CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

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

Covers prior authorization criteria and documentation requirements for tiopronin use to prevent cystine stones in patients with severe homozygous cystinuria; applies to members of Neighborhood Health Plan of Rhode Island.

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 SLC3A1 or SLC7A9 and pretreatment urinary cystine level to request prior authorization.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6minitial authorization duration

6mcontinuation authorization duration

>500 mg/daypretreatment urinary cystine

biallelicgenetic confirmation

Coverage Criteria for Tiopronin (Thiola)

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 SLC3A1 or SLC7A9 and pretreatment urinary cystine level to request prior authorization.

SourceLink

On This Page

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Genetic diagnosis: Diagnosis of homozygous cystinuria confirmed by genetic testing showing mutations in both alleles of the SLC3A1 or SLC7A9 genes

Supported by required documentation; submit genetic testing results (see documentation)

Urinary cystine level: Pre-treatment urinary cystine level is greater than 500 mg/day>500 mg/day

Measured prior to initiation

Adjunctive conservative measures: Requested medication is being used as an adjunct to high fluid intake, alkali, and diet modification (patient not responsive to these measures alone)

Per FDA-approved indication

Dosing is in accordance with the United States Food and Drug Administration approved labeling

Drug must be dosed per label

Continuation criteria

Continuation (re-authorization) covered when ALL of the following are met

Response: Member has experienced a decrease in urinary cystine levels compared to pretreatment baseline

Demonstrated improvement required for reauthorization

Adjunctive measures maintained: Therapy is being used as an adjunct to high fluid intake, alkali, and diet modification

Continued conservative measures required

Dosing is in accordance with the United States Food and Drug Administration approved labeling

Per label

Provider Actions, Documentation, and Authorization Process

Prior Authorization

Continuation authorization — Reauthorization (6 months)

Authorization of 6 months may be granted for continued treatment of severe cystinuria when all listed continuation criteria are met.

Decrease in urinary cystine from pretreatment baseline required
Therapy must be adjunctive to high fluid intake, alkali, and diet modification
Dosing must follow FDA-approved labeling
Reauthorization period: 6 months

Denial Risk

Non-covered indications

Use of tiopronin for any indication other than prevention of cystine stone formation in severe homozygous cystinuria (as an adjunct to conservative measures) is considered experimental/investigational and is not a covered benefit.

Non-covered: indications other than prevention of cystine stones in severe homozygous cystinuria

Denial Risk

Criteria not met — Grounds for denial

Prior authorization will be denied when required criteria/documentation are not met.

Lack of genetic confirmation of homozygous cystinuria (mutations in both alleles of SLC3A1 or SLC7A9)
Pretreatment urinary cystine ≤ 500 mg/day
Requested use is not adjunctive to high fluid intake, alkali, and diet modification
Use for indications other than prevention of cystine stone formation in severe homozygous cystinuria

Definitions

Severe homozygous cystinuria

Genetic confirmationDiagnosis confirmed by genetic testing showing mutations in both alleles of SLC3A1 or SLC7A9

Urinary cystine levelElevated pretreatment urinary cystine > 500 mg/day required

Therapy contextTreatment must be adjunctive to high fluid intake, alkali, and diet modification (patient not responsive to conservative measures)

Indication scopeTiopronin indicated for prevention of cystine kidney stones in severe homozygous cystinuria per FDA labeling; other indications are not covered

OpenPayer

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

Coverage Criteria for Tiopronin (Thiola)

Key Coding & Laboratory Thresholds

Provider Actions, Documentation, and Authorization Process

Background

Definitions