Subject: tiopronin-Thiola 2991-A SGM P2022
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THIOLA
(tiopronin)
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THIOLA EC
(tiopronin delayed release tablets)
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tiopronin
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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:
- Thiola [package insert]. San Antonio, TX: Mission Pharmaceutical Company; January 2021.
- Thiola EC [package insert]. San Antonio, TX: Mission Pharmaceutical Company; March 2021.
- Biyani CS, Cartledge JJ. Cystinuria- diagnosis and management. Eur Urology. 2006; 4:175-183.
- 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.
- Goldfarb DS, et al. Cystinuria and cystine stones. UpToDate, Lam, AO (Ed), Waltham, MA, 2022. URL: www.uptodate.com. Accessed March 14, 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 26, 2022
