HAE is caused by a defect in the gene that controls a protein known as C1 inhibitor. C1 inhibitor helps regulate certain complex reactions in the body, including reactions that lead to the production of bradykinin. Bradykinin is a chemical that causes blood vessels to dilate (widen) and leak fluid into surrounding tissue. It is this leakage of fluid that is thought to cause the swelling and painful attacks associated with HAE.
In people with HAE, the body either does not produce enough C1 inhibitor or produces C1 inhibitor that does not function properly. As a result, too much bradykinin is produced, blood vessels leak fluid, and swelling and painful attacks can occur.
Berinert is a C1 esterase inhibitor. It works by replacing C1 inhibitor that is missing or not working properly in people with HAE.
Berinert is approved for use in children as young as 13 years old. It has not been adequately studied in children younger than age 13 or in infants. Talk to your child's healthcare provider about the risks and benefits of using this medication in children.
Yes -- older adults can use this medicine. However, there were not enough older adults in clinical trials to determine if they would respond to or tolerate Berinert any differently than younger individuals. Therefore, older adults may need to be monitored closely until more information is available about the use of Berinert in this age group.
On occasion, your healthcare provider may recommend this medication for treating something other than the conditions discussed in this article. This is called an "off-label" use. Using Berinert to prevent HAE attacks would be an example of an off-label use.