Pharmacy Prior Authorization Criteria

Bylvay (odevixibat)

Indications for Prior Authorization

Bylvay (odevixibat)

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

  Limitation 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).

• For diagnosis of Alagille syndrome
  Indicated for the treatment of cholestatic pruritus in patients 12 months of age and older with Alagille syndrome.

Criteria

Bylvay

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Progressive Familial Intrahepatic Cholestasis (PFIC)

• Diagnosis of progressive familial intrahepatic cholestasis (PFIC) type 1, 2, or 3 confirmed by one of the following: [B-D, 2]
• Diagnostic test (e.g., liver function test, liver ultrasound and biopsy, bile analysis)
• Genetic Testing
AND
• Patient is experiencing both of the following: [1]
• Moderate to severe pruritus
• Patient has a serum bile acid concentration above the upper limit of the normal reference for the reporting laboratory
AND
• Patient is 3 months of age or older
AND
• Patient has had an inadequate response to at least two of the following treatments used for the relief of pruritus: [6]
• Ursodeoxycholic acid (e.g., Ursodiol)
• Antihistamines (e.g., diphenhydramine, hydroxyzine)
• Rifampin
• Bile acid sequestrants (e.g., Questran, Colestid, Welchol)
AND
• Prescribed by or in consultation with a hepatologist or gastroenterologist

Bylvay

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Progressive Familial Intrahepatic Cholestasis (PFIC)

• Patient demonstrates positive clinical response to therapy (e.g., reduced serum bile acids, improved pruritus)

Bylvay

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Alagille Syndrome (ALGS)

• Both of the following:
• Diagnosis of Alagille Syndrome (ALGS)
AND
• Molecular genetic testing confirms mutations in the JAG1 or NOTCH2 gene [E, 7, 9]
AND
• Patient is experiencing both of the following: [10]
• Moderate to severe cholestatic pruritus
• Patient has a serum bile acid concentration above the upper limit of the normal reference for the reporting laboratory
AND
• Patient has had an inadequate response to at least two of the following treatments used for the relief of pruritus: [F, 7-8]
• Ursodeoxycholic acid (e.g., Ursodiol)
• Antihistamines (e.g., diphenhydramine, hydroxyzine)
• Rifampin
• Bile acid sequestrants (e.g., Questran, Colestid, Welchol)
AND
• Patient is 12 months of age or older
AND
• Prescribed by or in consultation with a hepatologist or gastroenterologist

Bylvay

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Alagille Syndrome (ALGS)

• Patient demonstrates positive clinical response to therapy (e.g., reduced bile acids, reduced pruritus severity score)

P & T Revisions

2024-08-14, 2023-08-22, 2023-07-21, 2022-09-09, 2022-03-28, 2021-11-19, 2021-08-23, 2021-08-18

References

1. Bylvay (odevixibat) [prescribing information]. Albireo Pharma Inc. Boston, MA. June 2023.
2. PFIC Advocacy and Resource Network, Inc. Available at https://www.pfic.org/types-and-subtypes-of-pfic/ Accessed August 5, 2021.
3. 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.
4. 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.
5. www.albireopharma.com/patients-families/progressive-familial-intrahepatic-cholestasis-pfic. Last accessed August 12, 2021.
6. Clinical Consult with Sirish Palle M.D. October 29, 2021.
7. Ayoub MD, Kamath BM. Alagille Syndrome: Diagnostic Challenges and Advances in Management. Diagnostics (Basel). 2020;10(11):907. Published 2020 Nov 6. doi:10.3390/diagnostics10110907
8. Shneider BL, Spino C, Kamath BM, et al. Placebo-Controlled Randomized Trial of an Intestinal Bile Salt Transport Inhibitor for Pruritus in Alagille Syndrome. Hepatol Commun. 2018;2(10):1184-1198.
9. Diaz-Frias J, Kondamudi NP. Alagille Syndrome. [Updated 2021 Jun 26]. In: StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2021 Jan-. Available from: https://www.ncbi.nlm.nih.gov/books/NBK507827/
10. ClinicalTrials.gov: Available at: https://www.clinicaltrials.gov/study/NCT04674761?term=NCT04674761&rank=1. Accessed July 12, 2023.
11. Saleh M, Kamath BM, Chitayat D. Alagille syndrome: clinical perspectives. Appl Clin Genet. 2016;9:75-82. Published 2016 Jun 30. doi:10.2147/TACG.S86420

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Revision History

• 2024-08-14: 2024 Annual Review.
• 2023-08-22: Program update to standard reauthorization language. No changes to clinical intent.
• 2023-07-21: 2023 Annual Review (PFIC) & New Indication for Alagille syndrome.
• 2022-09-09: 2022 Annual Review
• 2022-03-28: Update Guideline
• 2021-11-19: 2022 PA UM Review
• 2021-08-23: New PA Um Criteria
• 2021-08-18: Bylvay New PA Criteria

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