CurrentCommunity-CarePolicy N/A

Adenosine deaminase severe combined immune deficiency (ADA-SCID) medication therapy coverage criteria

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Defines medical benefit coverage criteria, authorization periods, quantity limits, and required documentation for initial and continued therapy for ADA-SCID; applies to providers and members under Community-Care policies.

Policy Summary

PayerCommunity-Care

PolicyADA-SCID medication therapy coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Last ReviewSep 1, 2022

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation confirming ADA deficiency and specialist involvement.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsinitial authorization period

1 yearcontinuation authorization period

Nonequantity limit

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CurrentCommunity-CarePolicy N/A

Adenosine deaminase severe combined immune deficiency (ADA-SCID) medication therapy coverage criteria

Customize your policy alerts

Sign up for all Community-Care policy alerts

Know when Community-Care releases new policies or updates existing guidance.

Monitor payer policy activity

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Policy Summary

PayerCommunity-Care

PolicyADA-SCID medication therapy coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Last ReviewSep 1, 2022

Next Review DateN/A

Key ActionObtain prior authorization and submit documentation confirming ADA deficiency and specialist involvement.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

6 monthsinitial authorization period

1 yearcontinuation authorization period

Nonequantity limit

Coverage Criteria for ADA‑SCID Therapy

Continuation Therapy

Continuation approval is covered when ALL of the following are met:

Continuation Approval - ADA-SCID: 1) Ongoing diagnosis of adenosine deaminase severe combined immune deficiency (ADA-SCID); 2) Member is being monitored, has experienced disease stabilization or improvement, and is appropriate to continue therapy.

Continuation approval allowed for one year.

The policy does not list any explicit exclusions for adenosine deaminase severe combined immunodeficiency (ADA‑SCID) medication therapy. Coverage is governed by the stated initial and continuation criteria and authorization periods rather than by named exclusions.

No conditions or scenarios are specified in this document as not medically necessary. Medical necessity determinations for ADA‑SCID therapy should follow the policy's coverage criteria (diagnostic confirmation, specialist involvement, and documented SCID) and required documentation.

Applicable Coding

Applicable HCPCS CodesHCPCS

J3590

Unclassified biologics

Provider Actions, Authorization, and Documentation

Prior Authorization

Prior authorization required; approval durations

Prior authorization is required for both initial and continuation coverage. If approved, initial coverage is allowed for six months and continuation coverage is allowed for one year.

Note

Prescriber specialty and ongoing monitoring requirements

The prescription must be by, or in consultation with, an immunologist or a provider who specializes in ADA‑SCID. Ensure ongoing monitoring is documented for continuation requests showing disease stabilization or improvement.

Prescriber is an immunologist or ADA‑SCID specialist, or consultation with one is documented.
For continuation, document monitoring and evidence of stabilization or improvement.

Key Definitions

ADA deficiency confirmation

Absent or very low ADA activity in RBCsAbsent or very low (< 1% of normal) adenosine deaminase (ADA) activity in red blood cells

Elevated dATP in erythrocytesIncreased levels of deoxyadenosine triphosphate (dATP) in erythrocyte lysates compared to laboratory standards

Decreased ATP in RBCsSignificantly decreased concentration of adenosine triphosphate (ATP) in red blood cells

Low SAH hydrolase in RBCsAbsent or very low (< 5% of normal) levels of S-adenosylhomocysteine (SAH) hydrolase in red blood cells

Genetic confirmationPresence of biallelic pathogenic mutations in the ADA gene

Background

Adenosine deaminase severe combined immunodeficiency (ADA‑SCID) is a form of severe combined immunodeficiency caused by deficiency of the enzyme adenosine deaminase, resulting in accumulation of toxic metabolites and profound lymphocyte dysfunction. Diagnosis is confirmed by biochemical markers or genetic testing, including absent or very low ADA activity in red blood cells (< 1% of normal), elevated dATP in erythrocyte lysates, significantly decreased ATP in red blood cells, absent or very low (< 5% of normal) SAH hydrolase in red blood cells, elevated 2'-deoxyadenosine in plasma/urine/dried blood spots, or the presence of biallelic pathogenic ADA gene mutations. Treatment decisions should involve a specialist and include ongoing monitoring to document disease stabilization or improvement when continuing therapy.

Documentation Required

Required diagnostic and monitoring documentation

Include diagnostic confirmation of ADA deficiency using one of the specified laboratory or genetic findings and documentation that the prescriber is an immunologist or consulted with one.

One of the following must be documented: absent or very low (<1% of normal) ADA activity in RBCs; increased dATP in erythrocyte lysates; significantly decreased ATP in RBCs; absent or very low (<5% of normal) SAH hydrolase in RBCs; elevated 2'-deoxyadenosine in plasma/urine/dried blood spots; or biallelic pathogenic ADA gene mutations.
For continuation requests, include monitoring notes demonstrating stabilization or improvement.