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New Treatment For Hereditary Angioedema
The U.S. Food and Drug Administration has approved Cinryze [C1 Inhibitor (Human)], intended to protect people with hereditary angioedema. HAE is a rare and potentially life-threatening genetic disease that produces rapid attacks with swelling of the hands, feet, limbs, face, intestinal tract, or airway. Swelling of the larynx can be severe and lead to asphyxiation. Patients with HAE are more likely to experience attacks during stress, surgery, or infection, making Cinryze therapy an important prophylactic treatment during emergency and inpatient hospital visits.
Cinryze regulates clotting and inflammatory reactions and stops tissue swelling. It is administered intravenously as a 1,000 unit dosage and can be used every three or four days for the routine prevention of HAE attacks. The drug is supplied in powdered form and must be protected from light until reconstituted with sterile water. It is administered at room temperature within three hours of reconstitution.
Adverse reactions: Commonly seen side effects include upper respiratory tract infection, sinusitis, rash, and headache. More serious reactions include hypersensitivity reactions and thrombotic events. Because Cinryze is derived from human plasma, it may contain infectious agents, including viruses.
Patient monitoring: Assess for signs of venous thrombosis, stroke, and chest pain. Epinephrine should be immediately available to treat acute hypersensitivity reactions.
Patient teaching: Review the signs and symptoms of thrombosis, including new onset of limb or abdomen swelling, chest pain, shortness of breath, loss of sensation or motor power, or altered consciousness or speech.
Drug News is compiled by Susanne J. Pavlovich-Danis, RN, MSN, ARNP-C, CDE, CRRN, who maintains a private practice in Plantation, Fla., and is professor and area chair for nursing at the University of Phoenix, Fort Lauderdale.