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Beyond Swelling: Living With Hereditary Angioedema (HAE) Means Living With Stress and Uncertainty Between Attacks

A rare condition, HAE, causes unpredictable swelling attacks that can be life-threatening. Day-to-day, patients live with the ever-present worry: When will the next attack strike?

Illustration of someone choosing between a straight path and a swirling one

Swelling caused by hereditary angioedema (HAE) attacks can be unpredictable, painful, debilitating and even life-threatening when they affect breathing or other vital body functions. Many patients report severe attacks and half say their attacks last two or more days, according to an online survey of 100 people living with HAE conducted by Harris Poll and sponsored by CSL Behring. Patients miss work and miss out on a lot with 61% saying the condition made them cancel social events.

It’s not surprising then that 76% of respondents said they worry about HAE attacks on a daily basis, and 92% agreed that HAE attacks put a tremendous stress on their lives.

“We know that hereditary angioedema significantly impacts a patient’s quality of life,” said Dr. Timothy Craig, who treats HAE patients and is a tenured Professor of Medicine, Pediatrics and Biomedical Sciences at Penn State University. “They experience a high burden of disease from the attacks, but also between attacks.”

Craig is a clinical researcher with more than 300 publications to his name, who has worked with CSL on HAE research for years. As the principal investigator on a prospective new prophylactic HAE treatment being researched by CSL, he authored a 2022 article and a 2023 article in the Lancet medical journal about the treatment and its potential. CSL Behring, a business unit of CSL, makes medicines for rare diseases, including HAE.

Prophylactic treatments aim to stop attacks before they start. But even with existing prophylactic treatments, patients can still experience attacks. In the survey, nearly all patients and doctors agree efficacy means freedom from HAE attacks. There are quite a few treatment options, but there’s room for more that work toward that goal, Craig said.

Researchers, including Craig and CSL scientists, are actively looking for innovative treatments. Approved prophylactic treatments for HAE aim to compensate for a deficiency in C1-esterase inhibitor (C1-INH) or inhibit the release of a peptide called bradykinin (BK), both of which can lead to swelling attacks. But there’s another potential target for stopping HAE attacks that had not been explored until recently: activated Factor XII (FXIIa).

Scientists at CSL are currently studying how targeting FXIIa could assist in reducing HAE attacks. FXIIa is a plasma protein that initiates a chain of events that causes swelling. FXIIa comes into play earlier in the chain of events that lead to an HAE attack.

The potential treatment is currently being reviewed by the U.S. Food and Drug Administration (FDA) as a potential prophylactic treatment for HAE. The agency is expected to make a decision later this year. Additionally, an open label extension of the study continues, allowing more scientific data to be collected around the safety and efficacy of the treatment.

While the research continues, here’s what doctors and patients can do today:

For health care providers:

  • Ask patients about their attacks: Are they experiencing any? How often? How intense? How are attacks affecting them?
  • Encourage patients to track all attacks and their impacts on daily life – even those attacks that are less severe.
  • Ask patients if they have freedom from attacks with their current treatment or are they living life feeling worried about future attacks?
  • Explore the latest research to identify the best treatment option for the patient.

For people living with HAE:

  • Before your next appointment, take a moment to reflect on your recent attack history and how it has impacted your life. Keep track of all attacks, even those that are less severe: How many attacks did you experience and how often? How intense were they? Did you miss school or work or an important event? Do you feel stress or worry?
  • Discuss your recent attack history with your doctor and health care team to show how attacks impact your health and your quality of life.

It’s always important to be informed; find out more about HAE.