Juxtapid (lomitapide) — Highmark

homozygous familial hypercholesterolemia (HoFH)

Preferred products

age ≥ 18 years
diagnosis of HoFH (ICD-10: E78.0) supported by ONE of the following: genetic confirmation of two mutant alleles at the LDLR, APOB, PCSK9, or LDLRAP1 gene locus OR BOTH of the following: (1) untreated LDL-C > 400 mg/dL OR untreated TC > 500 mg/dL AND (2) cutaneous or tendon xanthoma before 10 years of age OR evidence of HeFH in both parents
EITHER experienced therapeutic failure to a maximally tolerated statin OR statin intolerant defined as EITHER (a) at least two separate trials of different statins associated with (i) statin-related rhabdomyolysis resolving upon discontinuation OR (ii) skeletal-related muscle symptoms resolving upon discontinuation OR (b) any course of statin therapy associated with (i) CK increase to 10× ULN OR (ii) LFTs increase to 3× ULN OR (iii) hospitalization due to severe statin-related adverse event (e.g., rhabdomyolysis)
EITHER current LDL-C > 70 mg/dL OR has ASCVD or major ASCVD risk factors (e.g., diabetes) with current LDL-C > 55 mg/dL
experienced therapeutic failure, contraindication, or intolerance to ezetimibe
experienced therapeutic failure, contraindication, or intolerance to plan-preferred Repatha
will continue to receive concurrent lipid-lowering therapies for treatment of HoFH

prescriber attests member has experienced a reduction in LDL-C from baseline
member will continue to receive concurrent lipid-lowering therapies for the treatment of HoFH

up to 6 months (12 months for Delaware Commercial fully insured and ACA members)