CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Tiopronin (Thiola) — Coverage Criteria for Prevention of Cystine Kidney Stones

This policy governs prior authorization and coverage criteria for tiopronin (Thiola) for prevention of cystine stone formation in members with severe homozygous cystinuria; applies to Neighborhood Health Plan of Rhode Island Medicaid members.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyTiopronin (Thiola) — Coverage Criteria for Prevention of Cystine Kidney Stones

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateJun 1, 2020

Next Review DateN/A

Key ActionSubmit genetic testing results showing mutations in both alleles of the SLC3A1 or SLC7A9 genes to support prior authorization.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsinitial auth

6 monthscontinuation auth

>=20 kgmin patient weight

>500 mg/dayurinary cystine threshold

SLC3A1/SLC7A9required genes

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyTiopronin (Thiola) — Coverage Criteria for Prevention of Cystine Kidney Stones

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateJun 1, 2020

Next Review DateN/A

Key ActionSubmit genetic testing results showing mutations in both alleles of the SLC3A1 or SLC7A9 genes to support prior authorization.

SourceLink

On This Page

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Initial Therapy

Authorization of 6 months may be granted when ALL of the following are met:

Initial Authorization for Tiopronin: A. Diagnosis of homozygous cystinuria confirmed by genetic testing showing mutations in both alleles of the SLC3A1 or SLC7A9 genes; B. The pre-treatment urinary cystine level is greater than 500 mg/day; C. The requested medication is being used as an adjunct to high fluid intake, alkali, and diet modification; D. Dosing is in accordance with the United States Food and Drug Administration approved labeling.

Continuation Therapy

Authorization of 6 months may be granted for continued treatment when ALL of the following are met:

Continuation Authorization for Tiopronin: A. Member has experienced a decrease in urinary cystine levels compared to pretreatment baseline; B. Therapy is being used as an adjunct to high fluid intake, alkali, and diet modification; C. Dosing is in accordance with the United States Food and Drug Administration approved labeling.

Use of tiopronin is limited to the FDA‑approved indication: prevention of cystine kidney stone formation in patients with severe homozygous cystinuria who are not responsive to conservative measures. All other indications are considered experimental/investigational and are not a covered benefit.

Key Clinical Thresholds & Coding-Adjacent Values

Pre-treatment urinary cystine — numeric threshold used in criteria

Pre-treatment urinary cystine threshold> 500 mg/day

Context for thresholdRequired for initial authorization for prevention of cystine stone formation

Measurement timingValue must be from a pretreatment (baseline) 24-hour urinary cystine collection

Minimum patient weight (FDA) — weight threshold for indication

Minimum patient weight for FDA indication>= 20 kg

PopulationApplies to adult and pediatric patients 20 kg and greater

Relation to indicationWeight is part of FDA-approved indication for tiopronin with adjunctive measures

Provider Actions, Documentation & Prior Authorization Requirements

Prior Authorization

Initial Authorization Criteria

Authorization of 6 months may be granted for prevention of cystine stone formation in a member with severe homozygous cystinuria when all of the following criteria are met: A. Diagnosis of homozygous cystinuria was confirmed by genetic testing showing mutations in both alleles of the SLC3A1 or SLC7A9 genes; B. The pre-treatment urinary cystine level is greater than 500 mg/day; C. The requested medication is being used as an adjunct to high fluid intake, alkali, and diet modification; D. Dosing is in accordance with the United States Food and Drug Administration approved labeling.

Prior authorization may be granted for up to 6 months for prevention of cystine stone formation in members with severe homozygous cystinuria.
Required pre-treatment urinary cystine > 500 mg/day.
Dosing must follow FDA-approved labeling.

Documentation Required

Required Genetic Testing

Submit genetic testing results showing mutations in both alleles of the SLC3A1 or SLC7A9 genes.

Documentation Required

Adjunctive Therapy Requirement

Therapy must be used as an adjunct to high fluid intake, alkali, and diet modification; dosing must follow FDA labeling.

Adjunctive measures (high fluid intake, alkali, diet modification) must be documented in the medical record.
Dosing must be consistent with FDA-approved labeling for the requested product.

Denial Risk

Non‑FDA Indications Not Covered

Tiopronin is indicated only for prevention of cystine stone formation in severe homozygous cystinuria as described above. All other indications are considered experimental/investigational and are not a covered benefit.

Non‑FDA indications are not covered and will be denied as investigational/experimental.

OpenPayer

Tiopronin (Thiola) — Coverage Criteria for Prevention of Cystine Kidney Stones

Coverage Criteria for Tiopronin (Thiola)

Key Clinical Thresholds & Coding-Adjacent Values

Provider Actions, Documentation & Prior Authorization Requirements

Background

Definitions