c1-esterase inhibitor

Replaces malfunctioning or missing C1 esterase inhibitor in patients with HAE. Suppression of the contact activation system by C1-esterase inhibitor 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.

Pharmacokinetics ⬆ ⬇

Absorption: IV administration results in complete bioavailability.

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-Life: 18 hr.

Time/Action Profile ⬆ ⬇

(symptom relief)

ROUTE	ONSET	PEAK	DURATION
IV	within 30 min	1.5 hr	>4 hr

Contraind./Precautions ⬆ ⬇

Contraindicated in:

History of life-threatening immediate hypersensitivity reactions, including anaphylaxis to C1 esterase inhibitor preparations.

Use Cautiously in:

Risk factors for thrombotic events;
OB: Use during pregnancy only if potential maternal benefits justify potential fetal risks;
Lactation: Use while breastfeeding only if potential maternal benefits justify potential risks to infant;
Pedi: Children <12 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: THROMBOEMBOLIC EVENTS

GI: abdominal pain, diarrhea, nausea, vomiting, abnormal taste

MS: muscle spasms

Neuro: headache

Misc: ↑severity of pain from HAE, HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS AND EXACERBATION OF HAE)

Interactions ⬆ ⬇

Drug-drug:

None noted.

Route/Dosage ⬆ ⬇

IV (Adults ): 20 units/kg.

Availability ⬆ ⬇

Lyophilized powder for injection: 500 units/vial

Assessment ⬆ ⬇

Assess for signs (facial, laryngeal, or abdominal swelling, dyspnea, pain, nausea, vomiting, cramps, diarrhea) and frequency of HAE.
Monitor for signs and symptoms of severe hypersensitivity reactions (hives, urticaria, chest tightness, wheezing, hypotension, anaphylaxis) during or after injection.

Toxicity and Overdose:

May cause thrombosis if used in doses exceeding 20 units/kg.

Implementation ⬆ ⬇

IV Administration:

Intermittent Infusion: Diluent: Medication and diluent vials should be at room temperature prior to dilution. Use diluent provided by manufacturer (10 mL sterile water) and transfer using the Mix2Vial transfer set and following manufacturer's guidelines. Gently swirl vial to ensure medication is fully dissolved; do not shake. Solution should be clear and colorless; do not administer solutions that are cloudy, discolored or contain a precipitate. Reconstituted solution is stable for 8 hrs; do not refrigerate or freeze. Do not use solutions beyond expiration date. If patient is to receive more than 1 vial, the contents of multiple vials may be pooled in a single administration syringe.
Rate: Administer via slow IV injection at a rate of 4 mL/min.
Y-Site Incompatibility: Do not mix with other products and administer via a separate infusion line.

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.
Inform patient that this medication is made from human blood and may carry a risk of transmitting infectious agents (viruses and possibly Creutzfeldt-Jakob [CJD]). Report all infections to the CSL Behring Pharmacovigilance Department at 1-866-915-6958.
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 of reproductive potential patients to notify health care professional if pregnancy is planned or suspected or if breastfeeding.

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Pronunciation ⬇

Classifications ⬆ ⬇

Indications ⬆ ⬇

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