Prior Authorization of Sickle Cell Anemia Agents - Pharmacy Services
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This bulletin updates prior authorization requirements and medical necessity guidelines for sickle cell anemia agents for providers and pharmacies enrolled in Pennsylvania Medical Assistance; it applies to fee-for-service and managed care delivery systems and affects prescribers and licensed pharmacies serving MA beneficiaries.
Siklos (hydroxyurea) tablet has been moved from non-preferred to preferred on the Statewide Preferred Drug List with an age restriction and prior authorization will continue for beneficiaries 18 years and older.
Guidance added for prior authorization of non-preferred hydroxyurea formulations when beneficiary has therapeutic failure, contraindication, intolerance, or cannot obtain a dose consistent with FDA labeling or compendia.
Coverage Criteria
Initial Therapy — Covered when ALL of the following are met
Covered when ALL of the following are met
Follow SECTION I procedures for submission.
Continuation / Renewal Therapy — Renewal covered when ALL of the following are met
Renewal covered when ALL of the following are met
Follow SECTION I procedures for submission and include documentation of clinical response.
Prescriptions for Sickle Cell Anemia Agents that meet any of the following conditions must be prior authorized: (1) a non-preferred agent (see the Statewide Preferred Drug List for the list of preferred products); (2) any agent prescribed in a quantity that exceeds the applicable quantity limit (see the Quantity Limits Chapter for limits and submission guidance); and (3) a prescription for Siklos (hydroxyurea) tablet when prescribed for a beneficiary who is 18 years of age or older.
Prior authorization personnel will evaluate requests against the clinical review guidelines in Section B. If the guidelines are not met on initial review, the request will be referred to a physician reviewer for a medical necessity determination. The physician reviewer may approve the request when, in their professional judgment, the services are medically necessary; otherwise the request may be denied.
Provider Actions & Authorization Requirements
Prior authorization required for non-preferred agents, quantity-limit exceedance, and Siklos (age ≥18)
Prior authorization is required for any non-preferred Sickle Cell Anemia Agent, for prescriptions that exceed the applicable quantity limit, and for Siklos (hydroxyurea) tablet when prescribed for beneficiaries aged 18 years or older. Submit requests following the procedures in SECTION I of the Prior Authorization Handbook.
- Non-preferred agents: see the Preferred Drug List (PDL).
- Quantity limits: see the Quantity Limits Chapter/website for drug-specific limits.
- Siklos tablet: PA required for beneficiaries ≥18 years.
Hydroxyurea trial required before Adakveo or L‑glutamine
Prior authorization for Adakveo (crizanlizumab-tmca) and L-glutamine powder requires documentation that the beneficiary has a history of therapeutic failure of, or a contraindication or intolerance to, maximum tolerated doses of hydroxyurea for at least six months before approval will be granted.
- Therapeutic failure/contraindication/intolerance must be to maximum tolerated hydroxyurea doses.
- Minimum hydroxyurea trial duration before these agents: at least six months.
Documentation required with PA submission (follow SECTION I)
Prior authorization requests must follow the procedures in SECTION I of the Prior Authorization Handbook and include supporting documentation demonstrating the FDA‑approved or medically accepted indication, age‑appropriateness, dose consistent with FDA/compendia/literature, prescribing or consultation by a hematologist/oncologist or sickle cell disease specialist, and any additional evidence required for exceptions (e.g., hydroxyurea trial history or inability to obtain appropriate dose).
- Evidence of indication per FDA labeling or medically accepted sources.
- Age and dose documentation consistent with FDA, compendia, or peer‑reviewed literature.
- Specialist prescribing or consultation (hematologist/oncologist or sickle cell disease specialist).
- For non-preferred hydroxyurea agents: documentation of therapeutic failure, contraindication, intolerance, or inability to obtain an appropriate dose with preferred formulations.
Requests failing Section B guidelines will be referred and may be denied
If a prior authorization request does not meet the clinical guidelines in Section B, it will be referred to a physician reviewer for a medical necessity determination and may be denied unless the physician reviewer determines the services are medically necessary.
- Section B clinical guidelines will be applied by PA personnel before referral.
- Referral to physician reviewer may result in denial if medical necessity is not established.
Definitions
Initial Prior Authorization Criteria
inv-12: Renewal of prior authorization
Follow SECTION I procedures for submission.
Continuation / Renewal Criteria
inv-12: Renewal of prior authorization
Follow SECTION I procedures for submission.
Step Therapy Requirements
| Step | Requirement | Applies to |
|---|---|---|
| 1 | History of therapeutic failure of, contraindication to, or intolerance of maximum tolerated doses of hydroxyurea for at least 6 months | Adakveo (crizanlizumab-tmca); L‑glutamine powder |
| 2 | For Siklos (hydroxyurea) tablet for beneficiaries ≥18 years: unable to obtain a hydroxyurea dose consistent with FDA labeling/compendia/literature with the preferred hydroxyurea capsule | Siklos (hydroxyurea) tablet — beneficiaries 18+ |
| 3 | For non‑preferred hydroxyurea agents: documentation of either (a) therapeutic failure/contraindication/intolerance to preferred hydroxyurea agents that would not be expected with the requested drug, OR (b) inability to obtain an appropriate dose with preferred hydroxyurea agents | Non‑preferred hydroxyurea agents |
Quantity Limits
Background
Hydroxyurea is a long-established, disease-modifying therapy for sickle cell disease and remains a foundational treatment option. Newer agents such as Adakveo (crizanlizumab‑tmca) and L‑glutamine powder are additional therapeutic options for patients who fail or cannot tolerate hydroxyurea; however, coverage for some of these agents requires prior authorization and adherence to step-therapy expectations that reflect a trial of hydroxyurea or an appropriate clinical justification for not using hydroxyurea.
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