C1-esterase inhibitor

(redirected from Berinert)

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 ?
They identified members with claims for one of four specialty drugs prescribed for HAE: Berinert, Cinryze, Firazyr, and Kalbitor.
FIRAZYR competes in Europe with CSL Behring's BERINERT P, a human plasma-derived C1-esterase inhibitor (C1-INH) product, and with Pharming Group N.
Berinert (r) P, a C1-inhibitor (C1-INH) concentrate, is effective in safely relieving abdominal pain and abdominal-wall tension in patients with hereditary angioedema (HAE) who experience abdominal attacks, according to data presented today at the European Academy of Allergology and Clinical Immunology (EAACI) XXV Congress.
Production delays have reportedly impacted the supply of one C1-INH product as demand for both it and Berinert continue to grow.
About Berinert Berinert , C1 Esterase Inhibitor (Human), a pasteurized, nanofiltered, plasma-derived intravenous therapy, treats acute attacks of facial, abdominal and laryngeal hereditary angioedema (HAE) symptoms by providing C1 Esterase Inhibitor deficient adult and adolescent patients with the missing human protein.
In addition to FIRAZYR, BioRx is a distributor of BERINERT [C1 esterase inhibitor (human)], the only FDA-approved C1 esterase inhibitor indicated for acute HAE attacks.
MARBURG, Germany, April 16, 2013 /PRNewswire/ -- CSL Behring today announced that European health authorities have approved an extended use of Berinert , a C1-esterase inhibitor (C1-INH) concentrate, for pre-procedure prevention (short-term prophylaxis) of acute episodes of hereditary angioedema (HAE), in adult and pediatric patients undergoing medical, dental or surgical procedures.
BioRx is one of a limited number of specialty pharmacies selected by CSL Behring to dispense Berinert [R], which was just recently approved by the US Food and Drug Administration (FDA) for the treatment of adult and adolescent patients with acute abdominal or facial attacks of HAE.
2, 2013 /PRNewswire/ -- CSL Behring announced today the launch of a financial assistance program designed to cover patients' out-of-pocket expenses for Berinert [sup.
CSL Behring Receives FDA Approval for Berinert In HAE Treatment II-50
Food and Drug Administration (FDA) has approved a label expansion for self-administration of Berinert [sup.
27, 2011 /PRNewswire/ -- CSL Behring announced today it has been granted national marketing authorization in Israel to market Berinert for the treatment of acute hereditary angioedema (HAE) attacks in any body location.