Volume 8, Issue 8

CFTR Dysfunction & Nutritional Deficiencies

Editor's note:

If you’re interested in other valuable CF medical education, our CF Virtual Roundtable webcast is now available on-demand! Start the activity here: virtual.dkbmed.com 

In this program, Peter Mogayzel, Jr., MD, PhD leads a case-based discussion with Patrick Flume, MD and Lisa Saiman, MD on challenges in treating lung infections. Watch as they cover the following topics:

  • Management of patients with declining FEV1
  • Treatment options
  • Reinforcing adherence
  • Working on inhaler techniques
  • Changing inhaled antibiotics
  • Considering a continuous, cycled, or intermittent therapy approach

Interactive monograph also available!


In this issue:

Nutritional deficiencies affect nearly all patients with cystic fibrosis. But what is known about the relationship between CF-related dysfunction in the GI tract and treatment with CFTR modifiers? What does the evidence say about their effect on pancreatic, intestinal, and liver function? Can CFTR modifier therapy actually improve nutritional status?

In this issue, Dr. Michael Wilschanski, Director of Hadassah Hospital's Pediatric Gastroenterology Unit at Hebrew University in Jerusalem, analyzes the current research that seeks to answer these questions.

Learning objectives:

  • Describe how the effects of CFTR dysfunction on the gastrointestinal tract lead to nutritional deficiencies
  • Explain the possible mechanisms by which CFTR modulator therapy has been shown to improve nutritional status
  • Identify the improvements provided by CFTR modulator therapy on pancreatic, intestinal, and certain aspects of liver function


Michael Wilschanski, MBBS
Michael Wilschanski, MBBS

Director, Pediatric Gastroenterology Unit,
Division of Pediatrics
Hadassah Medical Center, Hebrew University
Jerusalem, Israel

Program Directors:

Peter J. Mogayzel, Jr., MD, PhD

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

Noah Lechtzin, MD, MHA

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

Suzanne Sullivan, RN, BSN

Senior Clinical Nurse
The Johns Hopkins University
Baltimore, MD

Length of activity:

1.0 hour Physicians
1.0 contact hour Nurses

Launch date: May 31, 2019
Expiration date: May 30, 2021