Juxtapid (lomitapide) — Cigna

Homozygous Familial Hypercholesterolemia (HoFH)

Initial criteria

Patient has phenotypic confirmation of homozygous familial hypercholesterolemia;
Patient is known to have two LDL-receptor negative alleles; AND
Patient meets ONE of the following (a or b):
a) Patient meets ALL of the following [(1), (2), and (3)]:
(1) Patient has tried one high-intensity statin therapy (i.e., atorvastatin ≥ 40 mg daily; rosuvastatin ≥ 20 mg daily [as a single entity or as a combination product]); AND
(2) Patient has tried one high-intensity statin along with ezetimibe (as a single-entity or as a combination product) for ≥ 8 continuous weeks; AND
(3) Low-density lipoprotein cholesterol level after this treatment regimen remains ≥ 70 mg/dL; OR
b) Patient has been determined to be statin intolerant by meeting ONE of the following [(1) or (2)]:
(1) Patient experienced statin-related rhabdomyolysis; OR
(2) Patient meets ALL of the following [(a), (b), and (c)]:
(a) Patient experienced skeletal-related muscle symptoms; AND
(b) The skeletal-muscle related symptoms occurred while receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or combination products); AND
(c) When receiving separate trials of both atorvastatin and rosuvastatin (as single-entity or as combination products), the skeletal-related muscle symptoms resolved upon discontinuation of each respective statin therapy (atorvastatin and rosuvastatin)

According to the prescriber, the patient has experienced a response to therapy.
Examples of a response to therapy include decreasing low-density lipoprotein cholesterol (LDL-C), total cholesterol, non-high-density lipoprotein (non-HDL-C), or apolipoprotein B levels.