Livmarli (maralixibat oral solution and tablets) — Cigna

Initial criteria

• Patient age ≥ 12 months; AND
• According to the prescriber, the patient has moderate-to-severe pruritus; AND
• Diagnosis of progressive familial intrahepatic cholestasis confirmed by genetic testing demonstrating a pathogenic gene variant affiliated with PFIC (ATP8B1, ABCB11, ABCB4, TJP2, NR1H4, or MYO5B); AND
• Patient has a serum bile acid concentration above the upper limit of the normal reference range for the reporting laboratory; AND
• Patient has tried at least two systemic medications for progressive familial intrahepatic cholestasis (cholestyramine, fenofibrate, naltrexone, rifampicin, sertraline, or ursodeoxycholic acid [ursodiol]), unless contraindicated; AND
• Patient does not have any of the following: cirrhosis OR portal hypertension OR history of a hepatic decompensation event (variceal hemorrhage, ascites, hepatic encephalopathy); AND
• Medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in progressive familial intrahepatic cholestasis

Reauthorization criteria

• Patient does not have any of the following: cirrhosis OR portal hypertension OR history of a hepatic decompensation event; AND
• Patient had response to therapy (e.g., decrease in serum bile acids and decrease in pruritus), as determined by the prescriber; AND
• Medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in progressive familial intrahepatic cholestasis

Approval duration

Initial: 6 months; Reauth: 1 year