CurrentCignaPolicy IP0431

Kalydeco

Defines Cigna's medical necessity and prior authorization requirements for Kalydeco (ivacaftor) for treatment of cystic fibrosis and specifies eligible CFTR mutations, age limits, documentation, and preferred product rules affecting benefit coverage.

Policy Summary

PayerCigna

PolicyKalydeco

Policy CodePolicy IP0431

Change TypeCriteria revision with multiple additions

Effective DateApr 1, 2025

Next Review DateN/A

Key ActionPrior authorization is required and prescribers must provide genotyping evidence showing at least one pathogenic or likely pathogenic CFTR mutation.

SourceLink

POLICY UPDATE CHANGES

The patient mutation criterion wording was updated to require documentation that the patient has at least one CFTR mutation considered pathogenic or likely pathogenic.

A requirement was added that the patient meet one of: positive newborn screen, family history of CF, or clinical presentation consistent with CF.

A requirement was added that the patient have evidence of abnormal CFTR function by elevated sweat chloride, two CF-causing CFTR mutations, or abnormal nasal potential difference.

Infertility was added to conditions not recommended for approval.

Preferred product criteria were revised to require patients ≥ 2 years to try Trikafta first (or have a non-covered mutation) or already be on Kalydeco.

Removed prior broader documented diagnosis language as a standalone requirement.

≥1 mominimum approved age

1 yr

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Coverage Criteria

Initial Therapy (FDA-approved indication)

Covered when ALL of the following are met (A through F):

Initial Therapy (FDA-approved indication): A) Patient is ≥ 1 month of age;

Initial Therapy (FDA-approved indication): B) Documentation is provided that the patient has at least ONE CFTR mutation considered pathogenic or likely pathogenic from the enumerated list.

C) Clinical/newborn/family history requirement: Patient meets at least ONE of the following: positive cystic fibrosis newborn screening test; OR family history of cystic fibrosis; OR clinical presentation consistent with signs and symptoms of cystic fibrosis (examples include but are not limited to meconium ileus, sinopulmonary symptoms such as persistent cough or wheezing, pulmonary function tests consistent with obstructive airway disease, excess sputum production, bronchiectasis, sinusitis, failure to thrive, pancreatic insufficiency).

D) Evidence of abnormal CFTR function: Patient has evidence of abnormal cystic fibrosis transmembrane conductance regulator (CFTR) function demonstrated by at least ONE of the following: elevated sweat chloride test; OR two cystic fibrosis–causing CFTR mutations; OR abnormal nasal potential difference.

E) Prescriber specialization: Medication is prescribed by or in consultation with a pulmonologist or a physician who specializes in the treatment of cystic fibrosis.

F) Preferred product pathway: Preferred product criteria is met for the products listed in the policy's preferred product table (see policy for details).

Approval duration: 1 year.

Kalydeco (ivacaftor) must not be used in combination with other CFTR modulators that contain ivacaftor (for example, Orkambi, Symdeko, Trikafta, or other listed ivacaftor‑containing products). These agents contain the same active moiety and are not indicated in combination with Kalydeco. When coverage is available and medically necessary, dosing, frequency, duration, and site of care should be reasonable and supported by evidence; receipt of sample product does not satisfy coverage criteria.

Use of Kalydeco for the indication of infertility is explicitly listed as a condition not recommended for approval and therefore is not covered under this policy.

Treatment of patients who are homozygous for the F508del CFTR mutation is considered not medically necessary because clinical trials demonstrated no significant efficacy in this population.

An FDA‑cleared cystic fibrosis mutation test is required before using Kalydeco in patients with an unknown CFTR genotype. The policy specifies that an FDA‑cleared CF mutation test should be used prior to use of Kalydeco, and use without prior testing in patients with unknown mutation status is not covered.

Initial Therapy

Initial therapy

Initial therapy: See 'Initial Therapy (FDA-approved indication)' criteria in Coverage module: patient age ≥ 1 month; documented pathogenic or likely pathogenic CFTR mutation from the enumerated list; positive newborn screen, family history, or clinical presentation consistent with CF; evidence of abnormal CFTR function (elevated sweat chloride, two CF-causing mutations, or abnormal nasal potential difference); prescribed by or in consultation with a CF specialist; and preferred product rules where applicable.

Authorization: 1 year.

Continuation and Renewal Criteria

Continuation therapy

Continuation/renewal

Continuation: Approvals are provided for 1 year; continued coverage requires ongoing documentation as indicated and continued prescribing by or in consultation with a pulmonologist or physician who specializes in cystic fibrosis.

Preferred Product / Step Preference

Step	Required condition / action	Coverage status
1	Patient is ≥ 2 years of age AND the patient meets ONE of the following: a) Patient has tried Trikafta (elexacaftor/tezacaftor/ivacaftor) and, according to the prescriber, has experienced inadequate efficacy OR a significant intolerance (tablets or oral granules) [may require prior authorization]; OR b) Patient has at least one CFTR mutation considered pathogenic or likely pathogenic that is not covered by Trikafta (tablets or oral granules) [may require prior authorization]	Covered
2	Patient is < 2 years of age	Covered
3	Patient has already been started on therapy with Kalydeco (ivacaftor)	Covered

Provider Actions and Requirements

Prior Authorization

Prior Authorization Required

Prior authorization is required for prescription benefit coverage of Kalydeco (ivacaftor). All approvals are provided for the duration noted below (standard: 1 year). Kalydeco must be prescribed by or in consultation with a physician who specializes in cystic fibrosis due to required evaluation and monitoring.

Approval duration: 1 year
Prescriber requirement: specialist in condition treated (cystic fibrosis)

Step Therapy

Preferred Product / Step Preference

Trikafta (elexacaftor/tezacaftor/ivacaftor — tablets or oral granules) is the preferred product. For Employer Plans on the Total Savings Drug List, one of the following is required for Kalydeco coverage: patient is ≥ 2 years of age AND (a) prescriber documents inadequate efficacy or significant intolerance to Trikafta (may require prior authorization), OR (b) patient has at least one CFTR pathogenic/likely pathogenic mutation not covered by Trikafta (may require prior authorization). Also acceptable: patient < 2 years of age, or patient already started on Kalydeco therapy.

Coding Guidance

Coding guidance (general)mixed

No codes listed

Definitions

Evidence of abnormal CFTR function

Elevated sweat chlorideSweat chloride ≥ 60 mmol/L establishes CF; values 30–<60 mmol/L prompt CFTR genetic analysis; <30 mmol/L makes CF unlikely but may be considered with confirmatory testing.

Two CF-causing CFTR mutationsIdentification of two CF-causing CFTR mutations on genetic analysis establishes CF; full gene sequencing recommended when only one or undefined variant is found.

Abnormal nasal potential difference (NPD)Abnormal NPD (a CFTR physiologic test) indicating CFTR dysfunction supports diagnosis when genetic or sweat testing is inconclusive; NPD or intestinal current measurement (ICM) may be used.

Background

Kalydeco (ivacaftor) is a CFTR potentiator indicated for the treatment of cystic fibrosis in patients aged ≥ 1 month who have at least one CFTR mutation responsive to ivacaftor. The drug improves CFTR channel function for responsive mutations but has not shown efficacy for certain genotypes (for example, patients homozygous for F508del).

Site of Care

Note

Site-of-care and regimen must be reasonable and evidence-supported

Dose, frequency, duration of therapy, and site of care (including home setting) should be reasonable, clinically appropriate, and supported by evidence-based literature and adjusted based on severity, alternative treatments, and prior response.

No specific site-of-care restrictions are stated; selection should be evidence-based and clinically appropriate.
Receipt of sample product does not satisfy criteria requirements for coverage.