Subject: Livmarli 5005-A SGM P2022a

Drug

LIVMARLI  (maralixibat)

Policy:

      I.  INDICATIONS

          The indications below including FDA-approved indications and
          compendial uses are considered a covered benefit provided that
          all the approval criteria are met and the member has no
          exclusions to the prescribed therapy.

          FDA-Approved Indication
          Livmarli is indicated for the treatment of cholestatic pruritus in
          patients with Alagille syndrome (ALGS) 3 months of age and
          older.

          All other indications are considered experimental/investigational
          and not medically necessary.

 

    II.  DOCUMENTATION

          Submission of the following information is necessary to initiate
          the prior authorization review:
          A.  Initial requests: Genetic testing results confirming a
               diagnosis of Alagille syndrome (ALGS) (if applicable)
          B.  Continuation requests: Chart notes or medical records
               documenting a benefit from therapy (e.g., improvement in
               pruritis)

 
    II.  PRESCRIBER SPECIALTIES
          This medication must be prescribed by or in consultation with a
          hepatologist.

 
   IV.  CRITERIA FOR INITIAL APPROVAL

          Cholestatic pruritis in Alagille syndrome (ALGS)
          Authorization of 6 months may be granted for treatment of
          cholestatic pruritis in Alagille syndrome (ALGS) when all of the
          following criteria are met:
          A.  Member has a diagnosis of ALGS confirmed by either of the
               following:
               1.  Genetic testing
               2.  Member has both of the following:
                    i.      Bile duct paucity
                    ii.     Three of the five major clinical features of ALGS:
                            a.  Cholestasis
                            b.  Cardiac defect (e.g., stenosis of the peripheral
                                 pulmonary artery and its branches)
                            c.  Skeletal abnormality (e.g., butterfly vertebrae)
                            d.  Ophthalmologic abnormality (e.g., posterior
                                 embryotoxon)
                            e.  Characteristic facial features (e.g., triangular-
                                 shaped face with a broad forehead and a pointed
                                 chin, bulbous tip of the nose, deeply set eyes,
                                 and hypertelorism)
          B.  Member has evidence of cholestasis defined as the presence
               of one or more of the following:
               1.  Total serum bile acid greater than 3 times the upper limit
                    of normal (ULN) for age
               2.  Conjugated bilirubin greater than 1 mg/dL
               3.  Fat soluble vitamin deficiency otherwise unexplainable
               4.  Gamma-glutamyl transferase (GGT) greater than 3 times
                    ULN for age
               5.  Intractable pruritis explainable only by liver disease
          C.  Member does not have a history or presence of other
               concomitant liver disease
          D.  Member has not received a liver transplant
          E.  Member is 3 months of age or older

     V.  CONTINUATION OF THERAPY

           Authorization of 12 months may be granted for all members
          (including new members) requesting continuation of therapy
          when the member is experiencing benefit from therapy (e.g.,
          improvement in pruritis).

 

Place of Service:

Outpatient

The above policy is based on the following references:

1. Livmarli [package insert]. Foster City, CA: Mirum Pharmaceuticals, Inc.; March 2023.
2. Spinner NB, Gilbert MA, Loomes KM, Krantz ID. Alagille syndrome. GeneReviews® [Internet]. December 12, 2019. Last updated December 12, 2019. Accessed October 19, 2021. https://www.ncbi.nlm.nih.gov/books/NBK1273/#__NBK1273_dtls__.
3. Genetic and Rare Diseases Information Center. Alagille syndrome. Rare Disease Database. https://rarediseases.info.nih.gov. Updated October 20, 2017. Accessed October 18, 2021.
4. National Organization for Rare Disorders (NORD). Alagille syndrome. Rare Disease Database. https://rarediseases.org. Published 2020. Accessed October 18, 2021.

Copyright Aetna Inc. All rights reserved. Pharmacy Clinical Policy Bulletins are developed by Aetna to assist in administering plan benefits and constitute neither offers of coverage nor medical advice. This Clinical Policy Bulletin contains only a partial, general description of plan or program benefits and does not constitute a contract. Aetna does not provide health care services and, therefore, cannot guarantee any results or outcomes. Participating providers are independent contractors in private practice and are neither employees nor agents of Aetna or its affiliates. Treating providers are solely responsible for medical advice and treatment of members. This Clinical Policy Bulletin may be updated and therefore is subject to change.

May 07, 2023