Recommendations

Document Overview

Design and created by Guideline Central in participation with the Cystic Fibrosis Foundation.

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

Cystic Fibrosis Foundation

Publication Date: Nov 18, 2017

Page Last Updated: May 5, 2026

Cystic Fibrosis Foundation Grading System-2

Grade Definitions on Strength of Recommendation and Guide to Interpretation
Strength	Wording in the guideline	For the patient	For the clinician
S = Strong	"for" or “against”	Most individuals in this situation would want the recommended course of action, and only a small proportion would not. Formal decision aids are not likely to be needed to help individuals make decisions consistent with their values and preferences.	Most individuals should receive the recommended course of action. Adherence to this recommendation according to the guideline could be used as a quality criterion or performance indicator.
C = Conditional	"for" or “against”	The majority of individuals in this situation would want the recommended course, but many would not.	Recognize that different choices will be appropriate for individual patients and that clinicians must help each patient arrive at a management decision consistent with his or her values and preferences. Decision aids may be useful in helping individuals make decisions consistent with their values and preferences.

Grade Definitions of Quality and Certainty of the Evidence
Quality Grade	Definition
High	Very confident that the true effect lies close to that of the estimate of the effect.
Moderate	Moderately confident in the effect estimate. The true effect is likely to be close to the estimate of effect, but there is a possibility that it is substantially different.
Low	Confidence in the estimate is limited. The true effect may be substantially different from the estimate of effect.
Very low	Very little confidence in the effect estimate. The true effect is likely to be substantially different from the estimate of effect.

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

Full Text Guideline: www.atsjournals.org/doi/full/10.1513/AnnalsATS.201707-539OT

Document Objectives: Develop evidence-based guidelines for CFTR modulator therapy in patients with CF

Diseases/Conditions (MeSH)

D003550 - Cystic Fibrosis

Intended Users: Nurse, Nurse Practitioner, Physician, Physician Assistant, Respiratory Therapist