CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Brineura (cerliponase alfa) (Intraventricular) — Coverage Criteria

Policy governs coverage, dosing, and authorization requirements for Brineura (cerliponase alfa) administered intraventricularly for treatment of late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) for Medicaid, Commercial, and Medicare members of Neighborhood Health Plan of Rhode Island.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyBrineura (cerliponase alfa) (Intraventricular) — Coverage Criteria

Policy CodePolicy N/A

Change TypeNo material change

Effective DateJan 1, 2020

Next Review Date

Key ActionObtain prior authorization showing definitive CLN2 diagnosis, required Hamburg CLN2 motor and language scores, and documentation of ambulatory status.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsInitial length of authorization

300Max units per dose

14 daysDosing interval

J0567HCPCS code

E75.4Covered diagnosis

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Coverage Criteria for Brineura (cerliponase alfa)

Initial Therapy

Covered when ALL of the following are met

Universal safety and baseline criteria: Patient is at least 37 weeks post‑menstrual age and weighs at least 2.5 kg; no acute intraventricular access device‑related complications (e.g., leakage, extravasation, device failure); no ventriculoperitoneal shunt; no signs or symptoms of acute unresolved localized infection at the device insertion site (e.g., cellulitis, abscess) and no suspected or confirmed CNS infection (e.g., cloudy CSF, positive CSF gram stain, or meningitis); device reservoir implanted prior to first infusion with first dose preferably 5–7 days after implantation.

ECG monitoring required during each infusion for patients with history of bradycardia, conduction disorder, or structural heart disease.

Diagnostic confirmation: Definitive diagnosis of CLN2 confirmed by deficiency of the lysosomal enzyme tripeptidyl peptidase‑1 (TPP1) and/or molecular analysis showing two pathogenic variants in the TPP1/CLN2 gene.

Disease severity and functional status: Hamburg CLN2 Clinical Rating Scale combined motor and language domain score 3 to 6 with at least a score of 1 in each domain; patient is ambulatory.Motor + Language score between 3 and 6; at least 1 in each domain

Renewal

Covered when ALL of the following are met for renewal

Ongoing eligibility: Patient continues to meet universal and indication‑specific criteria (including concomitant therapy/performance status as applicable); absence of unacceptable toxicity or device complications (examples include meningitis or other intraventricular access device‑related infections, device complications, severe hypersensitivity/anaphylaxis, severe cardiovascular reactions, infusion‑associated reactions such as vomiting, seizure, rash, or pyrexia); 12‑lead ECG performed within the last 6 months (and ECG during each infusion for patients with cardiac abnormalities such as bradycardia, conduction disorder, or structural heart disease).

Demonstrated clinical response or stability: Patient has responded to therapy compared to pretreatment baseline with stability or lack of decline in motor function/milestones on the Motor domain of the Hamburg CLN2 Clinical Rating Scale. Decline is defined as an unreversed sustained 2‑category decline or an unreversed score of 0.No sustained 2‑category decline or unreversed score of 0 on Motor domain

Coverage under the universal safety criteria expressly excludes patients with ventriculoperitoneal shunts. Requests for Brineura (cerliponase alfa) must confirm the absence of a ventriculoperitoneal shunt to meet initial eligibility.

Continuation of therapy is not medically necessary if the patient experiences unacceptable toxicity or device complications. Examples include meningitis or other intraventricular access device–related infections, intraventricular access device–related complications, severe hypersensitivity or anaphylaxis, severe cardiovascular reactions, or infusion-associated reactions (for example, vomiting, seizure, rash, or pyrexia).

Renewal also requires ongoing compliance with universal device-safety criteria (for example, no acute intraventricular access device complications and no signs of localized device-site infection); presence of these safety issues at renewal may justify denial as not medically necessary.

Billing and Coding

Covered HCPCS CodesHCPCSCovered

J0567

Injection, cerliponase alfa, 1 mg: 1 billable unit = 1 mg

NDCNDC

68135-0811-xx

Brineura 150 mg/5 mL (30 mg/mL) solution, two single-dose vials per carton co-packaged with Intraventricular Electrolytes Injection 5 mL in a single-dose vial

Covered Diagnosis CodesICD-10Covered

E75.4

Neuronal ceroid lipofuscinosis

Maximum HCPCS units per dose

Maximum billable units per dose300 billable units (one kit containing 2 vials)

HCPCS billing unit definitionJ0567 - Injection, cerliponase alfa, 1 mg: 1 billable unit = 1 mg

Dosing intervalEvery 14 days (once every other week)

Dose by age example≥ 2 years: 300 mg (10 mL) once every other week

Provider Requirements and Authorization

Prior Authorization

Prior Authorization Required

Prior authorization is required before initiation of therapy. Requests must include documentation supporting the diagnosis and clinical criteria below. Medicare members who have received the medication within the past 365 days are exempt from step therapy requirements but still require prior authorization.

Prior authorization required for all members
Medicare members treated within past 365 days exempt from step therapy

Step Therapy

Step Therapy Exception

Medicare members previously treated with this medication within the past 365 days are not subject to step therapy requirements. Documented prior receipt within 365 days should be included with the authorization request to support the exception.

Background

Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) is caused by deficiency of the lysosomal enzyme tripeptidyl peptidase-1 (TPP1) and leads to progressive motor and language decline and seizures in children. Brineura (cerliponase alfa) is an intraventricular enzyme replacement therapy indicated to slow loss of ambulation and decline in motor and language function in pediatric patients with CLN2 disease.

Definitions and Diagnostic Criteria

Definitive diagnosis of CLN2

Definitive diagnostic methodsConfirmed by deficiency of TPP1 enzyme and/or molecular analysis showing two pathogenic variants in the TPP1/CLN2 gene

Genetic evidence detailMolecular analysis demonstrating two pathogenic variants/mutations in the TPP1/CLN2 gene on chromosome 11p15

Indication contextDiagnosis requirement supports FDA-labeled indication for CLN2 (TPP1 deficiency)

Hamburg CLN2 Clinical Rating Scale requirement

Required Hamburg CLN2 scoreCombined motor and language domain score of 3 to 6

Minimum per-domain requirementAt least a score of 1 in each of the motor and language domains