Omaveloxolone (RTA-408) Form

Omaveloxolone (Skyclarys™) is a nuclear factor erythroid 2–related factor 2 (Nrf2) pathway activator. FDA Approved Indication(s) Skyclarys is indicated for the treatment of Friedreich’s ataxia (FA) in adults and adolescents aged 16 years and older. Policy/Criteria Provider must submit documentation (such as office chart notes, lab results or other clinical information) supporting that member has met all approval criteria.
It is the policy of health plans affiliated with Centene Corporation® that Skyclarys is medically necessary when the following criteria are met:
I. Initial Approval Criteria A. Friedreich’s Ataxia (must meet all):

1. Diagnosis of FA;
   2. Documentation of genetic testing that shows a GAA triplet-repeat expansion in the frataxin (FXN) gene;
2. Prescribed by or in consultation with a neurologist;
   
   4. Age ≥ 16 years;
   5. Recent (within the last 30 days) baseline modified Functional Assessment Rating Scale (mFARS) score (see Appendix D);
3. Recent (within the last 30 days) baseline left ventricular ejection fraction ≥ 40%;
   7. Dose does not exceed both of the following (a and b):
      a. 150 mg per day;
      b. 3 capsules per day.
      Approval duration: 6 months
      B. Other diagnoses/indications (must meet 1 or 2):

4. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: Page 1 of 6

   CLINICAL POLICY Omaveloxolone CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

5. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid. II. Continued Therapy A. Friedreich’s Ataxia (must meet all):
6. Member meets one of the following (a or b): a. Currently receiving medication via Centene benefit or member has previously met initial approval criteria; b. Member is currently receiving medication and is enrolled in a state and product with continuity of care regulations (refer to state specific addendums for CC.PHARM.03A and CC.PHARM.03B);
7. Member is responding positively to therapy as evidenced by, including but not limited to, improvement or stabilization in any of the following parameters: FA symptoms (see Appendix D) or mFARS score;
8. If request is for a dose increase, new dose does not exceed both of the following (a and b):
   a. 150 mg per day; b. 3 capsules per day. Approval duration: 12 months B. Other diagnoses/indications (must meet 1 or 2):
9. If this drug has recently (within the last 6 months) undergone a label change (e.g., newly approved indication, age expansion, new dosing regimen) that is not yet reflected in this policy, refer to one of the following policies (a or b): a. For drugs on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the no coverage criteria policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.33 for health insurance marketplace, and CP.PMN.255 for Medicaid; or b. For drugs NOT on the formulary (commercial, health insurance marketplace) or PDL (Medicaid), the non-formulary policy for the relevant line of business: CP.CPA.190 for commercial, HIM.PA.103 for health insurance marketplace, and CP.PMN.16 for Medicaid; or

10. If the requested use (e.g., diagnosis, age, dosing regimen) is NOT specifically listed under section III (Diagnoses/Indications for which coverage is NOT authorized) AND criterion 1 above does not apply, refer to the off-label use policy for the relevant line of business: CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid. Page 2 of 6

    CLINICAL POLICY Omaveloxolone III. Diagnoses/Indications for which coverage is NOT authorized:
    A. Non-FDA approved indications, which are not addressed in this policy, unless there is sufficient documentation of efficacy and safety according to the off label use policies – CP.CPA.09 for commercial, HIM.PA.154 for health insurance marketplace, and CP.PMN.53 for Medicaid or evidence of coverage documents. IV. Appendices/General Information Appendix A: Abbreviation/Acronym Key FA: Friedreich’s ataxia
    FDA: Food and Drug Administration FXN: frataxin Appendix B: Therapeutic Alternatives
    Not applicable Appendix C: Contraindications/Boxed Warnings None reported
    mFARS: modified functional assessment rating scale
    Nrf2: nuclear factor erythroid 2–related factor 2 Appendix D: General Information • FA is a progressive, life-shortening ataxia which has cardinal symptoms of progressive gait and limb ataxia, lower limb areflexia, extensor plantar responses and dysarthria. In addition to ataxia, FA may cause fatigue, cardiomyopathy, and metabolic disturbances.
    • The mFARS is a disease specific, exam-based neurological rating scale which includes assessment of bulbar function, upper limb coordination, lower limb coordination, and upright stability. The mFARS has a maximum cumulative value of 93 points, where higher cumulative scores signify greater degree of disability. The rating scale is provided below:
    Neurologic assessment type (maximum points) Bulbar (5) (points) Upper limb coordination (36) Lower limb coordination (16) Upright stability (36) Cough (2)
    Speech (3) Finger-finger (3+3) Nose-finger (4+4) Dysmetria (4+4) Rapid movement (3+3) Finger taps (4+4) Heel-shin slide (4+4) Heel-shin tap (4+4) Sitting position (4) Stance feet apart (4) Stance feet apart with eyes closed (4) Stance feet together (4)
    Stance feet together with eyes closed (4) Tandem stance (4) Page 3 of 6

    CLINICAL POLICY Omaveloxolone Neurologic assessment type (maximum points) (points) Stance dominant foot (4) Tandem walk (3) Gait (5) V. Dosage and Administration Indication FA Dosing Regimen 150 mg PO QD Maximum Dose 150 mg/day VI. Product Availability
    Capsule: 50 mg
    VII.