Miscellaneous neurodegenerative disorders:
Indications for BRINEURA:
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. 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. See full labeling.
Any sign or symptom of acute, unresolved localized infection on or around the device insertion site (eg, cellulitis, abscess). Suspected or confirmed CNS infection (eg, meningitis, cloudy CSF, positive CSF gram stain). Patients with acute intraventricular access device-related complications (eg, leakage, fluid extravasation, device failure) 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. Monitor for meningitis and other device-related infections; obtain CSF sample for cell count and culture prior to each infusion. Have appropriate medical support readily available. Discontinue immediately if anaphylaxis or severe hypersensitivity reactions occur; consider risks/benefits prior to readministration. 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. Pregnancy. Nursing mothers.
Hydrolytic lysosomal N-terminal tripeptidyl peptidase.
Do not mix with other drugs.
Pyrexia, ECG abnormalities, CSF protein increase/decrease, vomiting, seizures, hypersensitivity, hematoma, headache, irritability, pleocytosis, device-related infection, bradycardia, feeling jittery, hypotension; meningitis, cardiovascular events.
Single-dose vials (5mL)—2 (w. Intraventricular Electrolytes 5mL vial) + Administration Kit—1 (infusion supplies)