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SkyclarysMedical Mutual

Friedreich’s ataxia

Initial criteria

  • Patient is age ≥ 16 years; AND
  • Patient has had genetic testing confirming biallelic pathogenic variants in the frataxin (FXN) gene consistent with a diagnosis of Friedreich’s ataxia [documentation required]; AND
  • Patient has had ALL of the following within the last year (a, b, and c):
  • a) Patient has a B-type natriuretic peptide (BNP) ≤ 200 pg/mL [documentation required]; AND
  • b) Patient has a left ventricular ejection fraction ≥ 40% [documentation required]; AND
  • c) Patient has a hemoglobin A1c ≤ 11% [documentation required]; AND
  • Patient has been assessed using the modified Friedreich’s Ataxia Rating Scale and has a score ≥ 20, but ≤ 80 [documentation required]; AND
  • Patient is ambulatory; AND
  • Patient does not have pes cavus; AND
  • The medication is prescribed by or in consultation with a neurologist or a physician who specializes in ataxias and/or neuromuscular disorders.

Reauthorization criteria

  • Patient is age ≥ 16 years; AND
  • Patient has had genetic testing confirming biallelic pathogenic variants in the frataxin (FXN) gene consistent with a diagnosis of Friedreich’s ataxia [documentation required]; AND
  • Patient is ambulatory; AND
  • 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; AND
  • The medication is prescribed by or in consultation with a neurologist, or a physician who specializes in ataxias and/or neuromuscular disorders.

Approval duration

1 year initial, 1 year reauth