An Overview of Uses for C1 Esterase Inhibitor
C1 esterase inhibitor
) is a protein found naturally in the blood. One of its jobs is to regulate certain reactions in the body that are involved in inflammation and swelling. C1 esterase inhibitor is also used to make certain medications that are approved for the treatment of hereditary angioedema (HAE). These medicines are derived from human blood and are, therefore, considered "biologic" products.
Hereditary angioedema is a rare genetic condition associated with a deficiency of properly functioning C1 esterase inhibitor. People with this condition experience sudden attacks of angioedema (swelling of the deep layers of the skin). The swelling episodes can occur in any area of the body, but most commonly affect the hands, feet, face, genitals, upper airways, larynx (voice box), and abdomen (stomach). Attacks usually only affect one area of the body at a time.
The frequency of HAE attacks can vary considerably from person to person, and can even vary amongst people in the same family. Some people may experience weekly attacks, while others may go years without having one. When attacks do occur, the type and severity of symptoms can also vary.
Although attacks will usually resolve on their own, the typical attack can last from two to five days. During that time, a person can experience significant symptoms. For example, abdominal (stomach) attacks may cause considerable pain, nausea, vomiting, and diarrhea
. Some attacks can be life-threatening. Swelling of the throat (called laryngeal attacks) can progress to the point where a person is unable to breathe and, if left untreated, could cause death.
C1 esterase inhibitor medications are one type of medicine used to treat people with HAE. This type of medicine includes the brand-name products Berinert and Cinryze.
Berinert is approved to treat attacks of swelling in people with HAE. It is used at the first sign of an attack, and helps to relieve symptoms and prevent the attack from progressing. Cinryze is approved for routine prophylaxis against HAE attacks, which means it is used regularly (usually every three to four days) to prevent the attacks from occurring.