CurrentWashington State Health Care AuthorityPolicy 30.90.90-1

Brineura (cerliponase alfa) coverage criteria

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This policy governs medical necessity and prior authorization for Brineura in pediatric patients with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 deficiency.

Policy Summary

PayerWashington State Health Care Authority

PolicyBrineura (cerliponase alfa) coverage criteria

Policy CodePolicy 30.90.90-1

Change TypeNew policy

Effective DateJul 1, 2019

Last ReviewMay 6, 2019

Next Review DateNot specified

Key ActionSubmit prior authorization with documentation supporting the initial or reauthorization criteria.

SourceLink

POLICY UPDATE CHANGES

The policy history identifies this document as a new policy issued on May 6, 2019.

≥3 yearsMinimum age

300 mg q2wDose limit

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CurrentWashington State Health Care AuthorityPolicy 30.90.90-1

Brineura (cerliponase alfa) coverage criteria

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Sign up for Washington State Health Care Authority Policy 30.90.90-1 alerts

Get alerted when Policy 30.90.90-1 changes without checking for updates manually.

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Policy Summary

PayerWashington State Health Care Authority

PolicyBrineura (cerliponase alfa) coverage criteria

Policy CodePolicy 30.90.90-1

Change TypeNew policy

Effective DateJul 1, 2019

Last ReviewMay 6, 2019

Next Review DateNot specified

Key ActionSubmit prior authorization with documentation supporting the initial or reauthorization criteria.

SourceLink

POLICY UPDATE CHANGES

The policy history identifies this document as a new policy issued on May 6, 2019.

≥3 yearsMinimum age

300 mg q2wDose limit

6 moInitial approval

12 moReauthorization

Medical Necessity and Coverage Criteria

Medical Necessity Criteria

Brineura may be considered medically necessary when used to slow the loss of ambulation in symptomatic pediatric patients age 3 years or older with CLN2/TPP1 deficiency.

Medical necessity: Cerliponase alfa is used to slow the loss of ambulation in a symptomatic pediatric patient age 3 years or older with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 (TPP1) deficiency.

Initial Therapy Criteria

Covered for initial approval when ALL criteria are met.

Initial therapy: Patient is 3 years of age or older.>= 3 years

Initial therapy: Patient has a documented diagnosis of late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) confirmed by TPP1 deficiency and genetic testing showing mutation of the TPP1 gene on chromosome 11p15.

Initial therapy: Medication is prescribed by or in consultation with a specialist with expertise in treating CLN2, such as a pediatric neurologist, pediatric epileptologist, or geneticist.

Initial therapy: Patient is ambulatory.

Initial therapy: Baseline CLN2 Clinical Rating Scale documentation shows a score of at least 1 in the motor domain and at least 1 in the language domain.motor >= 1 and language >= 1

Initial therapy: There are no acute intraventricular access device-related complications, such as leakage, device failure, or device-related infection, and the patient does not have a ventriculoperitoneal shunt.

Continuation Therapy Criteria

Covered for reauthorization when ALL criteria are met.

Continuation therapy: Documentation demonstrates positive clinical improvement, such as no decline in the CLN2 Clinical Rating Scale with a score of at least 1 in both the motor and language domains.motor >= 1 and language >= 1 with no decline

Continuation therapy: Medication is prescribed by or in consultation with a specialist with expertise in treating CLN2, such as a pediatric neurologist, pediatric epileptologist, or geneticist.

Continuation therapy: Patient is ambulatory.

Continuation therapy: There are no acute intraventricular access device-related complications, such as leakage, device failure, or device-related infection, and the patient does not have a ventriculoperitoneal shunt.

Coverage requires documentation that the patient has no acute intraventricular access device-related complications, including leakage, device failure, or device-related infection, and does not have a ventriculoperitoneal shunt. These circumstances may prevent approval or reauthorization.

Billing and Clinical Parameters

Covered HCPCS CodesHCPCSCovered

J0567

Injection, cerliponase alfa, 1 mg

Patient age

Minimum age3 years or older

Applies toInitial approval

Patient populationPediatric patients with late infantile neuronal ceroid lipofuscinosis type 2 (CLN2)

Baseline motor score

Required baseline motor scoreAt least 1

AssessmentCLN2 Clinical Rating Scale motor domain

DocumentationBaseline CLN2 Clinical Rating Scale score must be documented

Prior Authorization and Submission Requirements

Prior Authorization

Initial and Continued Authorization Required

Prior authorization is required for initial treatment with Brineura. If all initial clinical criteria are met, authorization is approved for 6 months; continued treatment requires reauthorization, with approval for 12 months when all reauthorization criteria are met.

Initial approval requires fulfillment of all applicable clinical criteria.
Reauthorization requires fulfillment of all continuation criteria.
Initial authorization duration: 6 months.
Reauthorization duration: 12 months.

Documentation Required

Required Clinical Documentation

Submit documentation supporting diagnosis, eligibility, specialist involvement, treatment status, and device safety. Initial requests must document that the patient is at least 3 years old; has genetically confirmed CLN2 with TPP1 deficiency and a TPP1 gene mutation on chromosome 11p15; is ambulatory; and has a baseline CLN2 Clinical Rating Scale score of at least 1 in both the motor and language domains.

Condition and Treatment Overview

Late infantile neuronal ceroid lipofuscinosis type 2 (CLN2), also known as tripeptidyl peptidase 1 deficiency, is a nervous system disorder caused by insufficient tripeptidyl peptidase 1 activity. The condition causes progressive loss of motor function and may include delayed speech, loss of developmental milestones, visual impairment, cerebral atrophy, and seizures. Cerliponase alfa (Brineura) is an enzyme replacement therapy intended to slow loss of the ability to walk or crawl in symptomatic pediatric patients age 3 years or older.

Clinical Rating Scale Definitions

CLN2 Clinical Rating Scale

PurposeScale used to calculate the patient's degree of disease severity

DomainsMotor and language

Use in coverage assessmentDocuments baseline motor and language scores for CLN2 treatment authorization

Motor domain

Score 3Grossly normal gait

Score 2Abnormal gait with independent walking of at least 10 steps and frequent falls or obvious clumsiness

Score 1No unaided walking or crawling only; cannot walk 10 unassisted steps

Initial Approval

Initial Approval — 6 Months

Initial approval is granted for 6 months when ALL criteria are met.

Initial approval: Patient is 3 years of age or older.>= 3 years

Initial approval: Patient has a documented diagnosis of late infantile neuronal ceroid lipofuscinosis type 2 (CLN2) confirmed by TPP1 deficiency and genetic testing showing mutation of the TPP1 gene on chromosome 11p15.

Initial approval: Medication is prescribed by or in consultation with a specialist with expertise in treating CLN2, such as a pediatric neurologist, pediatric epileptologist, or geneticist.

Initial approval:

Reauthorization

Reauthorization — 12 Months

Reauthorization is approved for 12 months when ALL criteria are met.

Reauthorization: Documentation demonstrates positive clinical improvement, such as no decline in the CLN2 Clinical Rating Scale with a score of at least 1 in both the motor and language domains.motor >= 1 and language >= 1 with no decline

Reauthorization: Medication is prescribed by or in consultation with a specialist with expertise in treating CLN2, such as a pediatric neurologist, pediatric epileptologist, or geneticist.

Reauthorization: Patient is ambulatory.

Reauthorization:

Brineura Dose and Frequency Limit

Brineura (cerliponase alfa)

Maximum covered dose300 mg

Administration routeIntravenous (IV)

Administration frequencyEvery other week

Policy Summary

PayerWashington State Health Care Authority

PolicyBrineura (cerliponase alfa) coverage criteria

Policy CodePolicy 30.90.90-1

Change TypeNew policy

Effective DateJul 1, 2019

Last ReviewMay 6, 2019

Next Review DateNot specified

Key ActionSubmit prior authorization with documentation supporting the initial or reauthorization criteria.

SourceLink

On This Page

Baseline language score

Required baseline language scoreAt least 1

AssessmentCLN2 Clinical Rating Scale language domain

DocumentationBaseline CLN2 Clinical Rating Scale score must be documented

Brineura dose

Dose300 mg

RouteIntravenous (IV)

FrequencyEvery other week

Document that Brineura is prescribed by or provided in consultation with a specialist experienced in treating CLN2, such as a pediatric neurologist, pediatric epileptologist, or geneticist.
Document no acute intraventricular access device-related complications, including leakage, device failure, or device-related infection, and no ventriculoperitoneal shunt.
For reauthorization, document positive clinical improvement, such as no decline in the CLN2 Clinical Rating Scale with a score of at least 1 in both the motor and language domains.
For reauthorization, resubmit documentation of specialist prescribing or consultation, continued ambulation, and absence of acute intraventricular access device-related complications or a ventriculoperitoneal shunt.

Denial Risk

Failure to Meet Clinical Criteria

The request will be denied when the applicable initial or reauthorization clinical criteria are not satisfied. Initial approval requires all initial criteria; continued approval requires all reauthorization criteria and documented positive clinical improvement.

Initial criteria include age 3 years or older, genetically confirmed CLN2 due to TPP1 deficiency, specialist involvement, ambulation, and qualifying baseline motor and language scores.
Reauthorization criteria include positive clinical improvement, specialist involvement, continued ambulation, and no acute intraventricular access device-related complications or ventriculoperitoneal shunt.
If all criteria are not met or required documentation is missing, authorization is not approved.

Billing Rule

Dose Exceeds Quantity Limit

Brineura is covered only within the authorized dose and frequency. Doses exceeding the quantity limit require clinical justification and authorization; otherwise, coverage may be denied.

Quantity limit: 300 mg IV every other week.
Do not exceed 300 mg per dose or administer more frequently than every other week without authorization.

Score 0Immobile, mostly bedridden

Language domain

Score 3Grossly normal, age-appropriate language

Score 2Language has become recognizably abnormal, worse than the individual's maximum

Score 1Hardly understandable

Score 0Unintelligible or no language

Patient is ambulatory.

Initial approval: Baseline CLN2 Clinical Rating Scale documentation shows a score of at least 1 in the motor domain and at least 1 in the language domain.motor >= 1 and language >= 1

Initial approval: There are no acute intraventricular access device-related complications, such as leakage, device failure, or device-related infection, and the patient does not have a ventriculoperitoneal shunt.

There are no acute intraventricular access device-related complications, such as leakage, device failure, or device-related infection, and the patient does not have a ventriculoperitoneal shunt.