c1 inhibitor (human)

Absorption: IV administration results in complete bioavailibility.

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-Life: Single dose: 56 hr.

Time/Action Profile ⬆ ⬇

ROUTE	ONSET	PEAK	DURATION
IV	within 1 hr	12 hr	3–4 days

Contraind./Precautions ⬆ ⬇

Contraindicated in:

Life-threatening immediate hypersensitivity reactions.

Use Cautiously in:

Patients with known risk of thrombotic events;
OB: Safety not established in pregnancy;
Lactation: Use while breastfeeding only if potential maternal benefit justifies potential risk to infant;
Pedi: Children <6 yr (safety and effectiveness not established).

Adv. Reactions/Side Effects ⬆ ⬇

CV: THROMBOEMBOLIC EVENTS

Derm: rash

Neuro: headache

Misc: HYPERSENSITIVITY REACTIONS (INCLUDING ANAPHYLAXIS)

Interactions ⬆ ⬇

Drug-drug:

None noted.

Route/Dosage ⬆ ⬇

IV (Adults and Children ≥12 yr): 1000 units every 3 or 4 days. If inadequate response, doses up to 2500 units (not to exceed 100 units/kg) every 3 or 4 days may be used.
IV (Children 6–11 yr): 500 units every 3 or 4 days. If inadequate response, doses up to 1000 units every 3 or 4 days may be used.

Availability ⬆ ⬇

Powder for injection: 500 units/vial

Assessment ⬆ ⬇

Assess for signs (facial or abdominal swelling, pain, nausea, vomiting, cramps, diarrhea) and frequency of HAE.
Assess for signs and symptoms of hypersensitivity reactions (hives, urticaria, tightness of the chest, wheezing, hypotension, anaphylaxis) during or after injection. Symptoms may be similar to HAE attacks; consider treatment methods carefully. If hypersensitivity occurs, discontinue infusion treat symptomatically. Epinephrine should be immediately available for treatment of acute severe hypersensitivity reaction.
Monitor patients with known risk factors for thrombotic events.

Implementation ⬆ ⬇

IV Administration:

Intermittent Infusion: Diluent: Reconstitute each of 2 vials with 5 mL of Sterile Water for Injection by removing the protective covering from one end of the double-ended transfer needle and inserting exposed needle through the center of the diluent vial stopper. Remove protective covering from the other end of the double-ended transfer needle. Invert diluent vial over the upright and slightly angled C1 inhibitor vial; then rapidly insert the free end of the needle through the center of the C1 inhibitor vial stopper. Vacuum in the vial will draw in the diluent. Do not use if there is no vacuum in the vial. Disconnect the two vials by removing the needle from the C1 inhibitor vial stopper and discard the diluent vial, along with the transfer needle directly into the sharps container. Gently swirl C1 inhibitor vial until all powder is completely dissolved. Solution is colorless to slightly blue; do not administer solutions that are discolored, turbid, or contain a precipitate. Insert the filter needle into the vial of reconstituted solution. Inject air into the vial and withdraw the reconstituted C1 inhibitor into the syringe. Repeat with a second vial to make complete dose.Concentration: 100 units/mL. Discard partially used vials. Attach a suitable needle or infusion set with winged adapter, and inject intravenously. Administer at room temperature within 3 hr of reconstitution. Do not freeze; protect solution from light.
Rate: Administer at an initial infusion rate 1 mL/min over 10 min or 500 units over 5 min. If tolerated, continue same as the maintenance infusion rate.
Y-Site Incompatibility: Do not mix with other materials.

Patient/Family Teaching ⬆ ⬇

Inform patient that C1 inhibitor is made from human plasma and may contain infectious agents (viruses and possibly Creutzfeldt-Jakob [CJD]) that can cause disease.
Instruct patient to notify health care professional immediately if signs and symptoms of allergic hypersensitivity reactions or thrombosis (new onset swelling and pain in limbs or abdomen, new onset chest pain, shortness of breath, loss of sensation or motor power, altered consciousness or speech) occur.
Advise female patients to notify if health care professional if pregnancy is planned or suspected or if breastfeeding.

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Pronunciation ⬇

Classifications ⬆ ⬇

Indications ⬆ ⬇

Action ⬆ ⬇

Pharmacokinetics ⬆ ⬇