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Date Printed: August 23, 2017: 06:08 AM

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This medical policy (medical coverage guideline) is Copyright 2017, Blue Cross and Blue Shield of Florida (BCBSF). All Rights Reserved. You may not copy or use this document or disclose its contents without the express written permission of BCBSF. The medical codes referenced in this document may be proprietary and owned by others. BCBSF makes no claim of ownership of such codes. Our use of such codes in this document is for explanation and guidance and should not be construed as a license for their use by you. Before utilizing the codes, please be sure that to the extent required, you have secured any appropriate licenses for such use. Current Procedural Terminology (CPT) is copyright 2017 American Medical Association. All Rights Reserved. No fee schedules, basic units, relative values, or related listings are included in CPT. The AMA assumes no liability for the data contained herein. Applicable FARS/DFARS restrictions apply to government use. CPT® is a trademark of the American Medical Association. The use of specific product names is illustrative only. It is not intended to be a recommendation of one product over another, and is not intended to represent a complete listing of all products available.

09-J2000-28

Original Effective Date: 04/01/15

Reviewed: 02/08/17

Revised: 03/15/17

Subject: Tiopronin (Thiola®) Tablet

THIS MEDICAL COVERAGE GUIDELINE IS NOT AN AUTHORIZATION, CERTIFICATION, EXPLANATION OF BENEFITS, OR A GUARANTEE OF PAYMENT, NOR DOES IT SUBSTITUTE FOR OR CONSTITUTE MEDICAL ADVICE. ALL MEDICAL DECISIONS ARE SOLELY THE RESPONSIBILITY OF THE PATIENT AND PHYSICIAN. BENEFITS ARE DETERMINED BY THE GROUP CONTRACT, MEMBER BENEFIT BOOKLET, AND/OR INDIVIDUAL SUBSCRIBER CERTIFICATE IN EFFECT AT THE TIME SERVICES WERE RENDERED. THIS MEDICAL COVERAGE GUIDELINE APPLIES TO ALL LINES OF BUSINESS UNLESS OTHERWISE NOTED IN THE PROGRAM EXCEPTIONS SECTION.

           
Dosage/ Administration Position Statement Billing/Coding Reimbursement Program Exceptions Definitions
           
Related Guidelines Other References Updates  
           

DESCRIPTION:

Tiopronin (Thiola) is indicated for the prevention of cystine (kidney) stone formation in patients with severe homozygous cystinuria with urinary cystine greater than 500 mg/day, who are resistant to treatment with conservative measures of high fluid intake, alkali and diet modification, or who have adverse reactions to d-penicillamine.

Cystine stones typically occur in approximately 10,000 persons in the United States who are homozygous for cystinuria. Cystinuria is an autosomal recessive disease formally classified into three subtypes. Clinical classification requires the measurement of urinary cystine levels, and those with homozygous cystinuria achieve a urinary cystine concentration of greater than 400 mg/day.

The goal of therapy is to reduce urinary cystine concentration below its solubility limit. It may be accomplished by dietary means aimed at reducing cystine synthesis and by a high fluid intake in order to increase urine volume and thereby lower cystine concentration. Unfortunately, the above conservative measures alone may be ineffective in controlling cystine stone formation in some homozygous patients with severe cystinuria (urinary cystine exceeding 500 mg/day). In such patients, d-penicillamine has been used as an additional therapy.

POSITION STATEMENT:

Initiation of tiopronin (Thiola) meets the definition of medical necessity when ALL of the following criteria are met:

1. Member is diagnosed with homozygous cystinuria

2. Member’s urinary cystine concentration is greater than 500 mg/day – laboratory documentation must be provided

3. Member is unresponsive to dietary modifications

4. Member is unresponsive to urinary alkalinization

5. Tiopronin is prescribed by (or in consultation with) a nephrologist or urologist

6. Use is not in combination with D-penicillamine

7. Dose does not exceed 2000 mg/day – dosage will be achieved using the fewest number of tablets per day

Approval duration: 6 months

Continuation of tiopronin (Thiola) meets the definition of medical necessity for the treatment of cystinuria when ALL of the following criteria are met:

1. The member has been previously approved by Florida Blue or another health plan in the past 2 years, OR the member has previously met all indication-specific criteria for coverage

2. Member demonstrates compliance (as evidenced by paid claims) with tiopronin therapy

3. Dose does not exceed 2000 mg/day – dosage will be achieved using the fewest number of tablets per day

Approval duration: 1 year

DOSAGE/ADMINISTRATION:

THIS INFORMATION IS PROVIDED FOR INFORMATIONAL PURPOSES ONLY AND SHOULD NOT BE USED AS A SOURCE FOR MAKING PRESCRIBING OR OTHER MEDICAL DETERMINATIONS. PROVIDERS SHOULD REFER TO THE MANUFACTURER’S FULL PRESCRIBING INFORMATION FOR DOSAGE GUIDELINES AND OTHER INFORMATION RELATED TO THIS MEDICATION BEFORE MAKING ANY CLINICAL DECISIONS REGARDING ITS USAGE.

FDA-approved

For the prevention of cysteine stone formation in patients with severe homozygous cystinuria with urinary cysteine greater than 500 mg/day, who are resistant to treatment with conservative measures of high fluid intake, alkali and diet modification, or who have adverse reactions to d-penicillamine.

• 800 mg/day, in three divided doses; adjust to therapeutic effect

Dose Adjustments

• Refer to prescribing information

Drug Availability

• 100 mg tablet

PRECAUTIONS:

Boxed Warning

None

Contraindications

• Pregnancy is a contraindication except in those with severe cystinuria when the potential benefit outweighs the risk of therapy.

• Nursing mothers should not use due to excretion in milk the potential risk to the fetus.

Do not reinitiate therapy in patients who have previously experienced aplastic anemia, agranulocytosis, or thrombocytopenia.

Precautions/Warnings

• Aplastic anemia

• Agranulocytosis

• Thrombocytopenia

Goodpasture’s syndrome

• Myasthenia gravis

• Proteinuria

BILLING/CODING INFORMATION:

The following codes may be used to describe:

HCPCS Coding

J8499

Prescription drug, oral, non-chemotherapeutic, Not Otherwise Specified

ICD-10 Diagnoses Codes That Support Medical Necessity

E72.01

Cystinuria

REIMBURSEMENT INFORMATION:

Refer to section entitled POSITION STATEMENT.

PROGRAM EXCEPTIONS:

Federal Employee Program (FEP): Follow FEP guidelines.

State Account Organization (SAO): Follow SAO guidelines.

Medicare Part D: BCBSF has delegated to Prime Therapeutics authority to make coverage determinations for the Medicare Part D services referenced in this guideline.

Medicare Advantage: No National Coverage Determination (NCD) and/or Local Coverage Determination (LCD) were found at the time of the last guideline revised date.

DEFINITIONS:

None

RELATED GUIDELINES:

None

OTHER:

None

REFERENCES:

  1. AHFS Drug Information. Bethesda (MD): American Society of Health-System Pharmacists, Inc; 2015 [cited 2017 Jan 18]. In: STAT!Ref Online Electronic Medical Library [Internet]. Available from: http://online.statref.com/.
  2. Biyani, Chandra S., and Jon J. Cartledge. "Cystinuria—diagnosis and management." eau-ebu update series 4.5 (2006): 175-183.
  3. Clinical Pharmacology [Internet]. Tampa (FL): Gold Standard, Inc.; 2016 [cited 2017 Jan 18]. Available from: http://www.clinicalpharmacology.com/.
  4. ClinicalTrials.gov [Internet]. Bethesda (MD): National Library of Medicine; 2000 Feb 29 - [cited 2017 Jan 23]. Available from: http://clinicaltrials.gov/.
  5. DRUGDEX® System [Internet]. Greenwood Village (CO): Thomson Micromedex; Updated periodically [cited 2017 Jan 18]. Available from: http://www.thomsonhc.com/.
  6. Mission Pharmaceutical Company. Thiola (tiopronin) tablet, coated. 2012 [cited 2017 Jan 23In: DailyMed [Internet]. Bethesda (MD): National Library of Medicine. Available from: http://dailymed.nlm.nih.gov/dailymed/drugInfo.cfm?setid=494a714e-923c-cd57-df6c-12886afb265a
  7. Orphan Drug Designations and Approval [Internet]. Silver Spring (MD): US Food and Drug Administration; 2016 [cited 2017 Jan 18]. Available from: http://www.accessdata.fda.gov/scripts/opdlisting/oopd/index.cfm/.
  8. Pearle MS, Goldfarb DS, Assimos DG, et al; American Urological Association (AUA); 2014. Medical management of kidney stones.

COMMITTEE APPROVAL:

This Medical Coverage Guideline (MCG) was approved by the BCBSF Pharmacy Policy Committee on 02/08/17.

GUIDELINE UPDATE INFORMATION:

03/15/15

New Medical Coverage Guideline.

11/01/15

Revision: ICD-9 Codes deleted.

03/15/16

Review and revision to guideline; consisting of updating position statement, precautions, and references.

03/15/17

Review and revision to guideline; consisting of updating position statement, coding and references.

Date Printed: August 23, 2017: 06:08 AM