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Recombinant human cerliponase alfa: Drug information

Recombinant human cerliponase alfa: Drug information
(For additional information see "Recombinant human cerliponase alfa: Patient drug information" and see "Recombinant human cerliponase alfa: Pediatric drug information")

For abbreviations, symbols, and age group definitions used in Lexicomp (show table)
Brand Names: US
  • Brineura
Brand Names: Canada
  • Brineura
Pharmacologic Category
  • Hydrolytic Lysosomal N-terminal Tripeptidyl Peptidase
Dosing: Pediatric

(For additional information see "Recombinant human cerliponase alfa: Pediatric drug information")

Note: Pretreat with antihistamines with or without antipyretics or corticosteroids 30 to 60 minutes prior to start of infusion.

Neuronal ceroid lipofuscinosis type 2

Neuronal ceroid lipofuscinosis type 2: Children ≥3 years and Adolescents: Intraventricular: 300 mg (10 mL) once every other week infused via implanted reservoir and infusion device specific for cerliponase alfa; begin therapy (first dose) 5 to 7 days after device implantation. Following cerliponase alfa infusion, administer 2 mL intraventricular electrolytes (included in administration kit).

Dosing: Kidney Impairment: Pediatric

Children ≥3 years and Adolescents: There are no dosage adjustments provided in the manufacturer's labeling.

Dosing: Hepatic Impairment: Pediatric

Children ≥3 years and Adolescents: There are no dosage adjustments provided in the manufacturer's labeling.

Adverse Reactions

The following adverse drug reactions and incidences are derived from product labeling unless otherwise specified. Adverse reactions are reported from a small study population.

>10%:

Cardiovascular: ECG abnormality (71%)

Gastrointestinal: Vomiting (63%)

Hematologic & oncologic: Hematoma (21%)

Hypersensitivity: Hypersensitivity reaction (46%; reactions may not be consistent with classic immune mediated hypersensitivity)

Immunologic: Antibody development (serum: 79%; CSF: 33%)

Nervous system: Abnormal cerebrospinal fluid (pleocytosis: 17%), abnormal proteins in cerebrospinal fluid (decreased: 71%; increased: 21%), headache (17%), irritability (17%), seizure (50%)

Miscellaneous: Fever (71%)

1% to 10%:

Cardiovascular: Bradycardia (8%), hypotension (8%)

Nervous system: Jitteriness (8%)

Respiratory: Hypoxia

Postmarketing: Hypersensitivity: Anaphylaxis

Contraindications

Sign or symptom of acute, unresolved localized infection on or around device insertion site (eg, cellulitis, abscess); suspected or confirmed CNS infection (eg, meningitis, cloudy CSF or positive CSF gram stain); acute intraventricular access device-related complications (eg, leakage, extravasation of fluid, device failure); ventriculoperitoneal shunts

Canadian labeling: Additional contraindications (not in US labeling): Severe hypersensitivity to cerliponase or any component of the formulation

Warnings/Precautions

Concerns related to adverse effects:

• Cardiovascular adverse reactions: Hypotension has been reported up to 8 hours after the completion of cerliponase alfa infusion; monitor vital signs. Use with caution in patients with a history of bradycardia, conduction disorder, or structural heart disease; monitor EKG during infusion.

• Device-related complications: Device-related complications, including device leakage and failure, extravasation of CSF fluid, and bulging of the scalp near the intraventricular access device, have been reported with cerliponase alfa. Monitor the device for signs of leakage or device failure. Discontinue use of cerliponase alfa and refer to device manufacturer's labeling if device-related complications occur. Material degradation of the intraventricular access device reservoir may occur with prolonged use; the device should be replaced prior to 4 years of single-puncture administrations (approximately 105 perforations).

• Device-related infections: Device-related infections, including bacterial meningitis and other infections requiring antibiotic treatment and device removal, have been reported with cerliponase alfa. In reported cases, patients were able to resume treatment after administration of antibiotics and removal and replacement of the intraventricular access device. Obtain a CSF sample for cell count and culture prior to each infusion and when clinically indicated; patients with CLN2 disease may not display signs and symptoms of infection. Monitor device insertion site for signs of infection. Use is contraindicated with signs of localized infection (eg, erythema, tenderness, discharge) on or near the device insertion site or suspected or confirmed CNS infection (eg, meningitis, cloudy CSF or positive CSF gram stain).

• Hypersensitivity reactions: Hypersensitivity reactions, including anaphylaxis, have been reported during and up to 24 hours after completion of cerliponase alfa infusion. Some patients also experienced pyrexia with vomiting, pleocytosis, or irritability. Pretreat with antihistamines with or without antipyretics or corticosteroids 30 to 60 minutes prior to start of infusion. Discontinue therapy immediately for severe hypersensitivity reactions or anaphylaxis and treat appropriately. Treatment of hypersensitivity reactions should be based on the severity of the reaction and may include temporarily interrupting the infusion and/or antihistamines, antipyretics, and/or corticosteroids. If the decision is made to readminister after anaphylaxis, appropriately trained personnel and emergency medications should be immediately available; initiate subsequent infusion at approximately one-half the initial infusion rate at which anaphylactic reaction occurred.

Other warnings/precautions:

• Appropriate use: Administer cerliponase alfa by FDA-approved infusion pump system via intraventricular implanted catheter access (consult prescribing information for device details); health care providers should be experienced with intraventricular drug administration.

Dosage Forms: US

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Solution, Intraventricular [preservative free]:

Brineura: 150 mg/5 mL (1 ea)

Generic Equivalent Available: US

No

Pricing: US

Kit (Brineura Intraventricular)

2 X 150 mg/5 mL (per each): $38,374.80

Disclaimer: A representative AWP (Average Wholesale Price) price or price range is provided as reference price only. A range is provided when more than one manufacturer's AWP price is available and uses the low and high price reported by the manufacturers to determine the range. The pricing data should be used for benchmarking purposes only, and as such should not be used alone to set or adjudicate any prices for reimbursement or purchasing functions or considered to be an exact price for a single product and/or manufacturer. Medi-Span expressly disclaims all warranties of any kind or nature, whether express or implied, and assumes no liability with respect to accuracy of price or price range data published in its solutions. In no event shall Medi-Span be liable for special, indirect, incidental, or consequential damages arising from use of price or price range data. Pricing data is updated monthly.

Dosage Forms: Canada

Excipient information presented when available (limited, particularly for generics); consult specific product labeling.

Kit, Intraventricular:

Brineura: 2 X 150 MG/5ML

Administration: Pediatric

Note: Premedicate with antihistamines with or without antipyretics or corticosteroids 30 to 60 minutes prior to infusion.

Intraventricular: For intraventricular use only; administer to the intraventricular space by infusion pump via a surgically implanted reservoir and catheter (see manufacturer labeling for specific product codes of reservoir, catheter, and infusion pump system required for cerliponase alfa administration). Inspect scalp prior to each infusion for leakage or failure of intraventricular access device or signs of infection. Use a 0.2 micron inline filter (provided in kit) and infuse 10 mL of cerliponase alfa at 2.5 mL/hour (4 hours total); after completion of dose, administer intraventricular electrolytes (2 mL volume, included in administration kit) at 2.5 mL/hour; total infusion time (combined drug and electrolyte infusions) is approximately 4.5 hours. See manufacturer labeling for details on intraventricular infusion.

Note: Monitor for anaphylaxis/hypersensitivity reactions during the infusion. Discontinue therapy immediately for severe hypersensitivity reactions or anaphylaxis and treat appropriately. Treatment of hypersensitivity reactions should be based on the severity of the reaction and may include temporarily interrupting the infusion and/or antihistamines, antipyretics, and/or corticosteroids. If the decision is made to readminister after anaphylaxis, appropriately trained personnel and emergency medications should be immediately available; initiate subsequent infusion at approximately 1/2 the initial infusion rate at which anaphylactic reaction occurred.

Use: Labeled Indications

Neuronal ceroid lipofuscinosis type 2: Delay the loss of ambulation in symptomatic pediatric patients ≥3 years of age with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2)

Metabolism/Transport Effects

None known.

Drug Interactions

There are no known significant interactions.

Pregnancy Considerations

Animal reproduction studies have not been conducted with cerliponase alfa.

Breastfeeding Considerations

It is not known if cerliponase alfa is present in breast milk. According to the manufacturer, the decision to breastfeed during therapy should consider the risk of infant exposure, the benefits of breastfeeding to the infant, and benefits of treatment to the mother.

Monitoring Parameters

Symptoms of anaphylaxis and hypersensitivity reactions (during infusion and within 24 hours following infusion); vital signs (blood pressure, heart rate) prior to start of infusion, periodically during infusion and postinfusion; skin integrity and signs of infection, device leakage or failure (prior to infusion); routine cerebrospinal fluid samples prior to each infusion and when clinically indicated (to detect subclinical device infections); in patients with cardiac abnormalities, ECG during infusion, and every 6 months in patients without cardiac abnormalities.

Mechanism of Action

Cerliponase alfa is a proenzyme that, once activated, cleaves tripeptides from the N-terminus of proteins. This leads to the breakdown of lysosomal storage materials that otherwise accumulate in patients with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), leading to progressive decline in motor function.

Intraventricular electrolytes (included in the administration kit) are used to flush the infusion line, port needle, and intraventricular access device in order to fully administer cerliponase alfa and maintain patency of the intraventricular access device.

Pharmacokinetics (Adult Data Unless Noted)

Distribution: Pediatric patients ≥3 years: VSS: CSF: Median range: 186 to 245 mL; with repeat dosing, volume was observed to decrease

Metabolism: Degraded via peptide hydrolysis

Half-life elimination: CSF: 6.2 to 7.7 hours

Time to peak: Pediatric patients ≥3 years: CSF: Median range: 4.3 to 4.5 hours after start of infusion; Plasma: Median range: 12 to 12.3 hours after start of infusion

Brand Names: International
International Brand Names by Country
For country code abbreviations (show table)

  • (AT) Austria: Brineura;
  • (BG) Bulgaria: Brineura;
  • (CO) Colombia: Brineura;
  • (CZ) Czech Republic: Brineura;
  • (DE) Germany: Brineura;
  • (FR) France: Brineura;
  • (GB) United Kingdom: Brineura;
  • (HU) Hungary: Brineura;
  • (MX) Mexico: Brineura;
  • (PL) Poland: Brineura;
  • (PR) Puerto Rico: Brineura;
  • (RU) Russian Federation: Brineura;
  • (SE) Sweden: Brineura;
  • (SK) Slovakia: Brineura
  1. Brineura (cerliponase alfa) [prescribing information]. Novato, CA: BioMarin Pharmaceutical Inc; March 2020.
  2. Brineura (cerliponase alfa) [product monograph]. Toronto, ON, Canada: BioMarin Pharmaceutical (Canada) Inc; December 2018.
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