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C1-esterase inhibitor(see won ess-ter-aze in-hib-it-or) ,
Pregnancy Category: C
ClassificationTherapeutic: none assigned
Treatment of acute abdominal, laryngeal, or facial 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 inihibor 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.
Metabolism and Excretion: Unknown.
Half-life: 18 hr.
Time/action profile (symptom relief)
|IV||within 30 min||1.5 hr||>4 hr|
Contraindicated in: History of life-threatening immediate hypersensitivity reactions, including anaphylaxis to C1 esterase inhibitor preparations.
Use Cautiously in: Risk factors for thrombotic events; Obstetric / Lactation: Use during pregnancy/lactation only if clearly needed; Pediatric: Children <12 yr (safety and effectiveness not established).
Adverse Reactions/Side Effects
Central nervous system
- headache (most frequent)
- thromboembolic events (life-threatening)
- abdominal pain (most frequent)
- diarrhea (most frequent)
- nausea (most frequent)
- vomiting (most frequent)
- abnormal taste
- muscle spasms (most frequent)
- hypersensitivity reactions including ANAPHYLAXIS and EXACERBATION OF HAE (life-threatening)
- ↑ severity of pain from HAE
Drug-Drug interactionNone noted.
Intravenous (Adults) 20 units/kg.
Lyophilized powder for intravenous use (requires reconstitution): 500 units/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. May cause thrombosis if used in doses exceeding 20 units/kg.
Potential Nursing DiagnosesActivity intolerance
- 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.
- 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 patients to notify health care professional if pregnancy is planned or suspected or if breastfeeding.
- Decrease in intensity of symptoms of HAE.