Hereditary Angioedema, or HAE, is an extremely rare genetic condition. Patients with HAE experience swelling under the skin in possibly many different parts of the body.
Individuals with HAE do not develop the condition, but rather they are born with it; treatment is only used to help manage symptoms.
With this being said, HAE symptoms often don’t show up in patients until childhood and progress during the teenage years. As a result, a lot of people don’t know that HAE is the source of their swelling until they are adults.
The biology behind this condition lies in a certain protein in the body that is off balance. In turn, the body signals small blood vessels to push fluid into nearby areas of the body. This eventually leads to the swelling that is seen in HAE individuals.
The location of swelling and magnitude of it depends on the individual, as do the frequency of bouts. Without proper treatment, people with HAE can have swelling attacks as often as every 1-2 weeks. These bouts can also be extremely difficult to manage.
The main cause of HAE is a dysfunction in a gene that controls a blood protein: the C1 inhibitor. Therefore, treatment options include C1 esterase inhibitors to treat bouts of swelling when they are already happening.
The treatment Berinert prides itself as “the only C1 esterase inhibitor approved for on-demand treatment of acute abdominal, facial, or laryngeal attacks of hereditary angioedema (HAE) in adults and pediatrics” via its website, http://www.berinert.com/. Berinert is given in the form of an injection into the veins.
For a while, it has been approved by the FDA in the United States, but now it has been approved in Canada too. Recently this past year, the biopharmaceutical company CSL Behring announced that Berinert was approved by Health Canada to treat pediatric HAE.
Of, course this is very exciting news for the HAE community, especially for those in Canada! The President of HAE Canada, Jacquie Badiou explains,
“The HAE Canada Team is so excited that CSL Behring listened to patients, caregivers, family members. This approval for Berinert in pediatric patients is a very important achievement for our children and parents living with HAE in Canada.”
HAE Canada is a patient advocacy group based in Ottawa, Ontario, that strives to help develop awareness about HAE as well as enable patients to live the best lives they can while struggling with the condition.
To learn more about this exciting approval for our neighbors to the north, read more in Business Insider here.