Livmarli (maralixibat) — Blue Cross Blue Shield of Montana

Initial criteria

• Diagnosis of Alagille syndrome with pruritus (medical records required) OR progressive familial intrahepatic cholestasis (PFIC) with pruritus (medical records required)
• If PFIC, must NOT have PFIC2 with ABCB11 variants resulting in non-functional or absent BSEP-3 protein
• Tried and had inadequate response to ONE prerequisite agent (Bylvay prerequisite: cholestyramine; Livmarli prerequisites: naltrexone OR rifampicin OR sertraline OR ursodiol) OR intolerance/hypersensitivity to ONE prerequisite agent OR FDA labeled contraindication to ALL prerequisite agents
• If FDA labeled indication, patient's age is within FDA labeling OR supported for use for patient's age for that indication
• If indication supported in compendia, allowed compendia: AHFS or DrugDex level 1, 2A, or 2B
• For Bylvay: INR < 1.4 AND ALT and total bilirubin < 10× ULN
• For Livmarli: patient has NOT had surgical interruption of the enterohepatic circulation of bile acid
• Serum bile acid concentration above ULN
• Patient has NOT had a liver transplant OR has had a transplant and use supported post-transplant
• Prescriber is a gastroenterologist/hepatologist or has consulted with such specialist
• Patient will NOT use requested agent with another IBAT inhibitor
• No FDA labeled contraindications to requested agent
• Requested quantity within FDA labeled or compendia-supported dosing

Reauthorization criteria

• Previously approved for requested agent through plan’s prior authorization process
• Patient has demonstrated clinical benefit with the requested agent
• Prescriber is a specialist in patient's diagnosis area (gastroenterologist or hepatologist) or has consulted with such specialist
• Patient will NOT use in combination with another IBAT inhibitor
• No FDA labeled contraindications
• Requested quantity within FDA labeled or compendia-supported dosing

Approval duration

12 months (BCBSOK: 36 months)