ZOKINVY (lonafarnib) 

SELF ADMINISTRATION

Indication for Prior Authorization:

Indicated in patients 12 months of age and older with a body surface area (BSA) of 0.39 m2 and above:

  • To reduce the risk of mortality in Hutchinson-Gilford Progeria Syndrome (HGPS)
  • For the treatment of processing-deficient Progeroid Laminopathies with either:
    • Heterozygous LMNA mutation with progerin-like protein accumulation
    • Homozygous or compound heterozygous ZMPSTE24 mutations
Limitation of Use:
  • Zokinvy is not indicated for other Progeroid Syndromes or processing-proficient Progeroid Laminopathies.  Based upon its mechanism of action, Zokinvy would not be expected to be effective in these populations
Coverage Criteria:
  • Patient is 12 months of age and older, AND
  • Prescribed by or in consultation with a specialist in progeria, genetics, and/or metabolic disorders, AND
  • Patient has a body surface area of 0.39 m2 and above, AND
  • One of the following:
    • Diagnosis of Hutchinson-Gilford Progeria Syndrome (HGPS), OR
    • For the treatment of processing-deficient Progeroid Laminopathies with one of the following:
      • Heterozygous LMNA mutation with progerin-like protein accumulation, OR
      • Homozygous or compound heterozygous ZMPSTE24 mutations
Dosing:
  • Starting dose for patients with a BSA of 0.39 m2 and above is 115 mg/m2 twice daily with morning and evening meals to reduce the risk of gastrointestinal adverse reactions
    • Please note, an appropriate dosage strength of Zokinvy is not available for patients with a BSA of less than 0.39 m2
  • After 4 months of treatment, increase the dosage to 150 mg/m2 twice daily with morning and evening meals
  • Round all total daily dosages to the nearest 25 mg increments
  • Please refer to the package insert for recommended dosing and administration based on BSA
Coverage Duration:
  • 1 year
Authorization is Not Covered for the Following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics Committee.

Additional Information:
  • Contraindications:
    • Strong or moderate CYP3A inhibitors or inducers
    • Midazolam
    • Lovastatin, simvastatin, and atorvastatin
Review History:
  • 2/18/2021- Original review
References:
  • Zokinvy Prescribing Information. Eiger BioPharmaceuticals, Inc. Palo Alto, CA. November 2020.

 

Last review date: April 20, 2021