CurrentCignaPolicy N/A

Cystic Fibrosis Transmembrane Conductance Regulator - Symdeko Prior Authorization Policy

Prior authorization policy for Symdeko (tezacaftor/ivacaftor and ivacaftor) covering FDA-approved indication for cystic fibrosis in patients ≥6 years with specified CFTR variants or two copies of F508del, and related documentation and prescribing requirements for Cigna-administered benefit plans.

Policy Summary

PayerCigna

PolicyCystic Fibrosis Transmembrane Conductance Regulator - Symdeko Prior Authorization Policy

Policy CodePolicy N/A

Change TypeAnnual revision (no criteria changes)

Effective Date

Next Review Date

Key ActionPrior authorization is recommended for Symdeko; approvals provided for 1 year and require prescription by or consultation with a pulmonologist or CF specialist.

SourceLink

POLICY UPDATE CHANGES

Annual Revision, Summary of Changes = No criteria changes.

Previous selected revisions replaced term 'mutation' with 'variant' across criteria.

Previously required listed mutations modified to require pathogenic or likely pathogenic classification.

1Approval duration (years)

≥6Minimum age

manyListed CFTR responsive variants

Coverage Summary

Prior authorization policy for Symdeko (tezacaftor/ivacaftor and ivacaftor) covering the FDA‑approved indication for cystic fibrosis in patients ≥ 6 years who are homozygous for F508del or have at least one CFTR variant responsive to tezacaftor/ivacaftor, with documentation and specialist prescribing requirements for Cigna‑administered benefit plans. Coverage stance: covered_with_criteria. Approvals are provided for 1 year when all policy criteria are met.

Prior Authorization & Documentation Requirements

Prior Authorization

Prior authorization required

Prior authorization is recommended for Symdeko; approvals provided for 1 year and require prescription by or consultation with a pulmonologist or CF specialist.

Documentation Required

Genotype documentation

Document CFTR genotype showing either two copies of F508del or at least one listed pathogenic/likely pathogenic variant; if genotype unknown, perform FDA-cleared CF mutation test with bidirectional sequencing when recommended.

If genotype unknown, use an FDA-cleared CF mutation test and verify with bidirectional sequencing when recommended by the test instructions.

Documentation Required

Evidence of CF diagnosis/function

Provide evidence of abnormal CFTR function via elevated sweat chloride, two CF-causing CFTR variants, or abnormal nasal potential difference, and documentation of positive newborn screen, family history, or clinical presentation consistent with CF.

Elevated sweat chloride test (≥ 60 mmol/L diagnostic; <30 mmol/L makes CF unlikely; 30–59 mmol/L requires further testing)
Two cystic fibrosis-causing CFTR variants
Abnormal nasal potential difference
Positive newborn screening test
Family history of cystic fibrosis
Clinical presentation compatible with CF (examples: meconium ileus, persistent cough, wheezing, bronchiectasis, sinusitis, failure to thrive, pancreatic insufficiency)

Denial Risk

Denial risk: unknown genotype or combination therapy

Claims will be considered not medically necessary (denied) if patient genotype is unknown without appropriate testing or if Symdeko is used in combination with other CFTR modulators.

Clinical Evidence & References

Evidence includes: Symdeko® tablets prescribing information (Vertex; September 2025) and the European Cystic Fibrosis Society Standards for CFTR variant‑specific therapy (2023/2024). The variant inclusion is driven by the policy’s responsiveness definition: a responsive variant is one for which tezacaftor/ivacaftor increases chloride transport to ≥10% of untreated normal over baseline or demonstrates a clinical FEV1 response (as enumerated in Table 1).

Background

Symdeko (tezacaftor/ivacaftor and ivacaftor) is a CFTR modulator indicated for treatment of cystic fibrosis in patients ≥ 6 years who are homozygous for the F508del variant or who have at least one CFTR variant responsive to tezacaftor/ivacaftor by clinical and/or in vitro evidence. Genotype confirmation is required (use an FDA‑cleared CF mutation test with bidirectional sequencing verification when recommended), and evidence of CFTR dysfunction (e.g., elevated sweat chloride, two CF‑causing CFTR variants, or abnormal nasal potential difference) is required for approval.

Quick thresholds & policy stats

Minimum age≥ 6 years

Approval duration1 year

Listed CFTR responsive variantsExtensive enumerated list (Table 1) — many specific pathogenic/likely pathogenic variants or two copies of F508del

Revision History

2026-02-11Annual RevisionLatest

Annual Revision, Summary of Changes = No criteria changes.

2025-02-05Selected Revision

Replaced term "mutation" with "variant" across criteria.

2024-04-10Selected Revision