Volume 10, Issue 5

CFTR Triple Therapy (ETI)

In this issue:

ETI — elexacaftor/tezacaftor/ivacaftor — is next-generation triple-combination CFTR modulator therapy. Its recent pivotal trials have shown both efficacy and safety. But:

  • Which patients with what CFTR mutations were in those trials?
  • Which other patients with which additional mutations might benefit from triple therapy?
  • Can ETI be used patients who become pregnant or expect to breastfeed?
  • What does the evidence say?

These are some of the key questions guest author Dr. Edward McKone from St. Vincent’s University Hospital and University College Dublin School of Medicine addresses in this issue of eCysticFibrosis Review

Learning objectives:

  • Describe the new evidence on the indications for triple therapy with elexacaftor/tezacaftor/ivacaftor (ETI) in patients with F508del and a second gating/residual function mutation. 
  • Discuss advice for patients who are on triple therapy with elexacaftor/tezacaftor/ivacaftor (ETI) and become pregnant or who plan to breastfeed.  

Author:

Edward McKone, MD
Edward McKone, MD

Clinical Professor
St. Vincent’s University Hospital and University College Dublin School of Medicine
National Referral Center for Adult Cystic Fibrosis
St. Vincent’s University Hospital and University College Dublin School of Medicine
Dublin, Ireland

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:

1.0 hour Physicians
1.0 contact hour Nurses

Launch date: August 23, 2022
Expiration date: August 22, 2024