c1-esterase inhibitor, recombinant

Contraindicated in:

Known/suspected hypersensitivity to rabbits/rabbit-derived products;
Previous immediate hypersensitivity reactions to C1 esterase inhibitor preparations.

Use Cautiously in:

Risk factors for thrombotic events;
OB: Use during pregnancy/lactation only if clearly needed;
Pedi: Safe and effective use in children <13 yr has not been established, increased risk of adverse reactions in children 13–17 yr).

Adv. Reactions/Side Effects ⬆ ⬇

CV: THROMBOEMBOLIC EVENTS

EENT: oropharyngeal pain (↑ in children 13–17 yr)

GI: abdominal pain (↑ in children 13–17 yr), diarrhea, nausea

Neuro: headache (↑in children 13–17 yr)

Misc: ANAPHYLAXIS AND EXACERBATION OF HAEHYPERSENSITIVITY REACTIONS INCLUDING

Interactions ⬆ ⬇

Drug-drug:

None noted.

Availability ⬆ ⬇

Lyophilized powder for intravenous use (requires reconstitution): 2100 IU/vial

Route/Dosage ⬆ ⬇

IV (Adults and adolescents ≥84 kg): 4200 IU, a second may be given if symptoms persist (not to exceed two doses/24 hr).
IV (Adults and adolescents <84 kg): 50 IU/kg, a second may be given if symptoms persist (not to exceed two doses/24 hr).

US Brand Names ⬆ ⬇

Action ⬆ ⬇

Replaces malfunctioning or missing C1 esterase inhibitor in patients with HAE. Suppression of the contact activation system by C1-esterase inihibitor prevents the cascade of events that leads to attacks of angioedema in HAE that are marked by increased vascular permeability, swelling, edema and laryngospasm.

Therapeutic effects:

Lessened severity of HAE.

Classifications ⬆ ⬇

Therapeutic Classification: replacement enzyme inhibitors

Pharmacologic Classification: enzyme inhibitors

Pharmacokinetics ⬆ ⬇

Absorption: IV administration results in complete bioavailability.

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-Life: 2.4–2.7 hr.

Time/Action Profile ⬆ ⬇

(symptom relief)

ROUTE	ONSET	PEAK	DURATION
IV	within 1 hr	unknown	16–24 hr

Patient/Family Teaching ⬆ ⬇

Explain purpose of medication to patient. Advise patient to read Patient Package Insert
May be self-administered with proper training. Patient must be able to recognize the signs and symptoms of HAE and must have the dexterity and comprehension to self-administer injection. Instruct patient using directions in Patient Package Insert. 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 HAE attack, prepare injection. Bring c1-esterase inhibitor with you to health care facility. Notify health care professional immediately if swelling does not decrease after injection.
Advise patient to notify health care professional immediately of signs of hypersensitivity reactions or thrombosis (new onset of swelling and pain in limbs or abdomen, new onset of chest pain, shortness of breath, loss of sensation or motor power, altered consciousness, vision, or speech) occur.
Caution patient to consult health care professional of planning to travel.
Advise female patients to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

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Indications ⬇

Contraind./Precautions ⬆ ⬇