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Cerliponase Alfa Dosage

Medically reviewed on August 31, 2017.

Applies to the following strengths: 150 mg/5 mL

Usual Pediatric Dose for Neuronal Ceroid Lipofuscinosis

3 years or older: 300 mg by intraventricular infusion once every other week

-Pretreatment with antihistamines with or without antipyretics or corticosteroids should occur 30 to 60 minutes prior to the start of infusion.
-This drug should be administered first, followed by the intraventricular electrolytes using the provided administration kit.

Use: To slow the loss of ambulation in symptomatic pediatric patients 3 years or older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available


Safety and efficacy have not been established in patients younger than 3 years.

Consult WARNINGS section for additional precautions.


Data not available

Other Comments

Administration advice:
-Administered into the cerebrospinal fluid (CSF) by intraventricular infusion via a surgically implanted reservoir and catheter (intraventricular access device).
-Must only be administered by the intraventricular route, using the provided administration kit; each vial of this drug and electrolytes is for single use only.
-Each infusion consists of 10 mL of this drug followed by 2 mL of intraventricular electrolytes. administered using an infusion set with a 0.2 micron inline filter.
-Infuse both this drug and electrolytes at an infusion rate of 2.5 mL/hr; complete infusion, including required electrolytes, is about 4.5 hours.
-The Codman(R) HOLTER RICKHAM Reservoirs should be used with the Codman(R) Ventricular Catheter.
-To be administered with the B Braun Perfusor(R) Space Infusion System; consult the manufacturer product information for additional information.
-The intraventricular access device must be implanted prior to the first infusion and the first dose administered at least 5 to 7 days after device implantation.
-Aseptic technique must be strictly enforced during preparation and administration.
-To be administered by, or under the direction of a physician knowledgeable in intraventricular administration.
-The manufacturer product information should be consulted for direction in the intraventricular infusion procedure.

Storage requirements:
-Store upright in a freezer (-25C to -15C [-13F to 5F]) in original carton to protect from light.
-Store the administration kit, including vials containing electrolytes, in original carton separately; do not freeze.

Reconstitution/preparation techniques:
-The manufacturer product information should be consulted.

-Cardiovascular: Monitor vital signs (e.g., blood pressure, heart rate) prior to infusion, periodically during infusion, and post-infusion in a healthcare setting. Perform ECG monitoring during infusion in patients with a history of bradycardia, conduction disorder, or with structural heart disease; regular 12-lead ECG evaluations should occur every 6 months in patients without cardiac abnormalities.
-Hypersensitivity: Observe patients closely during and after infusion for signs/symptoms off allergic reactions, including anaphylaxis.

Patient advice:
-Immediately contact your healthcare provider if any signs of infection occur.
-Immediately contact your healthcare provider if hypotension and/or bradycardia occur during or following infusion.
-Seek medical attention if any signs/symptoms of a hypersensitivity reaction (e.g., fever, vomiting, irritability) or signs/symptoms of anaphylaxis occur.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.