BYLVAY (odevixibat)

BYLVAY (odevixibat)

Self Administration - Oral

Indications for Prior Authorization:

Indicated for the treatment of pruritus in patients 3 months of age and older with progressive familial intrahepatic cholestasis (PFIC).

Limitations of Use:

Bylvay™ may not be effective in PFIC type 2 patients with ABCB11 variants resulting in non-functional or complete absence of bile salt export pump protein (BSEP-3).

Coverage Criteria:

For diagnosis of progressive familial intrahepatic cholestasis (PFIC):

• Dose does not exceed initial dosing of 40 mcg/kg which may be titrated up to 120 mcg/kg once daily (not to exceed a total daily dose of 6 mg), AND
• Patient is 3 months of age or older, AND
• Prescribed by or in consultation with a hepatologist, AND
• Chart note documentation is provided and confirms the diagnosis of pruritus associated with progressive familial intrahepatic cholestasis (PFIC), AND
• Diagnosis is confirmed by genetic testing, AND
• Documentation of member’s current weight; 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 and failed at least one systemic medication for progressive familial intrahepatic cholestasis (e.g., ursodeoxycholic acid (ursodiol), cholestyramine, rifampicin), AND
• Patient does not have cirrhosis, portal hypertension, or history of a hepatic decompensation event (e.g., variceal hemorrhage, ascites, hepatic encephalopathy).

Reauthorization Criteria:

For diagnosis of progressive familial intrahepatic cholestasis (PFIC):

• Dose does not exceed 120 mcg/kg once daily (not to exceed a total daily dose of 6 mg), AND
• Documentation is provided showing a positive clinical response to therapy (e.g., decrease in serum bile acids and decrease in pruritus), AND
• Patient does not have cirrhosis, portal hypertension, or history of a hepatic decompensation event (e.g., variceal hemorrhage, ascites, hepatic encephalopathy).

Coverage Duration:

• Initial: 6 months
• Reauthorization: 1 year

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information:

• Dosing:
  • Recommended dosage of Bylvay™ is 40 mcg/kg once daily in the morning with a meal. 
  • If there is no improvement in pruritus after 3 months, the dosage may be increased in 40 mcg/kg increments up to 120 mcg/kg once daily not to exceed a total daily dose of 6 mg.
  • Bylvay™ oral pellets are intended for use by patients weighing less than 19.5 kilograms.
  • Bylvay™ capsules are intended for use by patients weighing 19.5 kilograms or above.
  • Dose modification:
    • Interrupt Bylvay™ if new onset liver test abnormalities occur or symptoms consistent with clinical hepatitis are observed. Once the liver test abnormalities either return to baseline values or stabilize at a new baseline value, consider restarting Bylvay™ at the lowest dose of 40 mcg/kg, and increase as tolerated if appropriate. Consider discontinuing Bylvay™ permanently if liver test abnormalities recur.
    • Discontinue Bylvay™ permanently if a patient experiences a hepatic decompensation event (e.g., variceal hemorrhage, ascites, hepatic encephalopathy).
• Obtain baseline liver tests (e.g., ALT [alanine aminotransferase], AST [aspartate aminotransferase], TB [total bilirubin], DB [direct bilirubin] and International Normalized Ratio [INR]) and monitor during treatment.
• Warning and precautions include:
  • Liver test abnormalities
  • Diarrhea
  • Fat-soluble vitamin (FSV) deficiency
• Patients with PFIC may have impaired hepatic function at baseline. The efficacy and safety in PFIC patients with clinically significant portal hypertension and in patients with decompensated cirrhosis have not been established.

 Policy Updates:

• 02/15/2022 – New policy approved by P&T.

References:

• Bylvay (odevixibat) [prescribing information]. Albireo Pharma Inc. Boston, MA. July 2021.
• PFIC Advocacy and Resource Network, Inc. Available at https://www.pfic.org/types-and-subtypes-of-pfic/ Accessed August 5, 2021.
• Bylvay (odevixibat) [prescribing information]. Available at https://www.accessdata.fda.gov/drugsatfda_docs/label/2021/215498s000lbl.pdf?utm_medium=email&utm_source=govdelivery. Accessed August 5, 2021.
• Lexicomp [database online]. Available at www.uptodate.com/contents/odevixibat-drug-information?search=bylvay&source=panel_search_result&selectedTitle=1~1&usage_type=panel&kp_tab=drug_general&display_rank=1. Last accessed August 5, 2021.
• www.albireopharma.com/patients-families/progressive-familial-intrahepatic-cholestasis-pfic. Last accessed August 12, 2021.