Product Details

Brineura

Cerliponase alfa
150 mg/5 mL
Solution for Intracerebroventricular Infusion
5-mL Vial x 2's Pack

DIN/PIN/NPN

02484013

Manufacturer

BioMarin International Ltd.

Formulary Listing Date

2019-11-11  

Unit Price

100.6667

Amount MOH Pays

100.6667

Coverage Status

Exceptional Access Program Product

ODB Formulary Therapeutic Classification

Therapeutic Note

NO

ATC Code

A16AB17

Interchangeable Products

NO  

LU Clinical Criteria

NO  

EAP Criteria

Therapeutic Class Reimbursement Criteria
Metabolic/Genetic Modifiers

Cerliponase alfa

  • Brand(s): Brineura
  • Dosage Form/Strength: 150mg/5mL (30mg/mL) Injection
  • Updated December 2, 2020

Initiation Criteria: 

For the treatment of Neuronal Ceroid Lipofuscinosis Type 2 (CLN2) disease/ tripeptidyl peptidase 1 (TPP1) deficiency, in patients who meet the following criteria at the time of treatment initiation:

  1. Diagnosis of CLN2 disease is confirmed by TTP1 enzyme activity and CLN2 genotype analysis; AND

  2. CLN2 Rating Scale demonstrates the following requirements:
    •     A minimum score greater than or the same as 1 in each of the motor and the language domains; AND
    •      A minimum score greater than or the same as 3 for the aggregate motor- language score

    AND

  3. Patient is under the care of a prescriber/specialist with expertise in the diagnosis and management of CLN2 disease. 

Approval duration of initials: 24 weeks 

Renewal Criteria:

Renewal of funding will be considered for patients who do not meet any of the exclusion criteria and who have not demonstrated any of the stopping/discontinuation criteria while on therapy. 

Exclusion Criteria (Applies to both initiation and renewal criteria): 

Patients meeting any of the following criteria will not be funded: 

  • Requires ventilation support (except for non-invasive support at night); 

  • Presence of contraindications to intracerebroventricular (ICV) drug administration; 

  • Presence of acute ICV device complications; 

  • Presence of ventriculoperitoneal (VPS) shunts

Stopping/Discontinuation Criteria for Brineura:

Treatment with Brineura will be discontinued if: 

  • There is reduction of 2 or more points in the aggregate motor-language score of the CLN2 Clinical Rating Scale that is maintained over any 2 consecutive 24-week assessments; OR 

  • The aggregate motor-language score of the CLN2 Clinical Rating Scale reaches 0 (zero) at 2 consecutive 24-week assessments; OR 

  • The patient has developed contraindication to ICV drug administrations

Recommended dose:
300 mg (10 mL solution) administered by intracerebroventricular (ICV) infusion once every 2 weeks. 

Approval duration of renewals: 12 months

EAP Drug Request Form:

Standard Form for EAP Drug Requests

Product Monograph

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