CurrentAvMedPolicy N/A

Zokinvy

Prior authorization/step-edit form and clinical criteria for coverage of Zokinvy (lonafarnib) including initial and reauthorization requirements, dosing limits based on BSA, contraindicated concomitant therapies, diagnostic confirmation, and specialty pharmacy dispensing.

Policy Summary

PayerAvMed

PolicyZokinvy

Policy CodePolicy N/A

Change TypeNo material change

Effective Date

Next Review Date

Key ActionPrescribing physician must sign and print name on request and fax completed prior authorization with required documentation to 1-305-671-0200.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes were reported for this policy.

2Permitted diagnosis groups

12Initial authorization (months)

12Reauthorization (months)

>= 0.39 m2Minimum BSA

<= 300 mg/m2/dayMax maintenance dose

Coverage Summary

Trek Health ingests and normalizes Transparency in Coverage data and payer policy updates to give provider organizations a clear view of how commercial reimbursement behaves across markets, payers, and services. Our platform transforms raw payer disclosures into structured intelligence that supports contract evaluation, payer negotiations, and service line strategy. By combining market benchmarks with ongoing policy visibility, Trek helps teams identify variability, risk, and opportunity in commercial reimbursement. The result is faster insight, stronger negotiating positions, and more informed financial decisions.

This is a prior authorization/step-edit form for Zokinvy (lonafarnib) used to request coverage. Coverage is covered with criteria and requires completion of the form and supporting documentation. Initial authorizations are for 12 months and reauthorizations are for 12 months. Dosing limits and durations are based on body surface area (BSA) with BSA-based initial and maintenance dosing tables included on the form. The medication is dispensed via specialty pharmacy (PropriumRx); verify plan formulary status and document medical necessity if non-formulary.

Key thresholds & quick facts

Minimum age>= 12 months

Minimum BSA>= 0.39 m2

Initial authorization duration12 months

Reauthorization duration12 months

Initial dosing (total daily)230 mg/m2/day (115 mg/m2 twice daily) for first 4 months; doses rounded to nearest 25 mg

Maximum maintenance dose300 mg/m2/day (150 mg/m2 twice daily); doses rounded to nearest 25 mg

Initial Therapy Criteria (12 months)

Prior Authorization

Submit prior authorization request

Prescribing physician must sign and clearly print name (preprinted stamps not valid) on the prior authorization request. Complete member, prescriber, and drug information must be provided. Fax the completed form to 1-305-671-0200. Note: if the information provided is not complete, correct, or legible, the authorization process can be delayed.

Documentation Required

Provide diagnostic confirmation and clinical documentation

Include documentation of confirmatory mutation analysis or genetic test results for LMNA G608G (c.1824C>T) or processing-deficient laminopathies. Provide clinical features supportive of progeria, body surface area (BSA), relevant laboratory results, imaging (e.g., X‑ray/cardiac findings), and chart notes. Prior therapies should be verified via pharmacy paid claims or submitted chart notes.

Mutation analysis or genetic test results (e.g., LMNA G608G c.1824C>T or ZMPSTE24 variants)
Clinical features of progeria (e.g., characteristic facial appearance, sclerodermatous skin changes, alopecia, decreased joint ROM)
Body surface area (BSA)
Laboratory results
Imaging/radiographic findings (e.g., distal clavicular/phalangeal resorption, coxa valga, cardiac disease)
Chart notes supporting diagnosis and clinical status
Verification of prior therapies via pharmacy claims or chart notes

Continuation / Reauthorization Criteria (12 months)

Denial Risk

Risk of denial for inadequate documentation

Requests may be denied if required documentation is not provided. Ensure all required documentation, laboratory results, diagnostics, and chart notes are submitted to support each checked criterion to avoid denial.

Dosing Limits (BSA-based)

Dosing limits (BSA-based)

Dose does not exceed one of the following depending on phase: initial (first 4 months) and maintenance after 4 months; doses rounded to nearest 25 mg.

Dosing phase statements

Initial dosing (first 4 months): Initial: 115 mg/m2 twice daily (total daily 230 mg/m2) for 4 months; total daily dosage rounded to nearest 25 mgInitial total daily = 230 mg/m2/day
Maintenance dosing (after 4 months): Maintenance after 4 months: 150 mg/m2 twice daily (total daily 300 mg/m2); total daily dosage rounded to nearest 25 mgMaintenance total daily = 300 mg/m2/day
Rounding note: Doses rounded to the nearest 25 mg
BSA-to-dose examples: Dose tables provided mapping BSA ranges to total daily rounded doses and capsule counts (examples include: BSA 0.39-0.48 -> 100 mg total daily; 0.49-0.59 -> 125 mg; 0.6-0.7 -> 150 mg; up to 0.96-1.0 -> 225 mg initial; maintenance ranges up to 300 mg total daily for BSA 0.96-1.0)

The form includes dose tables that map specific BSA ranges to total daily rounded doses and the corresponding capsule counts for both the initial and maintenance phases. Refer to the authorization form for the full BSA ranges, exact rounded total daily doses and capsule examples; do not recreate the full table here.

Concomitant Medication Management

Concomitant therapy avoidance and interaction concerns are specified. The form lists midazolam and HMG-CoA reductase inhibitors (examples: lovastatin, simvastatin, atorvastatin) as contraindicated or to be avoided. It also specifies avoidance of strong or moderate CYP3A inhibitors (examples: fluconazole, itraconazole, etc.) and strong or moderate CYP3A inducers (examples: rifampin, carbamazepine, St. John's Wort, etc.). Additionally, strong or moderate CYP2C9 inhibitors are listed (examples: voriconazole, metronidazole, fluvastatin, sulfamethoxazole, etc.). If concomitant therapy is unavoidable, close monitoring and dose modifications must be implemented.

Documentation Required

Concomitant medication management

Avoid concomitant therapy with the listed interacting drugs (midazolam; HMG‑CoA reductase inhibitors such as lovastatin, simvastatin, atorvastatin; strong or moderate CYP3A inhibitors/inducers; strong or moderate CYP2C9 inhibitors). If concomitant therapy is unavoidable, monitor the member closely for adverse reactions and implement dose modifications as needed.

Avoid: midazolam
Avoid: HMG‑CoA reductase inhibitors (e.g., lovastatin, simvastatin, atorvastatin)
Avoid: strong/moderate CYP3A inhibitors (e.g., fluconazole, itraconazole) or inducers (e.g., rifampin, carbamazepine, St. John's Wort)
Avoid: strong/moderate CYP2C9 inhibitors (e.g., voriconazole, metronidazole, fluvastatin, sulfamethoxazole)
If unavoidable: close monitoring and dose modifications

Provider Actions & Administrative Requirements

Billing Rule

Specialty pharmacy dispensing

This medication is dispensed by the specialty pharmacy PropriumRx. Verify the patient's plan formulary status; if the drug is non‑formulary on the plan, document medical necessity.

Prior Authorization

Submit prior authorization and required documentation

Prescribing physician must sign and clearly print name on the prior authorization request (preprinted stamps not valid). Ensure the form includes complete member, prescriber, and drug information. Fax the completed request and supporting documentation (mutation testing, clinical features, BSA, labs, imaging, chart notes) to 1-305-671-0200. Incomplete, incorrect, or illegible information may delay or jeopardize authorization.

Background & Definitions

This form-based prior authorization is specifically for lonafarnib (Zokinvy) for treatment of Hutchinson-Gilford progeria syndrome (HGPS) and processing-deficient progeroid laminopathies. The request must meet clinical criteria and include diagnostic confirmation (e.g., LMNA G608G mutation or genetic testing for processing-deficient laminopathies). The form provides BSA-based dosing tables for initial and maintenance phases, identifies contraindicated or interacting concomitant medications that must be avoided or monitored, and requires documentation such as genetic test results, clinical features, BSA, labs, imaging and chart notes to support approval.

Definitions

HGPSHutchinson-Gilford progeria syndrome (HGPS)

BSABody surface area (m2) used for BSA-based dosing