CurrentCignaPolicy IP0566

Neurology - Skyclarys

Defines medical coverage criteria for Skyclarys (omaveloxolone) for treatment of Friedreich's ataxia in patients ≥16 years, including initial and continuation approval conditions, exclusions (not medically necessary uses), and clinical/billing notes for Cigna plans.

Policy Summary

PayerCigna

PolicyNeurology - Skyclarys

Policy CodePolicy IP0566

Change TypeAdded continuation criteria

Effective DateJul 1, 2025

Next Review DateJul 1, 202624d

Key ActionApprove for 1 year when criteria are met and ensure therapy is prescribed by or in consultation with a neurologist or physician specializing in ataxias/neuromuscular disorders.

SourceLink

POLICY UPDATE CHANGES

Added 'Patient is Currently Receiving Skyclarys' continuation criteria (Annual Review 08/15/2024).

1Covered Indication (Friedreich's ataxia)

2Not Covered Example Indications Listed

1 yearInitial Approval Duration

Coverage Summary

Defines medical coverage criteria for Skyclarys (omaveloxolone) for the treatment of Friedreich's ataxia in patients ≥ 16 years, including initial and continuation approval conditions, exclusions for non‑covered uses, and clinical/documentation requirements for Cigna plans. Coverage stance: covered with criteria.

Medical Necessity Criteria

FDA-Approved Indications - Friedreich's Ataxia

Approve for 1 year if the patient meets ONE of the following (A or B):

ANY of the following

A) Initial Therapy
- Patient is ≥ 16 years of age≥ 16 years
- Patient has had genetic testing confirming biallelic pathogenic variants in the frataxin (FXN) gene consistent with a diagnosis of Friedreich's ataxia
  Document genetic testing confirming biallelic pathogenic FXN variants
- ALL of the following
  - Patient has a B-type natriuretic peptide (BNP) ≤ 200 pg/mL≤ 200 pg/mL
    BNP within last year
  - Patient has a left ventricular ejection fraction ≥ 40%≥ 40%
    LVEF within last year
  - Patient has a hemoglobin A1c (HbA1c) ≤ 11%≤ 11%
    HbA1c within last year
- Patient has been assessed using the modified Friedreich's Ataxia Rating Scale (mFARS) and has a score ≥ 20, but ≤ 80mFARS ≥ 20 and ≤ 80
  Document mFARS score
- Patient is ambulatory
- Patient does not have pes cavus
- Medication is prescribed by or in consultation with a neurologist or a physician who specializes in ataxias and/or neuromuscular disorders
B) Patient is Currently Receiving Skyclarys (Continuation)
- Patient is ≥ 16 years of age≥ 16 years
- Patient has had genetic testing confirming biallelic pathogenic variants in the frataxin (FXN) gene consistent with a diagnosis of Friedreich's ataxia
  Document genetic testing confirming biallelic pathogenic FXN variants
- Patient is ambulatory
- According to the prescriber, the patient continues to benefit from therapy, as demonstrated by a slowed progression on the modified Friedreich's Ataxia Rating Scale
  Prescriber must attest continued benefit (slowed progression on mFARS)
- Medication is prescribed by or in consultation with a neurologist, or a physician who specializes in ataxias and/or neuromuscular disorders

Not Medically Necessary / Experimental Uses

Conditions Not Covered

Skyclarys for any use other than the FDA-approved indication above is considered not medically necessary, including:

ANY of the following

Metastatic melanoma (Skyclarys evaluated in combination with nivolumab or ipilimumab; results not published; more data needed)
Mitochondrial myopathy (Phase II study showed no differences in peak workload or 6-minute walk test vs placebo; more data needed)

Denial Risk

Not covered uses

Claims for Skyclarys for any use other than the FDA‑approved indication of Friedreich's ataxia per the policy criteria will be considered not medically necessary and denied.

Provider Actions & Authorization

Prior Authorization

Approval duration and prescriber specialty

Approve for 1 year when criteria are met; therapy must be prescribed by or in consultation with a neurologist or a physician who specializes in ataxias and/or neuromuscular disorders.

Documentation Required

Required diagnostic confirmation and assessments

Document genetic testing confirming biallelic pathogenic variants in the FXN gene, recent (within the last year) BNP, left ventricular ejection fraction (LVEF), and hemoglobin A1c (HbA1c) results, and a modified Friedreich's Ataxia Rating Scale (mFARS) score to support approval.

Documentation Required

Continuation benefit documentation

For patients currently receiving Skyclarys, the prescriber must attest that the patient continues to benefit from therapy as demonstrated by a slowed progression on the modified Friedreich's Ataxia Rating Scale (mFARS).

Denial Risk

Not covered uses

Claims for Skyclarys for indications other than Friedreich's ataxia as specified in the policy (for example, metastatic melanoma or mitochondrial myopathy) are considered not medically necessary and will be denied.

Background & Evidence

Skyclarys (omaveloxolone) is an Nrf2 activator indicated for Friedreich's ataxia in patients ≥ 16 years of age.

Pivotal clinical trials enrolled patients with genetically confirmed Friedreich's ataxia, required baseline mFARS 20–80, and the vast majority (~93%) were ambulatory.

Trial exclusions included cardiac and diabetes‑related criteria: patients with BNP > 200 pg/mL, left ventricular ejection fraction < 40%, and uncontrolled diabetes (e.g., HbA1c > 11% in non‑pivotal study definitions) were excluded.

Pivotal study and definitions

Pivotal study age range16 to 40 years

Primary efficacy measuremodified Friedreich's Ataxia Rating Scale (mFARS)

Definition: mFARSModified Friedreich's Ataxia Rating Scale, used to assess disease severity and progression.

Definition: BNPB-type natriuretic peptide, cardiac biomarker; threshold ≤ 200 pg/mL required.