CurrentViva Health PlanPolicy IC-0848

Avlayah (tividenofusp alfa-eknm) Coverage Criteria

Customize your policy alerts

Sign up for vivahealthplan Policy IC-0848 alerts

Get alerted when Policy IC-0848 changes without checking for updates manually.

Monitor payer policy activity

Receive email alerts as soon as relevant payer policies are published or updated.

This policy governs prior authorization and medical necessity coverage for intravenous Avlayah (tividenofusp alfa-eknm) for members with Mucopolysaccharidosis type II (Hunter syndrome). It applies to providers seeking initial or continued therapy authorization.

Policy Summary

PayerViva Health Plan

PolicyAvlayah (tividenofusp alfa-eknm) Coverage Criteria

Policy CodePolicy IC-0848

Change TypeNo material clinical or coverage changes

Effective Date05/05/2026

Last ReviewSep 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization; initial and renewal authorization are valid for 12 months when applicable criteria are met.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsPrior authorization validity

1,800/7 daysMaximum billable units

5 kgMinimum member weight

15 mg/kg weeklyPediatric maintenance dose

E76.1

OpenPayer is powered by Trek Health's payer performance platform. Trek continuously ingests, validates, and normalizes Transparency in Coverage data alongside payer policies and other commercial payer data to create a structured payer intelligence foundation. OpenPayer uses this foundation to deliver personalized search results, dynamically generated policy pages, and tailored policy monitoring based on each user's payers, specialties, billing codes, and areas of interest. The same intelligence powers broader payer performance workflows, including reimbursement benchmarking, contract evaluation, payer negotiations, and financial decision-making.

CurrentViva Health PlanPolicy IC-0848

Avlayah (tividenofusp alfa-eknm) Coverage Criteria

Customize your policy alerts

Sign up for vivahealthplan Policy IC-0848 alerts

Get alerted when Policy IC-0848 changes without checking for updates manually.

Monitor payer policy activity

Receive email alerts as soon as relevant payer policies are published or updated.

Policy Summary

PayerViva Health Plan

PolicyAvlayah (tividenofusp alfa-eknm) Coverage Criteria

Policy CodePolicy IC-0848

Change TypeNo material clinical or coverage changes

Effective Date05/05/2026

Last ReviewSep 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization; initial and renewal authorization are valid for 12 months when applicable criteria are met.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

12 monthsPrior authorization validity

1,800/7 daysMaximum billable units

5 kgMinimum member weight

15 mg/kg weeklyPediatric maintenance dose

E76.1

Covered diagnosis code

Coverage Criteria

Initial Therapy

Covered for initial therapy when all diagnosis, universal safety, and initial approval requirements are met.

Initial Therapy Requirements

Definitive MPS II diagnosis
- Deficient or absent iduronate 2-sulfatase enzyme activity is demonstrated in white cells, fibroblasts, or plasma in the presence of normal activity of at least one other sulfatase.
- Pathogenic or likely pathogenic variants in the IDS gene are detected by molecular genetic testing.
Universal safety and concomitant therapy requirements
- Avlayah will not be used in combination with another enzyme replacement therapy indicated for the treatment of Hunter syndrome, such as idursulfase.
- Hemoglobin levels will be obtained before treatment, at 3 months after initiation, and periodically thereafter as clinically indicated.
- Serum creatinine and urinary protein-to-creatinine ratio will be assessed, with monitoring for membranous nephropathy throughout therapy.
Initial approval requirements
- Member weighs at least 5 kg.>= 5 kg
- Documented age-appropriate baseline values have been obtained for one or more applicable clinical or biomarker measures.
  For very young members for whom FVC or 6MWT are not suitable, requests are reviewed case by case.
- Provider attests that the member does not have advanced neurological impairment.
- One of 3 treatment or neurologic-risk pathways
  - Member has tried Elaprase.
  - Clinical documentation demonstrates neurologic manifestations associated with MPS II.

Universal Safety and Concomitant Therapy Criteria

All universal safety and concomitant therapy criteria must be met.

Universal Criteria

Avlayah will not be used in combination with another enzyme replacement therapy indicated for the treatment of Hunter syndrome, such as idursulfase.
Hemoglobin levels will be obtained before treatment, at 3 months after initiation, and periodically thereafter as clinically indicated.before treatment, at 3 months, and periodically thereafter
Serum creatinine and urinary protein-to-creatinine ratio will be assessed, and the member will be monitored for development of membranous nephropathy throughout therapy.

Hunter Syndrome Diagnosis

The member must have a definitive diagnosis of MPS II by one of the following methods.

Definitive MPS II diagnosis

Deficient or absent iduronate 2-sulfatase (I2S) enzyme activity is demonstrated in white cells, fibroblasts, or plasma in the presence of normal activity of at least one other sulfatase.
Pathogenic or likely pathogenic variants in the IDS gene are detected by molecular genetic testing.

Continuation Therapy

Renewal requires continued eligibility, tolerability, and beneficial response to therapy.

Continuation Criteria

The member continues to meet the universal and other relevant indication-specific criteria identified for initial approval.
The member has no unacceptable toxicity from Avlayah.
Examples include severe hypersensitivity or infusion-associated reactions, severe anemia, or membranous nephropathy.
Beneficial response compared with pretreatment age-appropriate baseline
- Members 5 years of age or greater
  - Stabilization or improvement in percent predicted FVC or 6MWT; increased joint range of motion; decreased left ventricular hypertrophy; improved growth; improved quality of life; or reduction in uGAG/uHS levels.one or more applicable measures

Use of Avlayah in combination with another enzyme replacement therapy indicated for Hunter syndrome is excluded. This includes idursulfase.

Members with advanced neurological impairment are excluded from the initial approval pathway. The provider must attest that the member does not have advanced neurological impairment.

Coding and Quantity Requirements

HCPCS CodeHCPCSCovered

J1757	Injection, tividenofusp alfa-eknm, 1 mg; 1 billable unit = 1 mg (Effective 10/01/2026)
J3590	Unclassified biologics (Discontinue use on 10/01/2026)

NDCNDCCovered

84976-0001-xx

Avlayah 150 mg lyophilized powder for injection in a single-dose vial

Covered Diagnosis CodesICD-10Covered

E76.1

Mucopolysaccharidosis, type II

Maximum Billable Units

Maximum billable quantity1,800 billable units

Limit intervalEvery 7 days

Billing basisHCPCS billable units per dose and over time

Minimum Member Weight

Minimum member weightAt least 5 kg

ApplicationRequired for initial approval

Authorization and Documentation Requirements

Prior Authorization

12-Month Prior Authorization Required

Prior authorization is required. Initial authorization is valid for 12 months (365 days) and may be renewed every 12 months (365 days) thereafter.

Initial authorization: 12 months (365 days).
Renewal authorization: every 12 months (365 days).

Documentation Required

Elaprase or Neurologic-Risk Pathway

Initial approval requires one qualifying Elaprase or neurologic-risk pathway.

The member has tried Elaprase; or
Clinical documentation shows neurologic manifestations associated with MPS II; or
The provider attests that the member is presymptomatic and at risk for neurologic manifestations, supported by factors such as a genotype strongly associated with neuronopathic disease, a family history of neuronopathic MPS II sharing the same IDS mutation, or metabolic genetics consultation confirming a high probability of a neuronopathic course.

Clinical Background

Mucopolysaccharidosis type II (MPS II), also known as Hunter syndrome, is a lysosomal storage disorder associated with deficient or absent iduronate 2-sulfatase activity or pathogenic IDS gene variants. Under this policy, Avlayah (tividenofusp alfa-eknm) is an intravenous enzyme replacement therapy for eligible pediatric members with MPS II. Initial coverage requires a definitive diagnosis, weight of at least 5 kg, age-appropriate baseline assessments, an eligible treatment or neurologic-risk pathway, and required safety monitoring.

Definitions

Mucopolysaccharidosis Type II (MPS II; Hunter Syndrome)

DisorderMucopolysaccharidosis type II (MPS II), also called Hunter syndrome, is a lysosomal storage disorder

Enzyme-based diagnosisDeficient or absent iduronate 2-sulfatase (I2S) activity in white cells, fibroblasts, or plasma, with normal activity of at least one other sulfatase

Genetic diagnosisPathogenic or likely pathogenic variants in the IDS gene detected by molecular genetic testing

6MWT

Test nameSix-minute walk test (6MWT)

Initial useAn age-appropriate baseline functional measure for eligible members

Initial Approval

Initial therapy is covered when the definitive diagnosis, universal criteria, and initial approval criteria are all satisfied.

Definitive MPS II diagnosis
- Deficient or absent iduronate 2-sulfatase enzyme activity is demonstrated in white cells, fibroblasts, or plasma in the presence of normal activity of at least one other sulfatase.
- Pathogenic or likely pathogenic variants in the IDS gene are detected by molecular genetic testing.
Universal Criteria
- Avlayah will not be used in combination with another enzyme replacement therapy indicated for Hunter syndrome, such as idursulfase.

Renewal Authorization

Renewal may be authorized when all continuation criteria are met.

The member continues to meet the universal and other indication-specific relevant criteria identified for initial approval.
The member has no unacceptable toxicity from Avlayah.
Examples include severe hypersensitivity reactions, severe infusion-associated reactions, severe anemia, or membranous nephropathy.
Beneficial response compared with pretreatment age-appropriate baseline
- Members 5 years of age or greater
  - Stabilization or improvement in percent predicted FVC or 6MWT; increased joint range of motion; decreased left ventricular hypertrophy; improved growth; improved quality of life; or reduction in uGAG/uHS levels.

Step Therapy

Step	Initial approval pathway	Requirement
1	Elaprase trial	Member has tried Elaprase as one of the alternative pathways for initial approval.

Quantity Limits

DrugAvlayah (tividenofusp alfa-eknm)

Quantity limit1,800 billable units

Limit intervalEvery 7 days

Administration and Site of Care

Note

Administer by intravenous infusion

Administer Avlayah by intravenous infusion. The policy does not specify a required site of care.

Policy Summary

PayerViva Health Plan

PolicyAvlayah (tividenofusp alfa-eknm) Coverage Criteria

Policy CodePolicy IC-0848

Change TypeNo material clinical or coverage changes

Effective Date05/05/2026

Last ReviewSep 1, 2026

Next Review DateN/A

Key ActionObtain prior authorization; initial and renewal authorization are valid for 12 months when applicable criteria are met.

SourceLink

On This Page

Provider attests that the member is presymptomatic and at risk for neurologic manifestations based on genotype, family history, or metabolic genetics consultation.

Examples include large deletions, nonsense variants, severe splice-site variants, or a metabolic genetics consultation confirming a high probability of a neuronopathic course.

A quality-of-life response is a clinically meaningful change in the CHAQ/HAQ/MPS HAQ disability index.

Members less than 5 years of age

Reduction in spleen or liver volume; stabilization or improvement in FVC or 6MWT; or reduction in uGAG/uHS levels.one or more applicable measures
Use age-appropriate measures.

Document the member’s weight with age-appropriate baseline clinical values

Documentation Required

Baseline Clinical Measurements Required

Document age-appropriate baseline measurements before treatment. For very young members for whom FVC or 6-minute walk testing is unsuitable, requests are reviewed case by case.

Members age 5 years or older: obtain one or more of 6-minute walk test (6MWT), percent predicted forced vital capacity (FVC), joint range of motion, left ventricular hypertrophy, growth, quality-of-life measure (CHAQ/HAQ/MPS HAQ), and/or urinary glycosaminoglycan (uGAG)/urinary heparan sulfate (uHS).
Members younger than 5 years: obtain one or more of spleen volume, liver volume, FVC, 6-MWT, and/or uGAG/uHS.
Document baseline weight; the member must weigh at least 5 kg.

Documentation Required

Definitive MPS II Diagnosis

Document a definitive MPS II diagnosis before requesting authorization.

Deficient or absent iduronate 2-sulfatase (I2S) enzyme activity in white cells, fibroblasts, or plasma, with normal activity of at least one other sulfatase; or
Pathogenic or likely pathogenic IDS gene variants identified by molecular genetic testing.

Unsupported block type: documentation

Documentation Required

Safety Monitoring Required

Complete required safety monitoring before and during therapy.

Obtain hemoglobin before initiation, at 3 months after initiation, and periodically thereafter as clinically indicated.
Assess serum creatinine and urinary protein-to-creatinine ratio.
Monitor throughout therapy for development of membranous nephropathy.

Documentation Required

Renewal Response Documentation

Renewal requests must demonstrate continued eligibility, tolerability, and beneficial response compared with pretreatment age-appropriate baseline values.

The member continues to meet applicable universal and indication-specific criteria, including concomitant-therapy requirements and other relevant requirements identified for initial approval.
There is no unacceptable drug toxicity.
Members age 5 years or older: document stabilization or improvement in percent predicted FVC and/or 6-MWT, increased joint range of motion, decreased left ventricular hypertrophy, improved growth, clinically meaningful quality-of-life improvement, and/or reduced uGAG/uHS.
Members younger than 5 years: document reductions in spleen and/or liver volume, stabilization or improvement in FVC and/or 6-MWT, and/or reduced uGAG/uHS.

Denial Risk

Failure to Meet Initial Criteria

Requests may be denied when initial approval criteria are not fully met or required documentation is missing.

Member weighs less than 5 kg.
Definitive MPS II diagnosis is not established by qualifying I2S enzyme-activity or IDS genetic findings.
Required age-appropriate baseline clinical measurements are absent or insufficient, without an applicable case-by-case exception for unsuitable FVC or 6-MWT testing in a very young member.
Provider attestation that advanced neurological impairment is absent is missing or not supported.
No qualifying Elaprase trial, neurologic manifestations, or documented presymptomatic neurologic-risk pathway is provided.
Other initial universal or indication-specific requirements are not met.

Denial Risk

Prohibited Combination or Inadequate Monitoring

Treatment is restricted or may be denied when prohibited combination therapy is used or required safety monitoring is not completed.

Do not use Avlayah in combination with other enzyme replacement therapies indicated for Hunter syndrome, including idursulfase.
Required hemoglobin monitoring is missing or incomplete.
Serum creatinine, urinary protein-to-creatinine ratio, or monitoring for membranous nephropathy is missing or incomplete.

Denial Risk

Toxicity or Inadequate Response

Renewal may be denied for unacceptable toxicity or failure to demonstrate stabilization or improvement relative to pretreatment age-appropriate baseline values.

Unacceptable toxicity includes severe hypersensitivity reactions such as anaphylaxis; severe infusion-associated reactions; severe anemia; membranous nephropathy; or other clinically significant toxicity.
Renewal may be denied when the member fails to stabilize or improve in the applicable functional, respiratory, joint, cardiac, growth, quality-of-life, organ-volume, or uGAG/uHS measures.
Renewal may also be denied when continued eligibility or applicable universal and indication-specific criteria are no longer met.

A response measure; stabilization or improvement may support continued authorization

Membranous Nephropathy

ConditionMembranous nephropathy is a kidney disorder monitored during Avlayah therapy

Renal monitoringAssess serum creatinine and urinary protein-to-creatinine ratio throughout therapy

Renewal safety considerationMembranous nephropathy is an example of unacceptable toxicity that may affect renewal

Hemoglobin levels will be obtained before treatment, at 3 months after initiation, and periodically thereafter as clinically indicated.

Serum creatinine and urinary protein-to-creatinine ratio will be assessed, with monitoring for membranous nephropathy throughout therapy.

Initial Approval Criteria

Member weighs at least 5 kg.>= 5 kg
Documented baseline age-appropriate values have been obtained for one or more applicable clinical or biomarker measures.
For very young members for whom FVC or 6MWT are not suitable, requests are reviewed case by case.
Provider attests that the member does not have advanced neurological impairment.
One of 3 treatment or neurologic-risk pathways
- Member has tried Elaprase.
- Clinical documentation demonstrates neurologic manifestations associated with MPS II.
- Provider attests that the member is presymptomatic and at risk for neurologic manifestations based on genotype, family history, or metabolic genetics consultation.

one or more applicable measures

Quality-of-life response means a clinically meaningful change in the CHAQ/HAQ/MPS HAQ disability index.

Members less than 5 years of age

Reduction in spleen or liver volume; stabilization or improvement in FVC or 6MWT; or reduction in uGAG/uHS levels.one or more applicable measures
Use age-appropriate measures.