Blue Cross Blue Shield MA specialty drug Coverage Update

Coverage Criteria

Adakveo (crizanlizumab) Initial Therapy

Covered when ALL of the following are met

Adakveo initial: Patient is ≥16 years old; not receiving regularly scheduled blood (RBC) transfusion therapy; documented diagnosis of sickle cell disease (including HbSS, HbSC, HbSβ0-thalassemia or HbSβ+-thalassemia); has experienced between 2 and 10 sickle cell-related pain crises (vaso-occlusive crises) within the preceding 12 months; hemoglobin >4.0 g/dL.2-10 VOCs in prior 12 months; Hb >4.0 g/dL

If met, initial approval up to 6 months.

Adakveo (crizanlizumab) Continuation Therapy

Covered for continuation when ALL of the following are met

Adakveo continuation: Documentation demonstrating decreased number of vaso-occlusive crises (VOCs) from the original 6-month period or continued decreased VOCs achieved from the original 6-month period.clinical documentation of VOC reduction

If approved, authorization may be for up to 12 months.

Reblozyl (luspatercept) Initial Therapy - Transfusion-dependent beta thalassemia

Covered when ALL of the following are met

Reblozyl (transfusion-dependent beta thalassemia): Patient is ≥18 years old; does not have Hemoglobin S/B-thalassemia or alpha-thalassemia; documented diagnosis of beta-thalassemia or Hemoglobin E/beta-thalassemia; regularly transfused defined as 6-20 red blood cell (RBC) units in the most recent 24 weeks; no transfusion-free period ≥35 days during the most recent 24 weeks; platelet count <1000 x 10^9/L.6-20 RBC units in 24 weeks; no ≥35 day transfusion-free period

Initial approval per policy guidance.

Reblozyl (luspatercept) Initial Therapy - MDS

Covered when ALL of the following are met

Reblozyl (MDS, ESA-naive): Patient is ≥18 years old; confirmed diagnosis of very low- to intermediate-risk myelodysplastic syndromes (MDS); has not used prior erythropoiesis-stimulating agents (ESA-naive).age ≥18; ESA-naive

Policy includes other MDS-related indications per documentation.

Reblozyl (luspatercept) Continuation Therapy

Covered for continuation when ALL of the following are met

Reblozyl continuation: Documentation showing decreased RBC transfusions from the original 12-week period or continued decreased utilization of RBC transfusions achieved from the original 12-week period.reduction in transfusion requirement

If continuation approved, authorization may be for up to 12 months.

Oxbryta (voxelotor) Initial Therapy

Covered when ALL of the following are met

Oxbryta (voxelotor) initial: Patient is ≥4 years old; documented diagnosis of sickle cell disease; not receiving regularly scheduled blood (RBC) transfusion therapy; hemoglobin ≥5.5 and <10.5 g/dL.age ≥4; Hb ≥5.5 and <10.5 g/dL

If met, initial approval up to 6 months.

Oxbryta (voxelotor) Continuation Therapy

Covered for continuation when ALL of the following are met

Oxbryta continuation: Documentation demonstrating increase in hemoglobin of >1.0 g/dL from the original 6-month period or continued hemoglobin increase achieved from the original 6-month period.Hb increase >1.0 g/dL

If continuation approved, authorization may be for up to 12 months.

We do not cover the medications listed in this policy for conditions other than those explicitly listed. Requests for these products must meet the specific diagnosis and clinical entry criteria described in the policy for Reblozyl (luspatercept), Adakveo (crizanlizumab), and Oxbryta (voxelotor); use of these agents for other indications is not covered.

Requests based exclusively on use of drug samples are insufficient to meet coverage criteria or to support an exception. Additional clinical documentation demonstrating medical necessity must be submitted by the requesting prescriber for review.

Coding and Clinical Thresholds

CPT/HCPCS/ICD guidancemixed

No codes listed

Regular transfusion units — 6-20 units in most recent 24 weeks

DefinitionRegular transfused (Reblozyl) = 6–20 red blood cell (RBC) units in the most recent 24 weeks.

Transfusion frequency windowCounting period = most recent 24 weeks (168 days).

Transfusion-free period restrictionNo transfusion-free period ≥35 days during the most recent 24 weeks when qualifying as 'regularly transfused'.

Vaso-occlusive crises (VOCs) — 2-10 VOCs in preceding 12 months

Required VOCsMember must have experienced between 2 and 10 sickle cell–related pain crises (VOCs) in the preceding 12 months.

Applicable therapy

Provider Actions & Submission Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for the products referenced in this policy. Submit prior authorization requests to Blue Cross Blue Shield of Massachusetts Pharmacy Operations. Phone: 1-800-366-7778. Fax: 1-800-583-6289. Providers may also mail the completed Formulary Exception / Prior Authorization form to: Blue Cross Blue Shield of Massachusetts Pharmacy Operations Department, 25 Technology Place, Hingham, MA 02043.

Affected products: Reblozyl (luspatercept) — PA Required; Adakveo (crizanlizumab) — PA Required; Oxbryta (voxelotor) — PA Required
Contact: Pharmacy Operations Tel: 1-800-366-7778; Fax: 1-800-583-6289; Mailing address: 25 Technology Place, Hingham, MA 02043
Applies to commercial products including Managed Care (HMO/POS), PPO, Indemnity, MEDEX with Rx, Managed Major Medical with Custom BCBSMA Formulary

Step Therapy

Formulary Exception / Step Requirement

For formulary exception or non-formulary requests, the prescriber must document prior treatment failure with, or contraindication to, two covered formulary alternatives when such alternatives are available. Requests that do not meet the clinical criteria in this policy may be considered under Individual Consideration; include supporting clinical rationale and documentation.

Initial Therapy

Initial therapy — product-specific entry criteria for initial coverage

Initial coverage is granted when product-specific entry criteria are met as detailed below.

Adakveo initial (entry criteria): Patient is ≥16 years old; documented diagnosis of sickle cell disease; not receiving regularly scheduled RBC transfusion therapy; 2-10 VOCs in the preceding 12 months; hemoglobin >4.0 g/dL.2-10 VOCs in prior 12 months; Hb >4.0 g/dL

Initial approval up to 6 months.

Reblozyl initial (entry criteria): Patient is ≥18 years old; diagnosis consistent with transfusion-dependent beta thalassemia or qualifying MDS indications; regularly transfused defined as 6-20 RBC units in the most recent 24 weeks with no transfusion-free period ≥35 days (for transfusion-dependent indication); ESA-naive for the specified MDS indication.6-20 RBC units/24 weeks or ESA-naive for MDS

See product-specific sets for details.

Continuation Requirements

Continuation requirements — documentation of clinical benefit vs baseline required for continuation approvals

Continuation approvals require documentation of clinical benefit compared to baseline from the original approval period.

Adakveo continuation (documentation required): Submit documentation demonstrating a decrease in vaso-occlusive crises (VOCs) from the original 6-month approval period or maintenance of decreased VOCs compared to baseline.Clinical documentation of VOC reduction vs baseline

If approved, authorization may be for up to 12 months.

Reblozyl continuation (documentation required): Submit documentation demonstrating decreased RBC transfusion requirements from the original 12-week period or continued decreased utilization achieved during the original 12-week period.Reduction in transfusion frequency vs baseline

If approved, authorization may be for up to 12 months.

Definitions

Regularly scheduled blood (RBC) transfusion therapy — chronic/prophylactic transfusion definition and relevance to exclusions

DefinitionRegularly scheduled blood (RBC) transfusion therapy = chronic, prophylactic, or preventive transfusion regimen; used as an exclusion for some drug coverage criteria.

Relevance to Adakveo and OxbrytaPatients receiving regularly scheduled RBC transfusions are excluded from initial therapy eligibility for Adakveo and Oxbryta per policy criteria.

TerminologyAlso termed chronic, prophylactic, or preventive transfusion in the policy language.

Regularly transfused (Reblozyl) — defined as 6-20 RBC units in most recent 24 weeks with no transfusion-free period ≥35 days

Definition (Reblozyl)Regularly transfused = 6–20 RBC units in the most recent 24 weeks with no transfusion-free period ≥35 days during that time.

Site of Care

Note

Pharmacy site of care — applies to outpatient retail pharmacy with pharmacy coverage

This policy and prior authorization requirements apply when the member has pharmacy coverage and requests are submitted for outpatient retail pharmacy.

Submit requests under the outpatient pharmacy benefit when the member has pharmacy coverage

Step Therapy Requirements

Requirement	Details / evidence needed
Demonstrate prior failures of two covered formulary alternatives (when available)
Documentation of prior treatment failures or contraindications to two covered formulary alternatives must be submitted when requesting a non-formulary or non-covered medication
Requests based exclusively on samples are insufficient
Clinical information beyond sample use must be provided to demonstrate medical necessity (e.g., relevant baseline and follow-up measures)

Background

This policy covers therapies for two related groups of hematologic conditions. Sickle cell disease is addressed by Adakveo (crizanlizumab) to reduce vaso-occlusive crises and by Oxbryta (voxelotor) to increase hemoglobin in eligible patients. Transfusion-dependent anemias, including transfusion-dependent beta-thalassemia and certain myelodysplastic syndromes, are addressed by Reblozyl (luspatercept) for reduction of transfusion requirements in eligible adults.

Revision History

2024-01-01material_policy_changeLatest

Updated new indications for Reblozyl.

2023-07-01formatting_update

Policy reformatted.

2022-02-01material_policy_change

Updated age range for Oxbryta (voxelotor).

OpenPayer

Outpatient specialty pharmacy coverage for Reblozyl, Adakveo, and Oxbryta

Coverage Criteria

Coding and Clinical Thresholds

Provider Actions & Submission Requirements

Initial Therapy

Continuation Requirements

Definitions

Site of Care

Step Therapy Requirements

Background

Revision History