Replaces C1-esterase inhibitor which is deficient in patients with hereditary angioedema. C1 inhibitor is necessary in preventing the chain of events which alter vascular permeability resulting in life-threatening swelling in hereditary angioedema.
Therapeutic Effect(s):
Decreased intensity of hereditary angioedema attacks.
Known/suspected hypersensitivity to rabbits/rabbit-derived products
Previous immediate hypersensitivity reactions to C1-esterase inhibitor preparations.
Use Cautiously in:
Presence of indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives or certain androgens, morbid obesity, and immobility (↑ risk of thromboembolism)
OB: C1-esterase inhibitor (human) is preferred for the treatment of hereditary angioedema during pregnancy;
Pedi: Children <13 yr (safety and effectiveness not established).
Monitor for signs/symptoms of hypersensitivity reactions (rash, urticaria, pruritus, flushing, dizziness, vomiting, abdominal pain) and angioedema (swelling of throat, lips, tongue, face, dyspnea, wheezing, hoarseness). If reaction occurs, discontinue C1-esterase inhibitor immediately and provide supportive care.
Monitor for signs/symptoms of venous thromboembolism such as pulmonary embolism (chest pain, dyspnea, tachycardia) or deep vein thrombosis (calf pain or tenderness, lower extremity edema, localized warmth or erythema) or stroke (headache, facial numbness, unilateral weakness, aphasia, eye pain or swelling, vision changes). If thromboembolic event suspected, discontinue C1-esterase inhibitor.
Intermittent Infusion: Medication and diluent vials should be at room temperature before mixing. Reconstitution: Reconstitute by withdrawing 14 mL of sterile water for injection. Add diluent slowly; avoid forceful impact on powder. Swirl slowly to mix; avoid foaming. Repeat with 2nd vial. Solution should be clear and colorless; do not administer solutions that are discolored or contain particulate matter. Use solution immediately or may be refrigerated for ≤ 8 hr; do not freeze.
Rate: Inject slowly over 5 min.
Y-Site Incompatibility:
Do not administer other drugs through same IV line.
Explain purpose and side effects of C1-esterase inhibitor. Do not stop receiving drug without consulting health care provider. If an appointment is missed, reschedule as soon as possible. Advise patient to read Patient Information before starting and periodically during therapy in case of changes.
May be self-administered with proper training. Patient must be able to recognize the signs/symptoms of hereditary angioedema and must have the dexterity and comprehension to self-administer injection. Instruct patient or caregiver in correct technique for injection, storage, and place to discard syringes and needles. Advise patient to seek medical attention immediately in addition to injection due to potential for laryngeal edema. Instruct patient to record lot number from vial. At first sign of hereditary angioedema attack, prepare injection. Bring C1-esterase inhibitor with you to health care facility. Notify health care provider immediately if swelling does not decrease after injection.
Advise patient to notify health care provider immediately if signs/symptoms of hypersensitivity reactions or thrombosis occur.
Caution patient to consult health care provider of planning to travel.
Rep: Advise women of reproductive potential to notify health care provider if pregnancy is planned or suspected or if breastfeeding.