WHAT CAUSES HAE?

People with HAE have low levels of or a nonfunctioning protein called C1 esterase inhibitor (C1-INH).

The defect with C1-INH lies within a person’s genetic code, which is why HAE runs in families.

Without the right amounts of working C1-INH, blood vessels and capillaries in the body can become leaky and allow fluid to build up in the surrounding areas. This leads to the swelling experienced during an attack.

THE SYMPTOMS OF HAE

Swelling of the throat is a less common type of attack, but about 50% of patients have had one

70% to > 90% of patients experience abdominal pain caused by swelling of the gastrointestinal wall

Skin swelling most commonly affects the upper extremities vs lower extremities, followed by the face and genitals and, more rarely, the trunk and neck

Hereditary angiodema (HAE) is a rare inherited disease that affects about 1 in 50,000 to 1 in 100,000 people worldwide

The symptoms of HAE are often confused with other medical conditions like allergies or stomach problems, so it’s important to talk to your doctor about all your symptoms.

HAE usually causes swelling in specific parts of the body, though swelling can occur anywhere.

Women often experience HAE differently than men do

Women with HAE:

Are more likely to be symptomatic than men
Experience changes in the overall frequency of attacks as they go through various life stages (childhood, puberty, menstruation, pregnancies, and menopause)

Estrogen plays a role

Fluctuations in estrogen, a hormone, may trigger HAE attacks and affect the severity/frequency of the disease

HAE during pregnancy

Create an individualized HAE treatment plan for each pregnancy
Have an acute treatment available at the facility where you plan to deliver
Before taking any HAE therapies, consult with your doctor if you are pregnant or considering becoming pregnant

Pregnant Women

During pregnancies, 1 in 3 had more attacks, 1 in 3 stayed the same, and 1 in 3 had fewer attacks.

The World Allergy Organization recommends C1‑INH as a first line LTP treatment option in children with HAE and suggests first-line LTP with C1-INH in pregnant patients.

When a child has HAE...

Symptoms in children with HAE usually begin in the first or second decade of life.

Attacks are usually infrequent before puberty
Early onset of symptoms in childhood predict more severe disease in adulthood

Children who have 1 parent with HAE have a 50% chance of inheriting the disease

LIVING A FULL, ACTIVE LIFE WITH HAE

If you or a family member has HAE, you may be concerned about how it will affect your life. Many people find they can successfully manage the condition by working with their doctors to live healthy, active lives.

Having HAE means you need to take a few precautions, such as: