CurrentCignaPolicy N/A

Orkambi (lumacaftor/ivacaftor) prior authorization form coverage criteria

This document is a Cigna prior authorization request form for Orkambi (lumacaftor/ivacaftor) used to treat cystic fibrosis and related CFTR disorders; it governs documentation and submission requirements for providers requesting coverage. It affects prescribers, particularly pulmonologists and those managing cystic fibrosis patients, and Cigna pharmacy review staff.

Policy Summary

PayerCigna

PolicyOrkambi (lumacaftor/ivacaftor) prior authorization form coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionComplete the Orkambi-specific prior authorization form and attach required supportive documentation including genetic testing, chart notes, and dosing rationale.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

5 business daysstandard response time

855-840-1678fax to submit

800-882-4462urgent phone

Coverage Criteria and Requirements

Form completion and supporting clinical documentation

Coverage considered when ALL of the following are documented on the form and supporting attachments:

Prescriber and patient info: Completed patient and prescriber identification fields including Cigna ID and prescriber DEA/NPI/TIN

Form requires all asterisked items to be completed.

Diagnosis indicated as cystic fibrosis, CFTR-related disorder, or CRMS/CFSPID with appropriate ICD-10 code

Diagnosis must be selected on the form.

Genetic testing: Evidence of CFTR mutation testing (for example, documentation of F508del homozygosity when applicable)

Lab report or report from the Cystic Fibrosis Foundation Patient Registry or Mutation Analysis Program required.

Policy Summary

PayerCigna

PolicyOrkambi (lumacaftor/ivacaftor) prior authorization form coverage criteria

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionComplete the Orkambi-specific prior authorization form and attach required supportive documentation including genetic testing, chart notes, and dosing rationale.

SourceLink

On This Page

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The prior authorization form asks prescribers to indicate whether Orkambi will be used in combination with other CFTR modulators (specifically Kalydeco/ivacaftor, Symdeko/tezacaftor-ivacaftor, or Trikafta/elexacaftor-tezacaftor-ivacaftor). The form does not provide separate coverage pathways for combined therapy; instead, combination use must be declared on the form because it may affect the review and coverage decision.

When combination therapy is intended, include the names, doses, and rationale for concurrent CFTR modulators on the submission and attach supporting clinical documentation. Failure to declare combination use on the form may lead to delay while reviewers obtain clarification or may affect approval determinations.

The form requires supportive documentation for this drug and states that genetic testing, chart notes, lab/test results, and other supporting documents must be attached with the request. Requests submitted without the required genetic testing (for example, documentation of F508del status when applicable) or other supporting clinical documentation may not be processed or may be denied.

The form also queries prescriber expertise: it asks whether the prescriber is a pulmonologist or is prescribing in consultation with a pulmonologist. If the prescriber specialty or a documented pulmonology consultation is not provided, the submission may be delayed or risk denial. Ensure the prescriber specialty and consultation details are completed on the form and relevant notes are attached.

Medication and Diagnosis Coding

Requested medication formulationsmixed

Orkambi 100mg-125mg tablets	Formulation option listed on form
Orkambi 200mg-125mg tablets	Formulation option listed on form
Orkambi 100mg-125mg granule packets	Formulation option listed on form
Orkambi 150mg-188mg granule packets	Formulation option listed on form

Diagnosis codingICD-10

ICD-10

Diagnosis code(s) to be provided on the form (e.g., cystic fibrosis or CFTR-related disorder)

inv-06: Maximum typical daily tablets/packets

Maximum typical daily tablets≤ 4 tablets per day

Maximum typical daily granule packets≤ 2 granule packets per day

Requirement if exceeding limitsIf more than 4 tablets/day or more than 2 granule packets/day, provide clinical support for requesting this dosing (e.g., past medications tried, pertinent patient history)

Prior Authorization Submission & Provider Responsibilities

Prior Authorization

Prior Authorization Required

Prior authorization requires completion of the Orkambi-specific prior authorization form. The form must indicate diagnosis, requested medication formulation and dose, directions, quantity, duration of therapy, and whether the medication is intended for chronic/long-term use.

Medication, strength, dosing schedule, directions, quantity and duration must be specified
If dosing >4 tablets/day or >2 granule packets/day provide clinical rationale

Documentation Required

Required Supportive Documentation

Supportive documentation must be attached with the request. Examples include genetic testing (lab report or Cystic Fibrosis Foundation registry/mutation analysis report showing F508del status when applicable), pertinent chart notes, lab/test results, pulmonary function data, and other documentation of clinical status or decline.

Attach genetic testing or registry report when F508del mutation is relevant
Include chart notes, FEV1 results, BMI, pulmonary exacerbation history, CFQ‑R respiratory scores, and other pertinent labs/tests

Denial Risk

Missing Documentation May Result in Denial or Delay

Requests that are missing required supportive documentation (for example: genetic testing, chart notes, lab/test results, documented baseline or response data) may be delayed, not processed, or denied. Ensure all supporting attachments are included at time of submission.

Incomplete requests risk denial or processing delays
Attach all supporting files before submission to avoid repeat requests for information

Prior Authorization

Combination Therapy Declaration

The form asks whether Orkambi will be used in combination with other CFTR modulators (ivacaftor/Kalydeco, tezacaftor/ivacaftor/Symdeko, or elexacaftor/tezacaftor/ivacaftor/Trikafta). Indicate Yes or No. Combination use may affect coverage decisions.

Declare if therapy will be combined with Kalydeco, Symdeko, or Trikafta

Documentation Required

Prescriber Specialty / Consultation Required

The form requires documentation of the prescriber’s specialty. If the prescriber is not a pulmonologist or cystic fibrosis specialist, the form must indicate whether the therapy is prescribed in consultation with such a specialist. Lack of documentation of appropriate specialty or consultation may result in additional review or denial.

Confirm prescriber is a pulmonologist or CF specialist, or document consultation with one
If specialty/consultation not documented, expect possible denial or further review

Background

Orkambi (lumacaftor/ivacaftor) is a cystic fibrosis transmembrane conductance regulator (CFTR) modulator indicated for patients with CFTR mutations such as F508del. Clinical benefit is typically documented by measures such as stabilized or improved FEV1, reduced pulmonary exacerbations, improved BMI, or improvement on the Cystic Fibrosis Questionnaire-Revised respiratory domain.

For prior authorization purposes the form requests mutation status (including whether the patient has two copies of the F508del mutation when applicable), clinical response information for continuation requests, and other supportive documentation to substantiate medically necessary use.

Key Definitions and Dosing Limits

inv-13: Documented F508del homozygosity

DefinitionDocumented F508del homozygosity = two copies of the F508del mutation as documented by either the lab report or a report from the Cystic Fibrosis Foundation Patient Registry or Mutation Analysis Program

Acceptable documentation typesLab report

Alternate acceptable documentationReport from the Cystic Fibrosis Foundation Patient Registry or Mutation Analysis Program

inv-14: Exceeding typical dosing limits

Exceeding typical dosing definitionMore than 4 tablets per day or more than 2 granule packets per day

Documentation requiredProvide clinical support for requesting this dosing, such as past medications tried and pertinent patient history

Where to include rationaleAttach dosing rationale and supportive documentation with the prior authorization request form