HIGHLIGHTS OF PRESCRIBING INFORMATION
These highlights do not include all the information needed to use BRINEURA safely and effectively. See full prescribing information for BRINEURA.
BRINEURA (cerliponase alfa) injection, for intraventricular use
Initial U.S. Approval: 2017
INDICATIONS AND USAGE
Brineura is a hydrolytic lysosomal N-terminal tripeptidyl peptidase indicated to slow the loss of ambulation in symptomatic pediatric patients 3 years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency. (1)

DOSAGE AND ADMINISTRATION

Aseptic technique must be strictly observed during preparation and administration. Brineura should be administered by, or under the direction of a physician knowledgeable in intraventricular administration. Brineura is administered to the cerebrospinal fluid (CSF) by infusion via a surgically implanted reservoir and catheter. (2.1)
Pre-treatment of patients with antihistamines with or without antipyretics or corticosteroids is recommended 30 to 60 minutes prior to the start of infusion. (2.2)
The recommended dosage is 300 mg administered once every other week as an intraventricular infusion followed by infusion of Intraventricular Electrolytes over approximately 4.5 hours. (2.2)
For complete information on preparation, specific intraventricular access device for use, and administration, see the full prescribing information. (2.1, 2.3, 2.4, 2.5)
DOSAGE FORMS AND STRENGTHS

Injection: Brineura 150 mg/5 mL (30 mg/mL) solution, two single‑dose vials per carton co-packaged with Intraventricular Electrolytes Injection 5 mL in a single-dose vial. (3)
CONTRAINDICATIONS

Acute intraventricular access device-related complications (e.g., leakage, device failure, or device-related infection). (4)

Patents with ventriculoperitoneal shunts. (4)
WARNINGS AND PRECAUTIONS
Intraventricular Access Device-Related Complications: Inspect the scalp for skin integrity and for signs of intraventricular access device leakage. Do not administer if there are signs of device leakage or infection. Routinely send CSF samples for testing to detect subclinical device-related infections. (2.5, 5.1)
Cardiovascular Adverse Reactions: Monitor vital signs before, during, and post-infusion. Monitor Electrocardiogram (ECG) in patients with a history of bradycardia, conduction disorder, or with structural heart disease, during the infusion. In patients without cardiac abnormalities, perform regular 12-lead ECG eva luations every 6 months. (2.5, 5.2)
Hypersensitivity Reactions: Observe patients during and after the infusion.  If a severe hypersensitivity reaction occurs, immediately stop the infusion and initiate appropriate treatment. (5.3)
ADVERSE REACTIONS
Most common adverse reactions (≥8%) are: pyrexia, ECG abnormalities, decreased CSF protein, vomiting, seizures, hypersensitivity, increased CSF protein, hematoma, headache, irritability, pleocytosis, device-related infection, bradycardia, feeling jittery, and hypotension. (6.1)
To report SUSPECTED ADVERSE REACTIONS, contact BioMarin at 1-866-906-6100 or FDA at 1-800-FDA-1088 or www.fda.gov/medwatch.
See 17 for PATIENT COUNSELING INFORMATION.
Revised: 4/2017
FULL PRESCRIBING INFORMATION: CONTENTS*
1     INDICATIONS AND USAGE