Generic Name and Formulations:
Cerliponase alfa 30mg/mL; soln for intraventricular infusion.
To slow the loss of ambulation in late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency.
<3yrs: not established. See full labeling. Give by intraventricular infusion via implanted access device; administer first dose at least 5–7 days post-implantation. Pre-treat with antihistamines ± antipyretics or corticosteroids 30–60mins prior to infusion. Infuse Brineura first, followed by Intraventricular Electrolytes each at a rate of 2.5mL/hr. ≥3yrs: 300mg once every other week.
Patients with acute intraventricular access device-related complications (eg, leakage, device failure, infection) or ventriculoperitoneal shunts.
Should be administered by trained healthcare providers. Inspect the scalp to ensure access device is not compromised prior to each infusion. Discontinue if access device-related complications develop. Routinely test CSF samples to detect subclinical device infections. Monitor BP and HR before starting, during, and post-infusion. History of bradycardia, conduction disorder, structural heart disease: perform ECG during infusion; without cardiac abnormalities: perform ECG every 6 months. Have appropriate medical treatment available. Discontinue immediately if anaphylaxis or severe hypersensitivity reactions occur. Pregnancy. Nursing mothers.
Do not mix with other drugs.
Hydrolytic lysosomal N-terminal tripeptidyl peptidase.
Pyrexia, ECG abnormalities, CSF protein increase/decrease, vomiting, seizures, hypersensitivity, hematoma, headache, irritability, pleocytosis, device-related infection, bradycardia, feeling jittery, hypotension; cardiovascular events.
Single-dose vials (5mL)—2 (w. Intraventricular Electrolytes 5mL vial) + Administration Kit—1 (infusion supplies)
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