CurrentSecurity Health Plan of Wisconsin, Inc.Policy IC-0677

Skysona® (elivaldogene autotemcel) for Cerebral X-Linked Adrenoleukodystrophy

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This policy governs prior authorization, medical-necessity criteria, dosing, administration, and billing for Skysona intravenous gene therapy in pediatric male patients with active cerebral X-linked adrenoleukodystrophy (CALD).

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicySkysona® (elivaldogene autotemcel) for Cerebral X-Linked Adrenoleukodystrophy

Policy CodePolicy IC-0677

Change TypeNo material clinical or coverage changes

Effective DateN/A

Last ReviewFeb 3, 2026

Next Review DateN/A

Key ActionSubmit prior authorization with required clinical documentation; authorization is limited to one treatment course consisting of one dose and cannot be renewed.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

1 doseInitial authorization duration

1 unitMaximum units for one dose

4 to <18Required patient age range

Loes 0.5-9Required active CNS disease MRI score

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CurrentSecurity Health Plan of Wisconsin, Inc.Policy IC-0677

Skysona® (elivaldogene autotemcel) for Cerebral X-Linked Adrenoleukodystrophy

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Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicySkysona® (elivaldogene autotemcel) for Cerebral X-Linked Adrenoleukodystrophy

Policy CodePolicy IC-0677

Change TypeNo material clinical or coverage changes

Effective DateN/A

Last ReviewFeb 3, 2026

Next Review DateN/A

Key ActionSubmit prior authorization with required clinical documentation; authorization is limited to one treatment course consisting of one dose and cannot be renewed.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

1 doseInitial authorization duration

1 unitMaximum units for one dose

4 to <18Required patient age range

Loes 0.5-9Required active CNS disease MRI score

Required neurologic function score

J3387Covered billing code

Medical Necessity and Eligibility

Initial Approval: General Eligibility

Covered when ALL of the following general eligibility conditions are met:

ALL of the following

Patient is a male at least 4 years of age and less than 18 years of age.
Patient has been screened for hepatitis B virus (HBV), hepatitis C virus (HCV), human immunodeficiency virus 1 & 2 (HIV-1/HIV-2), and human T-lymphotrophic virus 1 & 2 (HTLV1/HTLV-2) in accordance with clinical guidelines prior to collection of cells for manufacturing.
Patient does not have an active infection, including clinically important localized infections.
Patient will be administered prophylaxis for infection according to best clinical practices and guidelines.
Vaccinations will not be administered within the 6 weeks prior to the start of myeloablative conditioning and until hematological recovery following treatment.
Provider will confirm that patient is up to date with all age-appropriate vaccinations, in accordance with current vaccination guidelines, prior to initiating therapy.
Used as single agent therapy, not applicable to lymphodepleting or bridging therapy while awaiting manufacture.
Patient will receive periodic life-long monitoring for hematological malignancies, such as myelodysplastic syndrome (MDS) and acute myeloid leukemia (AML).
Patient does not have a full ABCD1 gene deletion.
Patient will avoid concomitant therapy with anti-retroviral medications for at least one month prior to initiating medications for stem cell mobilization and for the expected duration for elimination of the medications, and until all cycles of apheresis are completed. If a patient requires antiretroviral medication for HIV prophylaxis, a negative HIV test must be confirmed before beginning mobilization.
Patient does not have disease secondary to head trauma.
Therapy will not be used to prevent the development of or treat adrenal insufficiency due to adrenoleukodystrophy.
Patient is eligible to undergo hematopoietic stem cell transplant (HSCT) and has not had a prior allogeneic HSCT.
Eligibility for allogeneic HCT is not absolute and varies by center; in general, it includes functional capacity, organ function, social support, and related criteria.
Males capable of fathering a child and their female partners of childbearing potential should use an effective method of contraception, such as an intrauterine device or combination of hormonal and barrier contraception, from the start of mobilization through at least 6 months after administration of Skysona.

Initial Approval: Cerebral Adrenoleukodystrophy (CALD) Disease Criteria

Patient must have a documented diagnosis of cerebral adrenoleukodystrophy (CALD) and meet ALL of the following disease-specific criteria:

ALL of the following

Both laboratory/genetic findings are required
- ONE of the following elevated VLCFA findings
  - Elevated very long chain fatty acids (VLCFA) confirmed by plasma C26:0-lysophosphatidylcholine (C26:0-LPC) level.
  - Elevated very long chain fatty acids (VLCFA) confirmed by fasting plasma VLCFA levels: C26:0, ratio of C24:0 to C22:0, and ratio of C26:0 to C22:0.
- Pathogenic variants in the ABCD1 gene detected by genetic testing.
Active CNS disease on central radiographic review

Renewal Criteria

Renewal is not available.

Duration of authorization has not been exceeded; however, renewal authorization is not available under this policy.

Coverage is not available when the patient has a full ABCD1 gene deletion, disease secondary to head trauma, or when Skysona is being used to prevent the development of or treat adrenal insufficiency due to adrenoleukodystrophy.

The patient must be eligible to undergo hematopoietic stem cell transplant (HSCT) and must not have had a prior allogeneic HSCT.

Concomitant antiretroviral therapy is prohibited for at least one month before initiating medications for stem cell mobilization, throughout the expected duration required for elimination of the medications, and until all cycles of apheresis are completed. If antiretroviral medication is required for HIV prophylaxis, a negative HIV test must be confirmed before mobilization begins.

Billing and Clinical Coding

HCPCS Code(s)HCPCSCovered

J3387

Injection, elivaldogene autotemcel, per treatment

NDCNDCCovered

73554-2111xx

Skysona up to 2 infusion bags, 20 mL/infusion bag, overwrap, and metal cassette

Covered Diagnosis CodesICD-10Covered

E71.511	Neonatal adrenoleukodystrophy
E71.520	Childhood cerebral X-linked adrenoleukodystrophy
E71.521	Adolescent X-linked adrenoleukodystrophy
E71.528	Other X-linked adrenoleukodystrophy
E71.529	X-linked adrenoleukodystrophy, unspecified type

Loes Score

Required Loes scoreBetween 0.5 and 9, inclusive

Scale34-point brain MRI scale

Clinical useQuantifies the extent of cerebral demyelinating disease and establishes active CNS disease when within the policy range

Neurologic Function Score

Required NFS1 or less (≤ 1)

AssessmentNeurologic Function Score

Corresponding disease status

Authorization, Documentation, and Denial Risks

Prior Authorization

One-Time Prior Authorization

Prior authorization is required for Skysona. Initial authorization is limited to one treatment course consisting of one dose and is not renewable.

Prior authorization validity is provided initially for one treatment course (1 dose).
Prior authorization may not be renewed.

Documentation Required

Required Clinical Documentation

Submit supporting clinical documentation with every authorization request. Records are reviewed at submission to evaluate medical necessity and should be provided by direct upload through the prior authorization web portal or by fax.

Medical records and chart notes
Laboratory results and confirmatory diagnostics
Documentation related to diagnosis, step therapy, and clinical markers, including genetic and mutational testing when applicable

Therapy and Disease Overview

Skysona (elivaldogene autotemcel) is an autologous hematopoietic stem-cell gene therapy for cerebral X-linked adrenoleukodystrophy (CALD), a disorder associated with ABCD1 pathogenic variants and progressive central nervous system demyelination. The policy applies to boys with active, early-stage cerebral disease and limited neurologic impairment.

Clinical Terms and Scales

Loes Score

DefinitionA 34-point brain MRI scale used to quantify the extent of cerebral demyelinating disease

Policy rangeThe score must be between 0.5 and 9, inclusive

Required imaging contextThe qualifying score must be demonstrated by central radiographic review of brain MRI showing active CNS disease

Neurologic Function Score (NFS)

DefinitionA neurologic assessment score used to evaluate neurologic function

Policy thresholdThe score must be 1 or less (≤ 1)

Clinical interpretation

Diagnostic Biomarkers

ABCD1 Pathogenic Variant

Required findingA pathogenic variant in the ABCD1 gene

Detection methodDetected by genetic testing

Clinical purposeConfirms cerebral X-linked adrenoleukodystrophy as part of the diagnostic requirements

Very Long Chain Fatty Acids (VLCFA)

Required biochemical findingElevated very long chain fatty acids (VLCFA)

Acceptable pathway 1Plasma C26:0-lysophosphatidylcholine (C26:0-LPC) level

Acceptable pathway 2

Covered Treatment

Regimen	Administration	Coverage criteria
Skysona (elivaldogene autotemcel) as single-agent autologous gene therapy	One intravenous treatment course; minimum dose 5.0 × 10^6 CD34+ cells/kg	Covered for male patients ages 4 to less than 18 years with documented cerebral X-linked adrenoleukodystrophy, pathogenic ABCD1 variants, elevated VLCFA, active CNS disease on MRI with a Loes score of 0.5–9 inclusive and gadolinium enhancement, and a Neurologic Function Score of ≤1, when applicable transplant eligibility and other policy requirements are met

Policy Summary

PayerSecurity Health Plan of Wisconsin, Inc.

PolicySkysona® (elivaldogene autotemcel) for Cerebral X-Linked Adrenoleukodystrophy

Policy CodePolicy IC-0677

Change TypeNo material clinical or coverage changes

Effective DateN/A

Last ReviewFeb 3, 2026

Next Review DateN/A

Key ActionSubmit prior authorization with required clinical documentation; authorization is limited to one treatment course consisting of one dose and cannot be renewed.

SourceLink

On This Page

Loes score between 0.5 and 9, inclusive, on the 34-point scale.

Gadolinium enhancement on MRI of demyelinating lesions.

Neurologic Function Score (NFS) is less than or equal to 1, indicating asymptomatic or mildly symptomatic disease.

Patients with isolated pyramidal tract disease will be reviewed on a case-by-case basis.

Asymptomatic or mildly symptomatic disease

Skysona Dose

Minimum recommended dose5.0 x 10^6 CD34+ cells/kg

AdministrationCD34+ cells in one or two infusion bags

Weight basisDose is calculated using the patient's weight before the first apheresis

Manufacturing CD34+ Cell Collection

Minimum target collection12 x 10^6 CD34+ cells/kg

Collection purposeCD34+ cells for Skysona product manufacturing

Collection processHematopoietic stem-cell mobilization followed by apheresis

Backup CD34+ Collection by Apheresis

Minimum backup collection≥ 1.5 x 10^6 CD34+ cells/kg

Collection methodApheresis

Storage requirementCells must be collected from the patient and cryopreserved before conditioning and Skysona infusion

Backup Collection by Bone Marrow Harvest

Minimum backup collection≥ 1.0 x 10^8 total nucleated cells/kg

Collection methodBone marrow harvest

Storage requirementCells must be collected from the patient and cryopreserved before conditioning and Skysona infusion

Documentation Required

CALD Diagnostic and Neurologic Records

For cerebral adrenoleukodystrophy (CALD), document the diagnosis and neurologic disease status with the required biochemical, genetic, imaging, and neurologic records.

Elevated very long chain fatty acids (VLCFA), demonstrated by a plasma C26:0-lysophosphatidylcholine (C26:0-LPC) level or fasting plasma VLCFA levels including C26:0, the C24:0/C22:0 ratio, and the C26:0/C22:0 ratio
Pathogenic ABCD1 gene variant confirmed by genetic testing
Central radiographic review of brain MRI showing active CNS disease, with a Loes score of 0.5–9 inclusive and gadolinium enhancement of demyelinating lesions
Neurologic Function Score (NFS) ≤ 1; isolated pyramidal tract disease is reviewed case-by-case

Documentation Required

Treatment Eligibility Documentation

Document that all initial-approval eligibility conditions are satisfied before treatment. The patient must be a qualifying candidate for Skysona and the required infection, vaccination, treatment, genetic, transplant, monitoring, and reproductive-safety conditions must be addressed.

Male patient at least 4 years old and younger than 18 years old
Screened for HBV, HCV, HIV-1/HIV-2, and HTLV-1/HTLV-2 before cell collection; no active infection, including clinically important localized infection
Infection prophylaxis will be administered according to clinical guidelines
No vaccinations within 6 weeks before myeloablative conditioning or until hematologic recovery; patient is up to date on age-appropriate vaccinations before therapy
Used as single-agent therapy, excluding lymphodepleting or bridging therapy while awaiting manufacture
Periodic lifelong monitoring for hematologic malignancies, including MDS and AML, will be provided
No full ABCD1 gene deletion
No concomitant antiretroviral therapy from at least 1 month before stem-cell mobilization through the required elimination period and completion of apheresis; if antiretroviral therapy is required for HIV prophylaxis, confirm a negative HIV test before mobilization
Disease is not secondary to head trauma and treatment is not intended to prevent or treat adrenal insufficiency due to adrenoleukodystrophy
Eligible for hematopoietic stem cell transplant (HSCT) and no prior allogeneic HSCT
Males capable of fathering a child and their female partners of childbearing potential will use effective contraception from mobilization through at least 6 months after Skysona administration

Denial Risk

Failure to Establish Medical Necessity

Failure to submit the required records or establish each initial-approval condition may result in denial because medical necessity cannot be established under the policy.

Missing medical records, chart notes, laboratory results, confirmatory diagnostics, or other supporting documentation
Failure to document diagnosis, step therapy, or applicable genetic, mutational, and other clinical markers
Failure to satisfy the required age, infection screening, vaccination, prophylaxis, treatment-use, genetic, transplant, monitoring, or contraception conditions

Denial Risk

CALD Criteria Not Met

Authorization may be denied when the CALD diagnostic, CNS disease, imaging, or neurologic criteria are not met or cannot be documented.

CALD is not established by the required elevated VLCFA findings and pathogenic ABCD1 variant
MRI does not demonstrate active CNS disease with a Loes score of 0.5–9 inclusive and gadolinium enhancement of demyelinating lesions
Neurologic Function Score is greater than 1, subject to case-by-case review of isolated pyramidal tract disease
Required diagnostic or neurologic records are absent or do not support the policy criteria

Denial Risk

No Renewal or Additional Treatment Course

Authorization is limited to one treatment course. Do not submit a renewal request or request an additional course after the authorized duration has been used.

Renewal is not permitted.
Authorization duration must not exceed the one-course limit described in the policy.

Billing Rule

Units Exceed Limit

Billing more than one billable unit exceeds the policy dosing limit for Skysona.

Maximum: 1 billable HCPCS unit for one dose

A score of 1 or less corresponds to asymptomatic or mildly symptomatic disease

Allogeneic HSCT

DefinitionHematopoietic stem-cell transplantation using cells from a donor rather than the patient

Eligibility requirementThe patient must be eligible to undergo HSCT

Prior-transplant requirementThe patient must not have had a prior allogeneic HSCT

Fasting plasma VLCFA levels including C26:0, the C24:0/C22:0 ratio, and the C26:0/C22:0 ratio