Volume 9, Issue 4

In the Clinic: Triple Combination CFTR Modulation

In this issue:

Elexacaftor/tezacaftor/ivacaftor — the newly approved triple combination CFTR modulator therapy.  What are the benefits?  For which patients?  What are the potential adverse effects?  Who’s most likely to receive them?  

In this issue, Dr. Scott Sagel from the Breathing Institute at the Children’s Hospital Colorado, part of the University of Colorado’s Anschutz Medical Campus, takes us to the clinic to discuss some answers. 

Learning objectives:

  • Describe the effects of triple combination CFTR modulator therapy for people with at least one copy of the F508del CFTR mutation. 
  • Explain how triple combination CFTR modulator therapy might impact the comorbidities and complications associated with cystic fibrosis.

Author:

Scott Sagel, MD, PhD
Scott Sagel, MD, PhD

Professor of Pediatrics
Asher-Accurso Chair of Cystic Fibrosis
Director, Pediatric Cystic Fibrosis Center 
Breathing Institute, Children’s Hospital Colorado
University of Colorado Anschutz Medical Campus

Program Directors:

Noah Lechtzin, MD, MHA

Director, Adult Cystic Fibrosis Program
Associate Professor of Medicine
Johns Hopkins University School of Medicine
Baltimore, MD

Peter J. Mogayzel, Jr., MD, PhD

Professor of Pediatrics
Director, Cystic Fibrosis Center
Johns Hopkins University School of Medicine
Baltimore, MD

Donna Peeler, RN

Clinical Nurse 
Pediatric Clinic Coordinator 
Johns Hopkins Cystic Fibrosis Center 
Baltimore, MD

Length of activity:

0.5 hour Physicians
0.5 contact hour Nurses

Launch date: June 2, 2020
Expiration date: June 1, 2020