C1-esterase inhibitor

C1-esterase inhibitor

(see won ess-ter-aze in-hib-it-or) ,


(trade name)


Therapeutic: none assigned
Pregnancy Category: C


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.

Therapeutic effects

Lessened severity of HAE.


Absorption: IV administration results in complete bioavailability.
Distribution: Unknown.
Metabolism and Excretion: Unknown.
Half-life: 18 hr.

Time/action profile (symptom relief)

IVwithin 30 min1.5 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 interaction

None noted.


Intravenous (Adults) 20 units/kg.


Lyophilized powder for intravenous use (requires reconstitution): 500 units/vial

Nursing implications

Nursing 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.
  • May cause thrombosis if used in doses exceeding 20 units/kg.

Potential Nursing Diagnoses

Activity intolerance


  • Intravenous Administration
  • 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.

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.
  • 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.

Evaluation/Desired Outcomes

  • Decrease in intensity of symptoms of HAE.
Drug Guide, © 2015 Farlex and Partners
References in periodicals archive ?
Based on drug class, the market has been segmented into C1-inhibitors (C1-esterase Inhibitor, Recombinant Inhibitor); Kallikrein Inhibitor (Kalbitor); Selective Bradykinin B2 Receptor Antagonist (Firazyr).
The C1-Esterase inhibitor (C1-INH) has been approved in the US for routine prophylaxis against angioedema attacks in adolescents and adults with HAE since October 2008.
Fresh frozen plasma (FFP) has been used in under-resourced areas for life-threatening attacks, as fresh plasma contains some active C1-esterase inhibitor. There has, however, been very little documentation of its effectiveness in blinded or placebo studies, [3,4] although it is widely used empirically.
Current treatment options include bradykinin receptor antagonist icatibant, ecallantide, C1-esterase inhibitor (C1-INH) concentrate, progestin, and antifibrinolytic agent tranexamic acid (TA) [4-17].
Administration of C1-esterase inhibitor during emergency coronary artery bypass surgery in acute ST-elevation myocardial infarction.
Hereditary angio-oedema (HAE) is a life-threatening autosomal dominant condition caused by a deficiency in the C1-esterase inhibitor (C1-INH), a pivotal protein in the complement and fibrinolytic pathways.
Haegarda is a self-administered, plasma-derived concentrate of C1-esterase inhibitor and the only subcutaneous therapy approved in the United States for routine prophylaxis to prevent HAE attacks in adolescent and adult patients.
CSL announced on Wednesday that it has become aware that Shire ViroPharma, a US-based pharmaceutical company, has filed a complaint in the US District Court for the District of Delaware, alleging infringement of a newly granted US patent in connection with a method of treating Hereditary Angioedema (HAE) by subcutaneously administering C1-esterase inhibitor. It went on to say that CSL remains highly confident that CSL830 does not infringe any valid claim of the Shire ViroPharma patent and will vigorously defend against the claims.
The product is a human plasma-derived, pasteurised and nanofiltered C1-esterase inhibitor (C1-INH) concentrate, for the treatment of hereditary angioedema.