. Adequate water intake assists in flushing out toxins and reducing the chances of potential attacks. In addition, alcohol should be avoided as could potentially disrupt the balance of C1-INH protein in the body.
Supplements for treating HAE
HAE is an inherited disorder affecting blood vessel function and requires personalized treatment to manage symptoms. Although there may be some potential benefits of supplement use for managing HAE, very few studies exist that look at the impact of supplements on patients specifically. Depending on the circumstances, a healthcare professional may recommend a multivitamin and mineral supplement to those with HAE episodes that reduce their food intake. Regular blood tests should also be performed to evaluate if any nutrient deficiencies or food allergens are present.
Research has looked into the possible roles of omega-3s and vitamin D in helping to reduce inflammation in individuals affected by HAE, but more research is still needed before definitive conclusions can be made regarding these nutrients’ effects on reducing symptom severity or frequency. Furthermore, not all individuals respond the same way to nutritional interventions, each person needs to consultsult with their doctor before starting supplementation. It is also recommended that any supplements taken are quality-controlled and GMP-certified to ensure the safety and efficacy of the products used.
Other Lifestyle Measures for Treating HAE
The guidelines released by the Angioedema Association provide necessary medical advice to those grappling with the condition. The association firmly believes that a correct diet alongside a positive lifestyle can help mitigate angioedema symptoms.
Physical activities are encouraged. A routine that compliments one’s health status is often deemed best. An important note is to consult with a physician before beginning any new training program. The guidelines also prioritize mental health. They suggest practicing relaxation exercises such as yoga, and meditation and seeking medical advice from therapists if needed.
Adopting these guidelines can significantly improve the quality of life for those dealing with angioedema. Monitoring progress, coupled with the apt treatment, can help control and manage this chronic condition, reducing its impact on everyday life.
Consult with a specialist
For individuals with HAE, it is important to consult with a specialist to develop an individualized treatment plan that works best for them. At Becker ENT and Allergy, our team of experienced healthcare professionals can provide personalized advice and guidance on managing episodes of angioedema, as well as nutrition and lifestyle tips to optimize overall health. We are dedicated to providing superior care and will work together with you to create a tailored approach that fits your needs. Schedule an appointment today to learn more about how we can help manage your symptoms!