Criteria
Coverage is subject to the specific terms of the member's benefit plan.
The use of cerliponase alfa (Brineura) may be considered medically necessary when
ALL
of the following criteria are met:
-
The individual must be between three (3) and eight (8) years of age;
and
-
The member must meet FDA-approved label for use (e.g., use outside of studied population will be considered investigational);
and
-
Cerliponase alfa (Brineura) must be prescribed by, or in consult with, a metabolic specialist, geneticist, or pediatric neurologist;
and
-
Documentation of the diagnosis must be submitted, as evidenced by the following:
-
Molecular analysis that has detected two pathogenic variants/mutations in the TPP1/CLN2 gene;
and
-
An enzyme assay confirming deficiency of tripeptidyl peptidase 1 (TPP1);
and
-
The individual must not have ventriculoperitoneal shunts;
and
-
Baseline results of motor and language domains of the Hamburg CLN2 Clinical Rating Scale must be submitted and meet the following parameters:
-
Results must show a combined score of less than six (6) in the motor and language domains;
and
- Results must show a score of at least one (1) in each of these domains.
Initial Authorization: Six (6) months
Reauthorization Criteria
Continuation of therapy with cerliponase alfa (Brineura) may be considered medically necessary when
ALL
of the following are met:
-
The individual must not have acute, unresolved localized infection on or around the device insertion site or suspected or confirmed CNS infection;
and
-
The individual maintains at a score of at least one (1) in the motor domain on the Hamburg CLN2 Clinical Rating Scale;
and
- The individual has responded to therapy compared to pretreatment baseline with stability/lack of decline* in motor function/milestones.
*Decline is defined as having an unreversed (sustained) 2-category decline or an unreversed score of 0 in the Motor domain of the CLN2 Clinical Rating Scale
Cerliponase alfa (Brineura) for all other indications not listed in this policy is considered experimental/investigational and therefore non-covered because the safety and/or effectiveness cannot be established by the available published peer-reviewed literature.
Procedure Code