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JuxtapidMedical Mutual

Homozygous Familial Hypercholesterolemia (HoFH)

Initial criteria

  • Patient age ≥ 18 years
  • Patient meets one of the following (i, ii, or iii):
  • i. Phenotypic confirmation of homozygous familial hypercholesterolemia OR
  • ii. Untreated LDL-C > 400 mg/dL AND (a. Clinical manifestation of HoFH before age 10 years OR b. At least one parent had untreated LDL-C or total cholesterol consistent with HeFH) OR
  • iii. Treated LDL-C ≥ 300 mg/dL AND (a. Clinical manifestations of HoFH before age 10 years OR b. At least one parent had untreated LDL-C or total cholesterol consistent with HeFH)
  • Patient meets one of the following (i or ii):
  • i. Tried at least one PCSK9 inhibitor for ≥ 8 continuous weeks AND LDL-C remains ≥ 70 mg/dL OR
  • ii. Patient is known to have two LDL-receptor negative alleles
  • Patient meets one of the following (i or ii):
  • i. Tried one high-intensity statin (atorvastatin ≥ 40 mg daily OR rosuvastatin ≥ 20 mg daily) AND tried high-intensity statin + ezetimibe for ≥ 8 continuous weeks AND LDL-C remains ≥ 70 mg/dL OR
  • ii. Patient determined to be statin intolerant by either:
  • a. Experienced statin-related rhabdomyolysis OR
  • b. Experienced skeletal-related muscle symptoms that occurred while receiving separate trials of both atorvastatin and rosuvastatin AND resolved upon discontinuation of each respective therapy

Reauthorization criteria

  • Initial criteria are still met
  • Provider has checked ALT and AST since initiation (monthly within the first year OR every 3 months after the first year) and adjusted dose if ALT or AST ≥ 3x ULN
  • Patient is having a beneficial response (as determined by the prescriber) AND reduction in LDL levels

Approval duration

initial 90 days; renewal 180 days