Livmarli (maralixibat) — Highmark

Initial criteria

• Diagnosis of Alagille syndrome (ICD-10: Q44.71) confirmed by genetic testing demonstrating a JAGGED1 or NOTCH2 deletion or mutation OR diagnosis of progressive familial intrahepatic cholestasis (PFIC) confirmed by genetic testing
• For ALGS: age ≥ 3 months; elevated serum bile acid levels above laboratory reference range; provider attests member experiences cholestatic pruritus explained only by liver disease; does NOT have decompensated cirrhosis, hepatic decompensation event, or portal hypertension
• For ALGS dosing: using Livmarli oral solution 9.5 mg/mL OR oral tablet and weighs ≥ 25 kg
• For PFIC: age ≥ 12 months; no PFIC type 2 with ABCB11 variant resulting in nonfunctional or absent bile salt export pump protein; elevated serum bile acid levels above laboratory reference range; provider attests member experiences cholestatic pruritus explained only by liver disease; does NOT have decompensated cirrhosis, hepatic decompensation event, or portal hypertension
• For PFIC dosing: using Livmarli oral solution 19 mg/mL OR oral tablet and weighs ≥ 25 kg

Reauthorization criteria

• Prescriber attests member has experienced positive clinical response to therapy as evidenced by both decrease in total serum bile acid levels from baseline AND improvement in pruritus
• Prescriber attests member has not progressed to cirrhosis, experienced a hepatic decompensation event, or developed portal hypertension
• Member continues on Livmarli 9.5 mg/mL or oral tablets for ALGS OR Livmarli 19 mg/mL or oral tablets for PFIC

Approval duration

initial 6 months, reauthorization 12 months