Bylvay (odevixibat capsules and oral pellets) — Cigna

Initial criteria

• Patient age ≥ 3 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 mutation in ATP8B1, ABCB11, ABCB4, TJP2, NR1H4, or MYO5B; AND
• Patient has a serum bile acid concentration above the upper limit of normal reference range for the reporting laboratory; AND
• Patient has tried at least two systemic medications for progressive familial intrahepatic cholestasis (fenofibrate, cholestyramine, naltrexone, rifampicin, sertraline, ursodeoxycholic acid [ursodiol]), unless contraindicated; AND
• Patient does not have cirrhosis OR portal hypertension OR history of hepatic decompensation event (e.g., variceal hemorrhage, ascites, hepatic encephalopathy); AND
• Medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or physician who specializes in progressive familial intrahepatic cholestasis.

Reauthorization criteria

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

Approval duration

initial 6 months; reauth 1 year