Who’s Who on Your Hereditary Angioedema (HAE) Treatment Team

Living with hereditary angioedema (HAE) means navigating a rare condition that causes painful swelling in various parts of the body, often including the face, hands, feet, genitals, abdomen, and airways. Attacks can be unpredictable and sometimes serious, making day-to-day life feel uncertain and stressful.

Because HAE affects everyone differently — how often attacks occur, how severe they are, and how they disrupt daily routines — managing it requires a personalized plan.

An immunologist or allergist is typically the “point person” who takes the lead role in diagnosing and treating HAE, but often, other specialists and healthcare professionals are involved in treating some of the symptoms, coexisting conditions, or other medical needs throughout your life.

Together, your HAE care team will work to help you stay prepared, protected, and in control. Here’s a who’s who of key members of your healthcare team and how each one helps support your health and quality of life.

Most often, an allergist-immunologist is the medical professional who diagnoses and treats HAE.

“Your allergist or immunologist is the primary specialist who does the diagnosis; they figure out what’s causing your symptoms,” says Rita Kachru, MD, an immunologist at UCLA Health in Santa Monica.

That process involves figuring out if your symptoms are related to histamine — meaning they’re caused by an allergic reaction — or to bradykinin, which is what causes the swelling in HAE, says Dr. Kachru.

An immunologist or allergist understands how your immune and inflammatory systems work, and how they can go awry in conditions like HAE, where in most cases, a missing or malfunctioning C1-inhibitor protein leads to swelling.

Your allergist-immunologist will:

• Confirm your diagnosis through blood tests that measure C4 and C1-inhibitor levels and function
• Prescribe and monitor your HAE treatments, which may include C1-inhibitor replacement therapy, bradykinin inhibitors like icatibant (Firazyr) or ecallantide (Kalbitor), and preventive medications such as lanadelumab (Takhzyro) or berotralstat (Orladeyo)
• Teach you when and how to self-administer your medication, so you can treat attacks quickly
• Develop a written treatment and emergency plan you can share with your primary-care provider or emergency department staff

Because HAE is rare, the allergist-immunologists who treat it are often based at academic or large medical centers, where they can stay up to date on new treatments and research.

Your primary care provider (PCP) plays an important role in your overall health and in coordinating care among any specialists you may need to see.

A primary care provider (PCP) is often your first stop when you (or your parents) notice any new or worsening symptoms. These doctors, trained in family medicine, internal medicine, or pediatrics, serve as your main point of contact with the healthcare system.

The next steps may depend on where you live and your provider’s experience in diagnosing and managing HAE. They may refer you to an allergist or immunologist if they suspect HAE, but because the symptoms aren’t always straightforward, they may refer you to another type of specialist.

Your primary care provider can also:

• Manage other health conditions that could worsen HAE or complicate treatment, such as high blood pressure or cardiovascular disease
• Ensure that all medications prescribed are compatible with your HAE therapy
• Reinforce your emergency plan and help educate local healthcare teams if you ever need urgent care

Swelling in the intestinal tract is a common and often painful symptom of HAE. Gastroenterologists are experts in diagnosing and managing these abdominal attacks, which can mimic other conditions like appendicitis or bowel obstruction.

If you have these symptoms, you may see a gastroenterologist during your diagnostic journey. However, if it’s already been established that HAE is causing your GI symptoms, it’s likely that your immunologist would help you manage these symptoms through medications and lifestyle habits, says Kachru.

There may also be situations in which your immunologist would coordinate with a gastroenterologist, she says. “For example, if a person with HAE was going in for a colonoscopy, we would discuss what precautions need to be taken before the procedure to minimize the risk of an attack. We would also talk with the anesthesiologist,” says Kachru.

Skin swelling — often on the face, hands, feet, or genitals — are common symptoms of HAE. Because that appears on the skin, sometimes people with HAE who haven’t been diagnosed may seek care with a dermatologist.

While there are certain conditions like atopic dermatitis or chronic urticaria (hives) that have some similarities and might be treated by an allergist or a dermatologist, that’s typically not the case with HAE, says Kachru.

“Most dermatologists don’t manage HAE; they would refer the patient to an allergist-immunologist,” she says.

Many women find that HAE attacks become more frequent or severe during puberty, pregnancy, or menopause.

“Because we know that hormones play a very big role in potentially driving these attacks or episodes, we work very closely with a woman’s ob-gyn,” says Kachru.

Your ob-gyn can:

• Help you choose safe birth control options. Estrogen-containing pills, patches, and rings are usually not recommended because they can trigger attacks. Progestin-only or nonhormonal methods are typically safer choices.
• Coordinate with your allergist-immunologist before, during, and after pregnancy to ensure that both you and your baby are protected.
• Develop a birth and postpartum plan that ensures medication is available in the hospital and that staff know how to respond to an HAE attack if one occurs.
• Support you through menopause, helping manage hot flashes and other symptoms with non-estrogen alternatives when possible.

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Living with a rare disease like HAE can be emotionally taxing. Anxiety about attacks, particularly those involving the airway, and the unpredictability of symptoms can take a toll on mental health. It can also trigger stress, which can contribute to attacks.

“I think it’s important to have a therapist or other mental health professional to be able to talk about how HAE is interfering with your life and what you can do about that,” says Dr. Craig.

Kachru agrees that adding a therapist to the healthcare team can help people have a better understanding of how to manage their stressors.

In addition to teaching skills to cope with stress, a psychologist, counselor, or social worker familiar with HAE can:

• Help you find ways to minimize the impact that HAE has on your life
• Offer support for depression or trauma that can develop after severe or frightening attacks
• Help family members understand how to provide practical and emotional support

Because HAE is rare, finding a doctor with real experience may take some effort, but it can make all the difference. Building a team that understands HAE and how it can touch every part of your health starts with a knowledgeable “point person” who can help you manage symptoms, prevent attacks, and connect you with the specialists you need.

To locate HAE specialists:

• Use the U.S. Hereditary Angioedema Association’s Physician Locator Tool. This connects you to providers experienced in diagnosing and treating HAE. You can find it here: HAEA.org Physician Locator.
• Contact a major academic or teaching hospital in your area. Large medical centers often have immunology, allergy, or rare disease clinics familiar with HAE.

• Managing hereditary angioedema (HAE) requires a team approach that’s led by your allergist or immunologist.
• Your primary care provider will be your source for your general health and acute non-HAE related issues, and specialists like ob-gyns and mental health professionals also play key roles.
• Because symptoms and triggers vary widely, having a personalized care plan and a healthcare team can help you prevent attacks and manage them quickly when they occur.
• The U.S. Hereditary Angioedema Association’s Physician Locator Tool (HAEA.org) can help you find specialists experienced in treating this rare condition.