Juxtapid — Blue Cross Blue Shield of Alabama

Initial criteria

• Diagnosis of homozygous familial hypercholesterolemia (HoFH) AND ONE of the following:
• - Genetic confirmation of bi-allelic pathogenic/likely pathogenic variants on different chromosomes at the LDLR, Apo-B, PCSK9, or LDLRAP1 genes, or ≥ 2 such variants at different loci OR
• - History of untreated LDL-C > 400 mg/dL (> 10 mmol/L) AND ONE of the following:
• • Cutaneous or tendon xanthomas before age < 10 years OR
• • Untreated elevated LDL-C levels consistent with heterozygous familial hypercholesterolemia (HeFH) in both parents (or in digenic form, one parent may have normal LDL-C and the other LDL-C consistent with HoFH)
• AND ONE of the following:
• - Tried a combination of a high-intensity statin (e.g., atorvastatin 40–80 mg, rosuvastatin 20–40 mg daily) and ezetimibe for ≥ 2 months and had an inadequate response OR
• - Intolerance or hypersensitivity to ALL combinations of a high-intensity statin and ezetimibe OR
• - FDA-labeled contraindication to ALL combinations of a high-intensity statin and ezetimibe
• AND ONE of the following:
• - Tried and had an inadequate response to a PCSK9 inhibitor OR