c1 inhibitor (human)

REMS

Routine prophylaxis against angioedema attacks in adult and adolescent patients with Hereditary Angioedema (HAE).

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.

Availability ⬆ ⬇

Powder for injection: 500 units/vial

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.

US Brand Names ⬆ ⬇

Action ⬆ ⬇

Replaces C1 inhibitor which is deficient in patients with HAE. C1 inhibitor is necessary in preventing the chain of events which alter vascular permeability resulting in life-threatening swelling in patients with HAE.

Therapeutic effects:

Decreased frequency, intensity and duration of HAE attacks.

Classifications ⬆ ⬇

Therapeutic Classification: antiangioedema agents

Pharmacologic Classification: proteinase inhibitors

Pharmacokinetics ⬆ ⬇

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

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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Indications ⬇