CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Kalydeco (ivacaftor) — Coverage Criteria for Cystic Fibrosis

Coverage policy for Kalydeco (ivacaftor) for treatment of cystic fibrosis in members of Neighborhood Health Plan of Rhode Island, specifying indications, required documentation, prescriber specialty, approval duration, and continuation criteria.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyKalydeco (ivacaftor) — Coverage Criteria for Cystic Fibrosis

Policy CodePolicy N/A

Change TypeNo material changes

Effective DateN/A

Next Review DateN/A

Key ActionSubmit genetic testing report confirming the presence of the appropriate CFTR gene mutation for prior authorization.

SourceLink

POLICY UPDATE CHANGES

No material clinical or coverage changes in this revision.

1 monthminimum patient age for coverage

12 monthsapproval duration

multipleresponsive CFTR mutations

Coverage Criteria for Kalydeco (ivacaftor)

COVERAGE CRITERIA

Authorization of 12 months may be granted for treatment of cystic fibrosis when ALL of the following are met:

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Initial coverage criteria

Indication: Patient has a diagnosis of cystic fibrosis and the request is for an FDA-approved or compendial use of ivacaftor.
Genotype requirement: Genetic testing was conducted and confirms the presence of one of the CFTR mutations listed in the policy that are responsive to ivacaftor potentiation. If genotype is unknown, perform an FDA-cleared CF mutation test followed by verification with bi-directional sequencing when recommended by the test instructions for use.
If genotype unknown, perform an FDA-cleared CF mutation test followed by bi-directional sequencing as recommended.
Age requirement: Member is at least 1 month of age.>= 1 month
Monotherapy constraint: Ivacaftor (Kalydeco) will not be used in combination with other medications containing ivacaftor.

Provider Requirements and Documentation

Prior Authorization

Prior Authorization Required

Prior authorization requires genetic testing confirming a covered CFTR mutation and meeting the listed approval criteria.

For initial requests, submit genetic testing report confirming the presence of the appropriate CFTR gene mutation.

Documentation Required

Prescriber Specialty Requirement

This medication must be prescribed by or in consultation with a pulmonologist.

Denial Risk

Required Documentation and Denial Risk

For initial prior authorization requests, include the genetic testing report that documents the CFTR gene mutation(s). Requests lacking the required genetic testing documentation may be denied.

Initial requests: genetic testing report confirming the presence of the appropriate CFTR gene mutation.
Include clinical notes or consultation documentation if prescribed in consultation with a pulmonologist.

Clinical Background

Ivacaftor (Kalydeco) is a CFTR potentiator indicated for the treatment of cystic fibrosis in patients age 1 month and older who have at least one CFTR gene mutation that is responsive to ivacaftor potentiation based on clinical and/or in vitro assay data. If a patient’s genotype is unknown, an FDA-cleared CF mutation test should be used to detect CFTR mutations with follow-up bi-directional sequencing when recommended by the test instructions.

Definitions and Tests

FDA-cleared CF mutation test — definition and sequencing guidance

DefinitionFDA-cleared CF mutation test: a test cleared by the U.S. Food and Drug Administration to detect the presence of CFTR gene mutations.

Follow-up sequencingIf the patient’s genotype is unknown, the FDA-cleared CF mutation test should be followed by verification with bi-directional sequencing when recommended by the mutation test instructions for use.

PurposeTo identify CFTR mutations that are responsive to ivacaftor potentiation and support initiation of Kalydeco therapy.

Age contextTesting applies to patients age 1 month and older being evaluated for ivacaftor treatment, consistent with the FDA-approved indication for Kalydeco.

Role in authorizationAn FDA-cleared CF mutation test (with sequencing if recommended) is required to confirm a responsive mutation as part of prior authorization for ivacaftor therapy.