Bylvay (odevixibat) — Point32Health
Alagille Syndrome
Initial criteria
- Documented diagnosis of Alagille Syndrome confirmed by genetic testing demonstrating the presence of a jagged canonical notch ligand 1 (JAG1) or notch receptor 2 (NOTCH2) mutation
- Documentation of cholestasis as defined by at least one of the following: Total serum bile acid greater than 3 times the upper limit of normal for age OR Conjugated bilirubin greater than 1 mg/dL OR Fat soluble vitamin deficiency that is otherwise unexplainable OR Gamma Glutamyl Transferase (GCT) greater than 3 times the upper limit for age OR Intractable pruritus explainable only by liver disease
- Prescribed by or in consultation with a hepatologist, gastroenterologist, or a provider who specializes in Alagille Syndrome (ALGS)
- Patient age ≥ 12 months
- Documentation of moderate to severe pruritus
- Documentation of one of the following: Inadequate response to at least two other medications to treat pruritus (e.g., ursodeoxycholic acid, antihistamines, rifampin, naltrexone, bile acid sequestrants) OR Clinical inappropriateness for use of other medications to treat pruritus
Reauthorization criteria
- Documented diagnosis of Alagille Syndrome confirmed by genetic testing demonstrating the presence of a jagged canonical notch ligand 1 (JAG1) or notch receptor 2 (NOTCH2) mutation
- Prescribed by or in consultation with a hepatologist, gastroenterologist, or a provider who specializes in Alagille Syndrome (ALGS)
- Patient age ≥ 12 months
- Documentation of a positive clinical response as evidenced by one of the following: Improvement in severity of pruritus OR Reduction in serum bile acid from baseline
- Documentation the patient has not had a liver transplant
Approval duration
initial 6 months; reauth 12 months