General

Pronunciation:
C1 es-ter-ase in-hib-it-or re-kom-bin-ant

Trade Name(s)

• Ruconest

Ther. Class.

replacement enzyme inhibitors

Pharm. Class.

enzyme inhibitors

Indications

Treatment of acute attacks of hereditary angioedema (HAE).

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 Effect(s):

Lessened severity of HAE.

Pharmacokinetics

Absorption: IV administration results in complete bioavailability.

Distribution: Unknown.

Metabolism and Excretion: Unknown.

Half-life: 2.4–2.7 hr.

TIME/ACTION PROFILE (symptom relief)

Contraindication/Precautions

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).

Adverse 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: hypersensitivity reactions including ANAPHYLAXIS AND EXACERBATION OF HAE

* CAPITALS indicate life-threatening.
Underline indicate most frequent.

Interactions

Drug-Drug

None noted.

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).

Availability

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

Implementation

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.

Evaluation/Desired Outcomes

Decrease in intensity of symptoms of HAE.