C1 ES-ter-ase in-HIB-it-or re-KOM-bin-ant

Therapeutic Classification: replacement enzyme inhibitors

Pharmacologic Classification: enzyme inhibitors

• Treatment of acute attacks of hereditary angioedema (HAE).

• 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.

• Lessened severity of HAE.

Absorption: IV administration results in complete bioavailability.

Distribution: Unknown.

Metabolism/Excretion: Unknown.

Half-life: 2.4–2.7 hr.

(symptom relief)

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: Lactation: 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).

CNS: headache (↑in children 13–17 yr).

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

CV: THROMBOEMBOLIC EVENTS.

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

Misc: HYPERSENSITIVITY REACTIONS INCLUDING ANAPHYLAXIS AND EXACERBATION OF HAE.

Drug-Drug:

• None noted.

• 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).

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

• 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.
• Monitor for thromboembolic events in patients with risk factors (indwelling venous catheter/access device, prior history of thrombosis, underlying atherosclerosis, use of oral contraceptives, morbid obesity, immobility) during and after administration.

IV Administration:

• Intermittent Infusion: Ensure c1 esterase inhibitor (recombinant) and diluent are at room temperature before mixing. 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 second 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 up to 8 hr; do not freeze.
• Rate: Inject slowly over 5 min.
• Y-Site Incompatibility: Do not mix with other medications or solutions.

• 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.

• Decrease in intensity of symptoms of HAE.

Ruconest

• Ineffective airway clearance (Indications).