C1-esterase inhibitor

C1-esterase inhibitor

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

Berinert

(trade name)

Classification

Therapeutic: none assigned
Pregnancy Category: C

Indications

Treatment of acute abdominal, laryngeal, or facial 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 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.

Pharmacokinetics

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

Time/action profile (symptom relief)

ROUTEONSETPEAKDURATION
IVwithin 30 min1.5 hr>4 hr

Contraindications/Precautions

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)

Cardiovascular

  • thromboembolic events (life-threatening)

Gastrointestinal

  • abdominal pain (most frequent)
  • diarrhea (most frequent)
  • nausea (most frequent)
  • vomiting (most frequent)
  • abnormal taste

Musculoskeletal

  • muscle spasms (most frequent)

Miscellaneous

  • hypersensitivity reactions including ANAPHYLAXIS and EXACERBATION OF HAE (life-threatening)
  • ↑ severity of pain from HAE

Interactions

Drug-Drug interaction

None noted.

Route/Dosage

Intravenous (Adults) 20 units/kg.

Availability

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

Implementation

  • 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.
References in periodicals archive ?
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.
HAE, which is caused by having insufficient amounts of a plasma protein called C1-esterase inhibitor (or C1-INH), affects approximately 6,000 to 10,000 people in the U.
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.
Administration of C1-esterase inhibitor during emergency coronary artery bypass surgery in acute ST-elevation myocardial infarction.
Lev's product candidates are based on C1-esterase inhibitor ("C1-INH"), a human plasma protein that mediates inflammation and is potentially applicable as a treatment for a range of medical indications.
Our role is to deliver the best therapies available for our patients and we believe RUCONEST provides a significant treatment option for HAE patients as the one and only recombinant, non-blood derived C1-esterase inhibitor replacement therapy with a proven and consistent efficacy and safety profile.
The product is a human plasma-derived, pasteurised and nanofiltered C1-esterase inhibitor (C1-INH) concentrate, for the treatment of hereditary angioedema.
Food and Drug Administration yesterday approved Ruconest, the first recombinant C1-Esterase Inhibitor product for the treatment of acute attacks in adult and adolescent patients with hereditary angioedema (HAE).
Lev's Phase III trial, designated CHANGE (C1-Inhibitor in Hereditary Angioedema Nanofiltration Generation evaluating Efficacy), is a multi-center, placebo-controlled, double-blind study designed to examine the efficacy of the Company's lead product candidate, nano-filtered C1-esterase inhibitor ("C1-INH").
LevPharma's product candidates are based on C1-esterase inhibitor ("C1-INH"), a human plasma protein that mediates inflammation and is potentially applicable as a treatment for a range of medical indications.
Patient enrollment in the trial, which is examining the efficacy of the Company's lead product candidate, C1-esterase inhibitor ("C1-INH"), began in March 2005.
Patient enrollment has begun in the trial designed to test the efficacy of the Company's lead product candidate, C1-esterase inhibitor ("C1-INH"), in treating HAE.