Zokinvy (lonafarnib) — Highmark

Initial criteria

• age ≥ 12 months
• diagnosis of processing-deficient PL
• one of the following: (a) documentation showing heterozygous LMNA mutation and progerin-like protein accumulation OR (b) documentation showing homozygous or compound heterozygous ZMPSTE24 mutations
• body surface area (BSA) ≥ 0.39 m2
• requested dosing regimen aligns with FDA-approved labeled dosing regimen (refer to Table 1)

Reauthorization criteria

• age ≥ 12 months
• diagnosis of processing-deficient PL
• one of the following: (a) documentation showing heterozygous LMNA mutation and progerin-like protein accumulation OR (b) documentation showing homozygous or compound heterozygous ZMPSTE24 mutations
• body surface area (BSA) ≥ 0.39 m2
• requested dosing regimen aligns with FDA-approved labeled dosing regimen (refer to Table 2)

Approval duration

initial up to 4 months; maintenance up to 12 months