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Procysbi (cysteamine bitartrate) Prior Authorization Program Summary
Policy Number: PH-1113
This program applies to Blue Partner, Commercial, GenPlus, NetResults A series, SourceRx, and Health Insurance Marketplace.
POLICY REVIEW CYCLE
Effective Date |
Date of Origin |
1/1/2024 |
|
FDA APPROVED INDICATIONS AND DOSAGE
Agent(s) |
FDA Indication(s) |
Notes |
Ref# |
Procysbi® |
Treatment of nephropathic cystinosis in adults and pediatric patients 1 year of age and older |
|
1 |
See package insert for FDA prescribing information: https://dailymed.nlm.nih.gov/dailymed/index.cfm
CLINICAL RATIONALE
Cystinosis |
Cystinosis is a rare autosomal recessive lysosomal storage disorder in which cystine accumulates in lysosomes of cells. It is a systemic disease wherein cystine crystals accumulate in all body cells and tissues. Cystinosis has three major clinical presentations depending on the severity of mutations affecting the CTNS gene: the infantile nephropathic form, the juvenile nephropathic form, and the adult (non-nephropathic ocular) form. The infantile nephropathic form is the most common and severe form, occurring in over 95% of patients, with consecutively progressive loss of glomerular function leading to end-stage renal disease (ESRD). ESRD usually develops by the end of the first or second decade of life. Nearly all nephropathic cystinosis patients will develop major extra-renal symptoms including retinal, endocrine, and neuromuscular complications by 30 years of age if cystine depletion therapy is not initiated early.(2) |
Efficacy |
A multicenter, open-label, randomized clinical trial was completed comparing Procysbi to immediate-release cysteamine bitartrate (Cystagon). All patients were required to be on a stable dose of immediate-release cysteamine bitartrate prior to randomization. The study demonstrated that Procysbi administered every 12 hours was non-inferior to immediate-release cysteamine bitartrate dosed every 6 hours.(1) |
Safety |
Procysbi is contraindicated in patients with a serious hypersensitivity reaction, including anaphylaxis, to penicillamine or cysteamine.(1) |
REFERENCES
Number |
Reference |
1 |
Procysbi prescribing information. Horizon Pharma USA, Inc. February 2022. |
2 |
Elmonem MA, Veys KR, Soliman NA, van Dyck M, van den Heuvel LP, Levtchenko E. (2016) Cystinosis: A review. Orphanet J Rare Dis 11:47. DOI: 10.1186/s13023-016-0426-y |
POLICY AGENT SUMMARY PRIOR AUTHORIZATION
Target Brand Agent(s) |
Target Generic Agent(s) |
Strength |
Targeted MSC |
Available MSC |
Final Age Limit |
Preferred Status |
|
||||||
Procysbi |
cysteamine bitartrate cap delayed release |
25 MG ; 75 MG |
M ; N ; O ; Y |
N |
|
|
Procysbi |
cysteamine bitartrate delayed release granules packet |
300 MG ; 75 MG |
M ; N ; O ; Y |
N |
|
|
CLIENT SUMMARY – PRIOR AUTHORIZATION
Target Brand Agent Name(s) |
Target Generic Agent Name(s) |
Strength |
Client Formulary |
Procysbi |
cysteamine bitartrate cap delayed release |
25 MG ; 75 MG |
Blue Partner ; Commercial ; GenPlus ; Health Insurance Marketplace ; NetResults A Series ; SourceRx |
Procysbi |
cysteamine bitartrate delayed release granules packet |
300 MG ; 75 MG |
Blue Partner ; Commercial ; GenPlus ; Health Insurance Marketplace ; NetResults A Series ; SourceRx |
PRIOR AUTHORIZATION CLINICAL CRITERIA FOR APPROVAL
Module |
Clinical Criteria for Approval |
PA |
Target Agent(s) will be approved when ALL of the following are met:
Length of Approval: 12 months *Step therapy requirement may not apply if a prior health plan paid for the medication - documentation of a paid claim may be required. |
This pharmacy policy is not an authorization, certification, explanation of benefits or a contract. Eligibility and benefits are determined on a case-by-case basis according to the terms of the member’s plan in effect as of the date services are rendered. All pharmacy policies are based on (i) information in FDA approved package inserts (and black box warning, alerts, or other information disseminated by the FDA as applicable); (ii) research of current medical and pharmacy literature; and/or (iii) review of common medical practices in the treatment and diagnosis of disease as of the date hereof. Physicians and other providers are solely responsible for all aspects of medical care and treatment, including the type, quality, and levels of care and treatment.
The purpose of Blue Cross and Blue Shield of Alabama’s pharmacy policies are to provide a guide to coverage. Pharmacy policies are not intended to dictate to physicians how to practice medicine. Physicians should exercise their medical judgment in providing the care they feel is most appropriate for their patients.
Neither this policy, nor the successful adjudication of a pharmacy claim, is guarantee of payment.
BCBSAL _ Commercial _ CSReg _ Procysbi (cysteamine bitartrate) _PA _ProgSum_ 1/1/2024