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Drug overview for BRINEURA (cerliponase alfa):
Generic name: CERLIPONASE ALFA
Drug class: Drugs to Treat Neuronal Ceroid Lipofuscinosis type 2 (CLN2)
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Cerliponase alfa is a biosynthetic (recombinant DNA origin) form of human tripeptidyl peptidase-1 (TPP1), a lysosomal enzyme that catabolizes polypeptides in the CNS.
No enhanced Uses information available for this drug.
Generic name: CERLIPONASE ALFA
Drug class: Drugs to Treat Neuronal Ceroid Lipofuscinosis type 2 (CLN2)
Therapeutic class: Metabolic Disease Enzyme Replacement Agents
Cerliponase alfa is a biosynthetic (recombinant DNA origin) form of human tripeptidyl peptidase-1 (TPP1), a lysosomal enzyme that catabolizes polypeptides in the CNS.
No enhanced Uses information available for this drug.
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The following indications for BRINEURA (cerliponase alfa) have been approved by the FDA:
Indications:
Treatment to slow loss of ambulation in late infantile neuronal ceroid lipofuscinosis type 2
Professional Synonyms:
Treatment to slow loss of ambulation in tripeptidyl peptidase 1 deficiency
Treatment to slow motor decline in late infantile neuronal ceroid lipofuscinosis type 2
Indications:
Treatment to slow loss of ambulation in late infantile neuronal ceroid lipofuscinosis type 2
Professional Synonyms:
Treatment to slow loss of ambulation in tripeptidyl peptidase 1 deficiency
Treatment to slow motor decline in late infantile neuronal ceroid lipofuscinosis type 2
The following dosing information is available for BRINEURA (cerliponase alfa):
The recommended dosage of cerliponase alfa in pediatric patients 3 years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) disease is 300 mg once every other week by intraventricular infusion.
It is recommended that the first dose of cerliponase alfa be administered at least 5-7 days after implantation of the intraventricular access device.
It is recommended that the first dose of cerliponase alfa be administered at least 5-7 days after implantation of the intraventricular access device.
Cerliponase alfa is administered directly into the CSF by intraventricular infusion via a surgically implanted intraventricular access device consisting of a reservoir (Codman(R) Holter Rickham reservoir) and catheter (Codman(R) ventricular catheter). The intraventricular access device must be implanted prior to (manufacturer recommends 5-7 days) administration of the first infusion. Infusions of cerliponase alfa should be administered using a controlled infusion device (B Braun Perfusor(R) Space infusion pump system).
Each infusion of cerliponase alfa should be followed by an infusion of intraventricular electrolytes (supplied by the manufacturer) to facilitate drug delivery and maintain patency of the intraventricular access device. Both cerliponase alfa and the electrolyte solution should be administered by intraventricular infusion at a rate of 2.5 mL/hour using the recommended syringe pump (B Braun Perfusor(R) Space infusion pump system); the duration of the complete infusion (cerliponase alfa plus the required infusion of intraventricular electrolytes) is approximately 4.5
hours. The complete infusion must be administered using an infusion set with a 0.2-mcm inline filter.
Proper aseptic technique must be strictly observed during preparation and administration of cerliponase alfa. Prior to use, vials of cerliponase alfa and intraventricular electrolytes should be stored upright in the freezer and kept in their original package to protect from light. The manufacturer-supplied administration kit (containing syringes, syringe needles, an extension line, infusion set with 0.2-mcm inline filter, and a port needle) should be stored separately from the drug and should not be frozen.
Prior to intraventricular infusion, vials of cerliponase alfa and intraventricular electrolytes should be thawed at room temperature for approximately 60 minutes. The vials should not be thawed or warmed in any other way or shaken; condensation will occur during the thawing process. Commercially available cerliponase alfa injection for intraventricular use is a clear to slightly opalescent and colorless to pale yellow solution, and the intraventricular electrolytes injection is a clear to colorless solution.
After the drug and electrolyte vials are completely thawed, they should be inspected visually for particulate matter or discoloration; the solutions should not be used if discoloration or particulate matter other than a few naturally occurring particles of the drug (e.g., thin translucent fibers or opaque particulate matter that is removed by the 0.2-mcm inline filter) are present. Vials containing intraventricular electrolytes also may contain particulate matter that may appear during the thawing period but should dissolve once the solution reaches room temperature. Cerliponase alfa and the intraventricular electrolytes solution should be used immediately once thawed; if not used immediately, unopened vials should be stored in the refrigerator at 2-8degreesC and used within 24 hours.
Thawed solution should not be refrozen. To prepare the cerliponase alfa solution for intraventricular infusion, a total of 10 mL should be withdrawn from two 5-mL vials of cerliponase alfa into a syringe; the syringe should be properly labeled to indicate that the contents contain cerliponase alfa. To prepare the electrolyte solution, a total of 2 mL should be withdrawn from the vial of intraventricular electrolytes into a syringe; the syringe should be properly labeled to indicate that the contents contain intraventricular electrolytes.
Syringes should be used immediately following preparation. If not used immediately, the syringes should be stored in the refrigerator at 2-8degreesC and used within 4 hours. Vials of cerliponase alfa and intraventricular electrolytes are intended for single use only; any unused portions should be discarded.
Cerliponase alfa should not be diluted or mixed with any other drug. Prior to infusion, the patient's scalp should be inspected for signs of leakage or failure of the intraventricular access device and for potential infections; the scalp should be prepared for intraventricular infusion per institution standard of care. A total of 0.5-1
mL of CSF should be withdrawn to check patency of the intraventricular access device and the specimen should be sent for culture. (See Intraventricular Access Device-related Complications under Cautions: Warnings/Precautions.) The infusion system also should be inspected periodically during infusion for signs of leakage or delivery failure. Following completion of the infusion, the infusion components, needles, unused solutions, and other waste materials should be disposed of in accordance with local requirements.
Each infusion of cerliponase alfa should be followed by an infusion of intraventricular electrolytes (supplied by the manufacturer) to facilitate drug delivery and maintain patency of the intraventricular access device. Both cerliponase alfa and the electrolyte solution should be administered by intraventricular infusion at a rate of 2.5 mL/hour using the recommended syringe pump (B Braun Perfusor(R) Space infusion pump system); the duration of the complete infusion (cerliponase alfa plus the required infusion of intraventricular electrolytes) is approximately 4.5
hours. The complete infusion must be administered using an infusion set with a 0.2-mcm inline filter.
Proper aseptic technique must be strictly observed during preparation and administration of cerliponase alfa. Prior to use, vials of cerliponase alfa and intraventricular electrolytes should be stored upright in the freezer and kept in their original package to protect from light. The manufacturer-supplied administration kit (containing syringes, syringe needles, an extension line, infusion set with 0.2-mcm inline filter, and a port needle) should be stored separately from the drug and should not be frozen.
Prior to intraventricular infusion, vials of cerliponase alfa and intraventricular electrolytes should be thawed at room temperature for approximately 60 minutes. The vials should not be thawed or warmed in any other way or shaken; condensation will occur during the thawing process. Commercially available cerliponase alfa injection for intraventricular use is a clear to slightly opalescent and colorless to pale yellow solution, and the intraventricular electrolytes injection is a clear to colorless solution.
After the drug and electrolyte vials are completely thawed, they should be inspected visually for particulate matter or discoloration; the solutions should not be used if discoloration or particulate matter other than a few naturally occurring particles of the drug (e.g., thin translucent fibers or opaque particulate matter that is removed by the 0.2-mcm inline filter) are present. Vials containing intraventricular electrolytes also may contain particulate matter that may appear during the thawing period but should dissolve once the solution reaches room temperature. Cerliponase alfa and the intraventricular electrolytes solution should be used immediately once thawed; if not used immediately, unopened vials should be stored in the refrigerator at 2-8degreesC and used within 24 hours.
Thawed solution should not be refrozen. To prepare the cerliponase alfa solution for intraventricular infusion, a total of 10 mL should be withdrawn from two 5-mL vials of cerliponase alfa into a syringe; the syringe should be properly labeled to indicate that the contents contain cerliponase alfa. To prepare the electrolyte solution, a total of 2 mL should be withdrawn from the vial of intraventricular electrolytes into a syringe; the syringe should be properly labeled to indicate that the contents contain intraventricular electrolytes.
Syringes should be used immediately following preparation. If not used immediately, the syringes should be stored in the refrigerator at 2-8degreesC and used within 4 hours. Vials of cerliponase alfa and intraventricular electrolytes are intended for single use only; any unused portions should be discarded.
Cerliponase alfa should not be diluted or mixed with any other drug. Prior to infusion, the patient's scalp should be inspected for signs of leakage or failure of the intraventricular access device and for potential infections; the scalp should be prepared for intraventricular infusion per institution standard of care. A total of 0.5-1
mL of CSF should be withdrawn to check patency of the intraventricular access device and the specimen should be sent for culture. (See Intraventricular Access Device-related Complications under Cautions: Warnings/Precautions.) The infusion system also should be inspected periodically during infusion for signs of leakage or delivery failure. Following completion of the infusion, the infusion components, needles, unused solutions, and other waste materials should be disposed of in accordance with local requirements.
No dosing information available.
No generic dosing information available.
The following drug interaction information is available for BRINEURA (cerliponase alfa):
There are 0 contraindications.
There are 0 severe interactions.
There are 0 moderate interactions.
The following contraindication information is available for BRINEURA (cerliponase alfa):
Drug contraindication overview.
Cerliponase alfa is contraindicated in patients with acute intraventricular access device-related complications (e.g., leakage, device failure, device-related infection) or ventriculoperitoneal shunts.
Cerliponase alfa is contraindicated in patients with acute intraventricular access device-related complications (e.g., leakage, device failure, device-related infection) or ventriculoperitoneal shunts.
There are 1 contraindications.
Absolute contraindication.
| Contraindication List |
|---|
| Central nervous system infection |
There are 0 severe contraindications.
There are 2 moderate contraindications.
Clinically significant contraindication, where the condition can be managed or treated before the drug may be given safely.
| Moderate List |
|---|
| Bradycardia |
| Structural disorder of heart |
The following adverse reaction information is available for BRINEURA (cerliponase alfa):
Adverse reaction overview.
Adverse effects reported in 8% or more of patients receiving cerliponase alfa in clinical studies include pyrexia, ECG abnormalities, decreased CSF protein, vomiting, seizures, hypersensitivity, increased CSF protein, hematoma, headache, irritability, pleocytosis, device-related infection, bradycardia, feeling jittery, and hypotension.
Adverse effects reported in 8% or more of patients receiving cerliponase alfa in clinical studies include pyrexia, ECG abnormalities, decreased CSF protein, vomiting, seizures, hypersensitivity, increased CSF protein, hematoma, headache, irritability, pleocytosis, device-related infection, bradycardia, feeling jittery, and hypotension.
There are 10 severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Abnormal ECG Hypersensitivity drug reaction Seizure disorder |
Bradycardia Hypotension Infection |
| Rare/Very Rare |
|---|
|
Anaphylaxis Bacterial meningitis Hypoxia Tachycardia |
There are 11 less severe adverse reactions.
| More Frequent | Less Frequent |
|---|---|
|
Decreased protein level in cerebrospinal fluid Elevated protein level in cerebrospinal fluid Fever Headache disorder Irritability Pleocytosis of cerebrospinal fluid Vomiting |
Hematoma Nervousness |
| Rare/Very Rare |
|---|
|
Diarrhea Skin rash |
The following precautions are available for BRINEURA (cerliponase alfa):
Safety and efficacy of cerliponase alfa have not been established in pediatric patients younger than 3 years of age.
Contraindicated
Severe Precaution
Management or Monitoring Precaution
Contraindicated
| None |
Severe Precaution
| None |
Management or Monitoring Precaution
| None |
There are no adequate and well-controlled studies of cerliponase alfa in pregnant women. Animal reproduction studies with the drug have not been performed to date.
It is not known whether cerliponase alfa is distributed into human milk, affects milk production, or affects the breast-fed infant. The known benefits of breast-feeding should be considered along with the mother's clinical need for cerliponase alfa and any potential adverse effects of the drug or disease on the infant.
No enhanced Geriatric Use information available for this drug.
The following prioritized warning is available for BRINEURA (cerliponase alfa):
No warning message for this drug.
No warning message for this drug.
The following icd codes are available for BRINEURA (cerliponase alfa)'s list of indications:
| Treatment to slow loss of walking in late infantile CLn2 | |
| E75.4 | Neuronal ceroid lipofuscinosis |
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