CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Zokinvy (lonafarnib) coverage for progeria and progeroid laminopathies

Defines coverage criteria, initial and continuation approval requirements, quantity limits, approval durations, and prescribing/documentation requirements for Zokinvy (lonafarnib) for Medicaid members beginning 2021-07-01.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyZokinvy (lonafarnib) coverage for progeria and progeroid laminopathies

Policy CodePolicy N/A

Change TypeNo material change

Effective DateJul 1, 2021

Next Review Date

Key ActionAuthorization requests must include genetic testing results confirming diagnosis and documentation that Zokinvy is prescribed by or in consultation with a progeria/genetics/metabolic disorders specialist; dose must not exceed FDA-recommended regimen.

SourceLink

POLICY UPDATE CHANGES

No material change to clinical coverage

3FDA-Approved / Covered Indications listed

6 monthsInitial approval duration

6 monthsRenewal approval duration

120 caps/30dQuantity limit examples

Coverage Summary

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Initial Therapy Criteria & Operational Requirements

Prior Authorization

Prior authorization required with documentation

Prior authorization is required. Authorization requests must include genetic testing results confirming the eligible diagnosis, documentation that Zokinvy is prescribed by or in consultation with a progeria/genetics/metabolic disorders specialist, and dosing consistent with the FDA-recommended regimen (initial 115 mg/m2 twice daily, increased to 150 mg/m2 twice daily after 4 months).

Genetic test results confirming LMNA or ZMPSTE24 mutation
Specialist name and consult note (progeria, genetics, or metabolic disorders)
Requested dose aligns with FDA dosing (115 mg/m2 BID, escalate to 150 mg/m2 BID after 4 months)

Surface thresholds and key parameters

Body Surface Area>= 0.39 m^2

FDA recommended dosing115 mg/m^2 twice daily initially; increase to 150 mg/m^2 twice daily after 4 months

Initial approval duration6 months

Quantity-limit example120 capsules / 30 days

Continuation Therapy Criteria

Documentation Required

Continuation documentation of benefit

For renewals, the prescriber must document that the member is tolerating therapy and has experienced a positive clinical response to treatment.

No new or worsening heart failure
No stroke incidence
Reduction in seizures

Ineligible Indications / Exclusions

Zokinvy is not indicated for other progeroid syndromes or for processing-proficient progeroid laminopathies; use in those populations is considered experimental/investigational and not medically necessary.

Denial Risk

Ineligible indications excluded

Requests for Zokinvy for other progeroid syndromes or for processing‑proficient progeroid laminopathies are considered experimental/investigational and will be denied as not medically necessary. Genetic testing that distinguishes the eligible diagnoses (e.g., LMNA or ZMPSTE24 mutations) must be provided with the request.

Provider Actions & Documentation Requirements

Documentation Required

Document specialist involvement and genetic confirmation

Provide genetic testing results confirming LMNA or ZMPSTE24 mutations and documentation that Zokinvy is prescribed by or in consultation with an appropriate specialist (progeria, genetics, or metabolic disorders) with the authorization request.

Prior Authorization

Prior authorization required with documentation

Prior authorization is required. Authorization requests must include genetic testing results confirming the eligible diagnosis, documentation of specialist prescriber or consult, and the requested dose must not exceed the FDA‑recommended regimen (115 mg/m2 twice daily, increased to 150 mg/m2 twice daily after 4 months).

Background

Zokinvy (lonafarnib) is a farnesyltransferase inhibitor indicated to reduce risk of mortality in Hutchinson-Gilford Progeria Syndrome (patients 12 months of age and older with body surface area ≥ 0.39 m2) and to treat certain processing-deficient progeroid laminopathies (heterozygous LMNA mutations with progerin-like protein accumulation or homozygous/compound heterozygous ZMPSTE24 mutations). It is not indicated for other progeroid syndromes or processing-proficient progeroid laminopathies.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyZokinvy (lonafarnib) coverage for progeria and progeroid laminopathies

Policy CodePolicy N/A

Change TypeNo material change

Effective DateJul 1, 2021

Next Review Date

SourceLink

On This Page

OpenPayer

Zokinvy (lonafarnib) coverage for progeria and progeroid laminopathies

Coverage Summary

Initial Therapy Criteria & Operational Requirements

Continuation Therapy Criteria

Ineligible Indications / Exclusions

Applicable Codes

Provider Actions & Documentation Requirements

Clinical Evidence & References

Background

Revision History