CurrentCignaPolicy N/A

Sickle Cell Disease - Hydroxyurea Products Preferred Step Therapy Policy

Defines Cigna's preferred step therapy requirements for coverage of Siklos (hydroxyurea tablets) as a Step 2 product versus Step 1 hydroxyurea products (Droxia capsules) for patients with sickle cell disease; applies to Cigna-administered health benefit plans.

Policy Summary

PayerCigna

PolicySickle Cell Disease - Hydroxyurea Products Preferred Step Therapy Policy

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateSep 24, 2025

Key ActionObtain prior authorization for Siklos when a trial of a Step 1 product (Droxia) has not been completed or exception criteria are not met.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

1 yearapproval duration for authorizations

2hydroxyurea products listed

6 monthsrecommended trial duration

≥2 yrsage indication (Siklos)

Coverage Criteria for Siklos (hydroxyurea)

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COVERAGE CRITERIA

Siklos is covered as medically necessary when ALL of the following apply or when specified exceptions apply:

coverage pathways

Step therapy failure pathway: Patient has tried one Step 1 Product (Droxia).
Trial of one Step 1 product required before Step 2
Dose availability pathway: Patient requires Siklos 100 mg or 1,000 mg tablet strengths to achieve a dosage that cannot be achieved with available Droxia strengths.
Allows approval when tablet strengths needed are not achievable with capsule strengths
Swallowing impairment pathway: Patient cannot swallow or has difficulty swallowing Droxia capsules.
Swallowing inability justifies bypassing Step 1

administrative: All approvals are provided for 1 year in duration.

Per policy statement, approvals expire at 1 year

Requests for a Step 2 product (Siklos) without documentation that the patient has tried one Step 1 product (Droxia) are considered not medically necessary, unless the request meets one of the explicit exceptions. The policy allows bypassing the Step 1 trial only when the patient requires Siklos 100 mg or 1,000 mg tablet strengths that cannot be achieved with Droxia, or when the patient cannot swallow or has difficulty swallowing Droxia capsules. Prior authorization is required when the Step 1 product has not been tried and approval will be granted only when one of the stated exceptions is documented.

Referenced Product Codes & Key Values

Referenced product codesNDC

NDC	Product-specific NDCs referenced generically in policy (prescribing information cited)

Hydroxyurea clinical response trial duration

Minimum trial duration6 months at maximum tolerated dose before declaring hydroxyurea treatment failure

Expected onset of clinical response3–6 months for clinical response to hydroxyurea therapy

Long-term therapy indicationContinue hydroxyurea long-term if clinical response is achieved

Provider Actions, Prior Authorization & Documentation

Prior Authorization

Prior Authorization for Step 2 (Siklos)

Prior authorization is required for Step 2 product (Siklos) if the Preferred Step Therapy requirement (trial of a Step 1 hydroxyurea product) has not been met at the point of service. Coverage will be determined by the Preferred Step Therapy criteria; requests for a Step 2 product that do not meet the criteria are at risk for denial.

Step 2 product: Siklos

Step Therapy

Preferred Step Therapy Requirement

This program requires trial of a Step 1 hydroxyurea product (Droxia) before approving a Step 2 product (Siklos), unless one of the specified exception criteria is met. All approvals are provided for 1 year.

Required trial: one Step 1 product (Droxia)
Exceptions: see clinical criteria (e.g., dosing strength or inability to swallow Droxia)

Documentation Required

Required Documentation to Support Prior Authorization or Exception

Provider must submit documentation consistent with the applicable benefit plan and clinical information to support that a Step 1 trial was completed or that an exception applies (for example: documentation of prior Droxia trial, clinical rationale for needing Siklos strengths, or documented swallowing impairment).

Include dates and duration of prior Step 1 therapy, dosing information, and relevant clinical notes
If claiming an exception, provide supporting clinical evidence (e.g., inability to swallow capsules, dosing needs not met by Droxia strengths)

Denial Risk

Denial Risk for Unmet Step Therapy

If the Preferred Step Therapy criteria are not met at point of service and adequate documentation is not provided to demonstrate an exception, the request for a Step 2 product (Siklos) may be denied.

Denial risk applies when no documented trial of a Step 1 product (Droxia) and no qualifying exception is supplied

Product Definitions

Siklos — product summary

Indication (age)Indicated for adults and pediatric patients ≥2 years of age

Clinical usesReduce frequency of painful crises and reduce need for blood transfusions

Available strengths100 mg and 1,000 mg functionally scored tablets (can be split into 50 mg and 250 mg increments)

AdministrationTablets can be swallowed whole or dispersed immediately before use in a small quantity of water in a teaspoon

Droxia — product summary and administration

Dosage forms and strengthsAvailable as 200 mg, 300 mg, and 400 mg capsules

Swallowing instructionsCapsules should be swallowed whole and should not be opened, broken, or chewed

Comparison noteDroxia capsules are not available as scored tablets and cannot be dispersed like Siklos tablets

Background

Per National Heart, Lung, and Blood Institute guidance, the only currently proven disease‑modifying treatments for sickle cell disease are hydroxyurea and chronic blood transfusions. Hydroxyurea reduces the frequency of painful crises and the need for transfusions; clinical response can take several months, and a 6‑month trial at maximum tolerated dose is recommended before declaring treatment failure.