Livmarli — Medical Mutual

Alagille Syndrome

Initial criteria

Patient is age ≥ 3 months; AND
Patient has moderate-to-severe pruritus, according to the prescriber; AND
Diagnosis of Alagille syndrome was confirmed by genetic testing demonstrating a JAG1 or NOTCH2 deletion or pathogenic variant; 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 Alagille syndrome, unless contraindicated; AND
Patient does not have any of the following: cirrhosis OR portal hypertension OR history of a hepatic decompensation event; AND
The medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in Alagille syndrome

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, as determined by the prescriber (e.g., decrease in serum bile acids and decrease in pruritus); AND
The medication is prescribed by or in consultation with a hepatologist, gastroenterologist, or a physician who specializes in Alagille syndrome

6 months initial, 1 year reauth