Generic Name:

C1 Inhibitor (Human)

Trade Name:

Cinryze

Action:

Unregulated bradykinin production is thought to contribute to the increased vascular permeability and angioedema observed in HAE. C1 inhibitor therapy is believed to suppress contact system activation via inactivation of plasma kallikrein and factor XIIa, thus preventing bradykinin production.

Indication:

• Use requires approval by an attending physician in the Allergy/Immunology Service
• Heart Failure Routine prophylaxis against angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE)
• Treatment of angioedema attacks in adolescent and adult patients with Hereditary Angioedema (HAE)
•  

Administrative Guidelines:

Usual dose and route:

•  Prophylaxis: 1000 units IV every 3 or 4 days

•  Treatment: 1000 units IV x 1 dose

Standard Concentration

•  1000 units/10mL (100 units/mL) 60mL Syringe

•  Administer over 30 minutes via sigma multi-doser

Critical Elements/Special Considerations:

•  Must be administered within 3 hours once mixed

•  MUST flush line with sterile water for injection, after infusion, to administer full dose

•  Made from human blood; counsel patients who are Jehovah's Witness

•  Requires dedicated line; do not administer with any other product

Precaution and Side Effects

•  Upper respiratory infection, sinusitis

•  Rash

•  Headache

•  Hypersensitivity reactions may occur. Epinephrine should be immediately available to treat any acute severe hypersensitivity reactions

•  Thrombotic events have occurred in patients receiving off-label high dose C1 inhibitor therapy. Monitor patients with known risk factors for thrombotic events.

Rev. 5/09