Livmarli (maralixibat) — Blue Cross Blue Shield of Texas

Initial criteria

• ONE of the following: (A) patient has diagnosis of Alagille syndrome with pruritus OR (B) patient has diagnosis of PFIC with pruritus AND does NOT have PFIC2 with ABCB11 variants resulting in non-functional or complete absence of bile salt export pump protein (BSEP-3)
• Patient has ONE of the following: (A) tried and had inadequate response to ONE prerequisite agent OR (B) intolerance/hypersensitivity to ONE prerequisite agent OR (C) FDA labeled contraindication to ALL prerequisite agents; prerequisite agents include: Bylvay → cholestyramine, Livmarli → naltrexone, rifampicin, sertraline, ursodiol
• If the patient has an FDA labeled indication: ONE of the following: (A) patient's age is within FDA labeling for indication OR (B) support exists for using the agent for patient's age for that indication
• If patient has compendia-supported indication same requirements apply
• If the requested agent is Bylvay: INR < 1.4 AND ALT and total bilirubin < 10× upper limit of normal (ULN)
• If the requested agent is Livmarli: patient has NOT had surgical interruption of the enterohepatic circulation of bile acid
• Patient has serum bile acid concentration above ULN
• ONE of the following: (A) patient has NOT had liver transplant OR (B) patient has had liver transplant and support exists for use post transplant
• Prescriber is a specialist in gastroenterology or hepatology or has consulted one
• Patient will NOT use requested agent with another Ileal Bile Acid Transport (IBAT) inhibitor agent
• Patient does NOT have any FDA labeled contraindications to the requested agent
• Requested dose is within FDA labeled dosing or supported in compendia

Reauthorization criteria

• Patient previously approved for the requested agent through plan’s PA process
• Patient has had clinical benefit with the requested agent
• Prescriber is or consulted a specialist in gastroenterology or hepatology
• Patient will NOT be using the requested agent with another IBAT inhibitor
• Patient does NOT have any FDA labeled contraindications
• Requested dose within FDA labeled dosing or supported in compendia

Approval duration

12 months (36 months for BCBSOK)