Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients With Cystic Fibrosis

Publication Date: November 18, 2017
Last Updated: March 14, 2022

Recommendations

Ivacaftor for gating mutations other than G551D or R117H

The committee recommends IVA for individuals aged 2–5 years with a diagnosis of CF and gating mutations other than G551D or R117H. For individuals under 2 years of age, the committee makes no recommendation. (, )
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The committee suggests IVA for individuals aged 6–11 years with a diagnosis of CF with PPFEV1 less than 40% and a gating mutation other than G551D or R117H. (C, VL)
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The committee suggests IVA treatment for individuals aged 6–11 years with a diagnosis of CF with PPFEV1 40%–90% and a gating mutation other than G551D or R117H. (C, L)
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The committee suggests IVA be used for individuals aged 6–11 years with a diagnosis of CF with PPFEV1 greater than 90% and a gating mutation other than G551D or R117H. (C, L)
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The committee suggests IVA for individuals aged 12–17 years with a diagnosis of CF with PPFEV1 40%–90% and a gating mutation other than G551D or R117H. (C, L)
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The committee suggests IVA for individuals aged 12–17 years with a diagnosis of CF with PPFEV1 greater than 90% and a gating mutation other than G551D or R117H. (C, M)
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The committee suggests IVA for individuals aged 18 years or older with a diagnosis of CF with PPFEV1 less than 40% and a gating mutation other than G551D or R117H. (C, M)
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The committee suggests IVA for individuals with a diagnosis of CF aged 18 years or older with PPFEV1 40%–90% and a gating mutation G551D or R117H. (C, L)
322286
The committee suggests IVA for individuals aged 18 years or older with a diagnosis of CF with PPFEV1 greater than 90% and a gating mutation G551D or R117H. (C, M)
322286
The committee suggests IVA for individuals aged 12–17 years with a diagnosis of CF with PPFEV1 less than 40% and a gating mutation other than G551D or R117H. (C, M)
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Overview

Title

Use of Cystic Fibrosis Transmembrane Conductance Regulator Modulator Therapy in Patients With Cystic Fibrosis

Authoring Organization

Cystic Fibrosis Foundation