Next steps after an HAE diagnosis

Last updated Oct. 2, 2023, by Susie Strachan
Fact-checked by Patricia Silva, PhD

A new diagnosis can be confusing and difficult to comprehend. It may bring on feelings of grief, along with denial, bargaining, anger, and sadness. And facing a disease like hereditary angioedema (HAE) — whether for you or your child — can unleash a flood of questions, including how it’s caused, triggered, and treated.

Work closely with your healthcare provider on developing a comprehensive treatment plan. Set up regular checkups for monitoring and addressing swelling episodes. Your doctor can help you find groups with people living with HAE and their caregivers who understand your situation and can provide valuable support.

To feel more knowledgeable about your diagnosis and how it can affect your life, educate yourself about HAE. Put together a support team, review your finances and health insurance, and look into other available resources, including support groups.

Accepting your diagnosis

HAE is diagnosed after a doctor has examined the affected area or areas, reviews your medical and family history, and does tests to determine the type of angioedema.

Coming to terms with an HAE diagnosis head-on is the best way to adjust to your new reality. You can jump-start this process by making a list of questions and discussing them with your healthcare provider.

The disease can seem unpredictable. HAE swelling can occur anywhere on your body, although the lips, hands, feet, and the skin around your eyes are the most common areas. Swelling attacks may happen regularly, often without an obvious trigger.

The uncertainty of when you may experience symptoms can affect your mental health. You may find yourself dealing with some complex emotions. Talking with family and friends about all the decisions you need to make can ease your anxiety and help you come to terms with your new diagnosis.

Understanding how to learn to live with HAE also may make you feel more in control of your health and help with stress management.

Educating yourself

Learning about HAE can give you insight into what causes the disease, what sets off attacks, what to avoid, and how to treat HAE symptoms.

HAE is caused by genetic abnormalities that lead to a deficiency in the levels of bradykinin, a protein that regulates blood pressure and inflammation. HAE is a chronic disease characterized by sudden but temporary swelling in the deeper layers of the skin, although people can experience a range of symptoms. Swelling usually appears on its own, without hives or a rash.

There are three types of hereditary angioedema. Types 1 and 2 are caused by mutations in the SERPING1 gene. Type 3 is caused by mutations in the F12 gene. The most common form of the disorder is HAE type 1.

Attacks may occur without warning. They can be painful and disfiguring, and last for hours or days.

The disease affects men and women equally. But women may be more likely to have attacks — including when pregnant — possibly because of higher levels of estrogen.

Reading published work from researchers investigating HAE and learning about experimental treatments aimed at slowing down and eventually curing the disease may offer hope.

Angioedema News has the latest news and research about HAE, resource pages about the disease, along with columns about life with HAE written by people who have the condition.

Avoiding triggers

HAE attacks are caused by an overproduction of a protein called bradykinin, which increases blood vessel leakiness, resulting in swelling. This happens when bradykinin is activated in the deep layers of the skin, allowing fluid to flow from the bloodstream into nearby tissues.

While it can be difficult to pinpoint what sets off some attacks, there are certain HAE triggers you may be able to avoid or manage. These can include trauma, viral infections, stress, and medical or dental procedures. Other triggers are allergens, hormonal fluctuations, and repetitive physical activities.

It can be useful to write in a journal or try a tracking app to keep a record of your symptoms. Discuss symptoms with your healthcare provider, who should be able to keep you up to date about options for approved treatments to prevent attacks and manage swelling.

Dealing with an HAE attack

Common HAE symptoms include recurring skin swellings, abdominal symptoms from gastrointestinal angioedema, and airway symptoms — such as difficulty breathing or a choking sensation — due to neck and throat swelling.

Your healthcare provider should discuss with you how to handle an HAE attack, including how to administer treatment at home. You may also want to consider preventive treatments that can be taken before events that may trigger attacks, such as medical surgery or certain dental procedures like having a tooth pulled.

If your symptoms cause your throat to swell and you have difficulty breathing, this may become life-threatening. Seek emergency healthcare immediately.

HAE-related swelling is not the same as swelling from an allergy and should not be treated as an allergic reaction.

Deciding on a treatment approach

After being diagnosed with HAE, your healthcare team should help you map out a treatment approach.

Approved treatments for HAE include on-demand treatments for an attack, short-term preventive treatments for situations that have the potential to set off an attack, and long-term preventive treatments that can reduce the frequency and severity of attacks.

A treatment approach should be based on your overall health and fit with your personal preferences. It may include lifestyle modifications to help you manage your disease.

Putting together a care team

Your HAE care team will start with your medical team, but it may expand to include family, friends, and professional caregivers.

HAE is typically managed by allergy and immunology specialists. You also may see a pulmonologist, who treats respiratory issues, and a primary care provider or nurse practitioner to help you manage your symptoms.

Along with a healthcare team, you may want to involve others in your healthcare. For example, family members and friends can support you by picking up prescriptions, cleaning your home and cooking, and keep you motivated when you’re feeling overwhelmed.

Make sure your family knows which HAE symptoms could be a medical emergency, and that they know what to do if it ever seems life-threatening.

Caring for children with HAE

As attacks can occur at school, a parent or family caregiver may have to suddenly go to the school if their child has an attack. To ease the burden of unexpected trips to school, find a trusted person as a backup and add them to your child’s school’s pickup list.

You also may want to work with the caregivers at places like day cares, schools, clubs, and summer camps on a plan to ensure your child’s safety. It’s suggested that you provide:

• a written description of HAE and its treatment for when attacks occur
• a written authorization for administering medication and emergency treatment, signed by your child’s personal healthcare provider
• a supply of medication which you should refill as needed.

Finances and insurance

The cost of treating HAE can place a heavy financial burden on you and your family, and even affect your quality of life.

It will depend on many factors, including the type and frequency of the HAE medication, whether you need to be treated by specialists, and if you need to go to the emergency room or be cared for in a hospital.

In the U.S., financial assistance and health insurance is available at the federal level, through private or employer-based insurance programs, and some HAE medication manufacturers.

At the federal level, Medicare coverage for all forms of angioedema includes the following:

• Part A may cover part of the cost of in-patient treatment at a hospital.
• Part B may cover the cost of an out-patient treatment, minus any applicable copay, coinsurance, or deductible amounts.
• Part D’s prescription drug plan or a Medicare Advantage plan can cover part of the cost of prescription medications.

Private and employer-based health insurance plans vary in their coverage of the cost of HAE medical care and medications. You may find yourself dealing with high insurance premiums or out-of-pocket essentials, so research insurance coverage options and financial assistance programs.

Your healthcare provider may be able to offer guidance about which insurance companies provide financial coverage for HAE treatments.

The U.S. Hereditary Angioedema Association’s health advocates can provide guidance on reimbursement for HAE medications. Contact them by phone at 866-798-5598.

Some HAE medication manufacturers may provide financial assistance for insurance premiums and copays.

Useful resources

Start by speaking with your personal healthcare provider when looking for patient resources for HAE education, community engagement, support services, and groups. They can direct you to local or online resources, as well as organizations that serve people with HAE and their caregivers. Your doctor also can direct you to therapy or counseling for mental health support.

HAE organizations often have online and in-person support groups. They also may offer HAE navigators and advocates who can guide you in making connections with HAE physicians and peers living with HAE who can share experiences and coping strategies.

Hereditary Angioedema International offers links to more than 90 countries around the world with HAE organizations that offer resources such as educational materials, links to care centers, hospitals, and available medication, along with personal support.

In North America, the U.S. Hereditary Angioedema Association and HAE Canada promote awareness, organize educational and fundraising events, and support clinical research into the disease.

Angioedema News publishes columns by people who have HAE. You may find relevant and reassuring insights into the struggles of daily life with HAE.

Advocacy

Once you are more familiar with what living with HAE entails, you may want to give back to the community.

Some people become advocates for HAE-related organizations, working to improve awareness of the disease, access to timely and affordable care, and pushing for increased funding research into HAE. Advocates also can engage healthcare professionals and politicians about improvements to public policies, such as better access to affordable treatments and insurance coverage.

Others might enjoy speaking at educational events, becoming mentors and sharing their lived experiences with those who were recently diagnosed with HAE, or participating in awareness and fundraising events.

Angioedema News is strictly a news and information website about the disease. It does not provide medical advice, diagnosis, or treatment. This content is not intended to be a substitute for professional medical advice, diagnosis, or treatment. Always seek the advice of your physician or other qualified health provider with any questions you may have regarding a medical condition. Never disregard professional medical advice or delay in seeking it because of something you have read on this website.