Cerliponase alfa (Brineura) is a hydrolytic lysosomal N-terminal tripeptidyl peptidase indicated to slow the loss of ambulation in symptomatic individuals three years of age and older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency. CLN2 disease is one of a group of disorders known as neuronal ceroid lipofuscinoses, collectively called Battens disease. Deficiency in TPP1 activity results in the accumulation of lysosomal storage materials normally metabolized by this enzyme in the CNS, leading to progressive decline in motor function. Cerliponase alfa (Brineura), a proenzyme, is activated in the lysosome and cleaves tripeptides from the N-terminus of proteins.
Coverage is subject to the specific terms of the member's benefit plan.
Federal Employee Program members (FEP) should check with their Retail Pharmacy Program to determine if prior approval is required by calling the Retail Pharmacy Program at 1-800-624-5060 (TTY: 1-800-624-5077). FEP members can also obtain the list through the www.fepblue.org website.
Cerliponase alfa (Brineura) may be considered medically necessary to slow the loss of ambulation in individuals with late infantile CLN2 who meet ALL of the following:
Continuation of treatment with cerliponase alfa (Brineura) after initiation of therapy, may be considered medically necessary to slow the loss of ambulation in individuals late infantile CLN2 who meet the following:
Cerliponase alfa (Brineura) is considered experimental/investigational for any other indication and therefore non-covered. Scientific evidence does not support its use for any other indications.
NOTE: In addition to the above criteria, product specific dosage and/or frequency limits may apply in accordance with the U.S. Food and Drug Administration (FDA)-approved product prescribing information, national compendia, Centers for Medicare and Medicaid Services (CMS) and other peer reviewed resources or evidence-based guidelines. Blue Cross Blue Shield of North Dakota may deny, in full or in part, reimbursement for utilization that does not fall within the applicable dosage and/or frequency limits.