Subject: tiopronin-Thiola 2991-A SGM P2023

Drug

THIOLA  (tiopronin)

THIOLA EC  (tiopronin delayed release tablets)

tiopronin

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 Indications
          Thiola and Thiola EC are indicated, in combination with high fluid
          intake, alkali, and diet modification, for the prevention of cystine
          stone formation in adult and pediatric patients 20 kg and greater
          with severe homozygous cystinuria who are not responsive to
          these measures alone.

          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:
               1.  Genetic testing results showing mutations in both alleles
                    of the SLC3A1 or SLC7A9 genes; and
               2.  Lab results documenting baseline urinary cystine levels.
          B.  Continuation of therapy requests: lab results documenting a
               decrease in urinary cystine levels compared to baseline.

  III.  CRITERIA FOR INITIAL APPROVAL

          Cystinuria
          Authorization of 12 months may be granted for prevention of
          cystine stone formation in a member with severe homozygous
          cystinuria (biallelic mutations/variants in the SLC3A1 or the
          SLC7A9 gene) when all of the following criteria are met:
          A.  Diagnosis of homozygous cystinuria (biallelic
               mutations/variants in the SLC3A1 or the SLC7A9 gene) was
               confirmed by genetic testing showing mutations in both
               alleles of the SLC3A1 or SLC7A9 genes.
          B.  The requested medication is being used as an adjunct to high
               fluid intake, alkali, and diet modification.
          C.  The member has elevated urinary cysteine levels at baseline.

 
   IV.  CONTINUATION OF THERAPY

          Authorization of 12 months may be granted for continued
          treatment of severe cystinuria in members requesting
          reauthorization who have experienced a decrease in urinary
          cystine levels compared to pretreatment baseline.

Place of Service:

Outpatient

The above policy is based on the following references:

1. Thiola [package insert]. San Antonio, TX: Mission Pharmaceutical Company; January 2021.
2. Thiola EC [package insert]. San Antonio, TX: Mission Pharmaceutical Company; March 2021.
3. Biyani CS, Cartledge JJ. Cystinuria- diagnosis and management. Eur Urology. 2006; 4:175-183.
4. Pearle MS, Goldfarb DS, Assimos DG, Curhan G, Denu-Ciocca CJ, Matlaga BR, Monga M, Penniston KL, Preminger GM, Turk TM, White JR; American Urological Association. Medical management of kidney stones: AUA guideline. J Urol. 2014 Aug;192(2):316-24.
5. Goldfarb DS, et al. Cystinuria and cystine stones. UpToDate, Lam, AO (Ed), Waltham, MA, 2022. URL: www.uptodate.com. Accessed October 27, 2022.

 

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.

June 18, 2023