Volume 9, Issue 12

CFTR Modulators vs Malnutrition

In this issue:

The effects of malnutrition in people with cystic fibrosis are critical concerns for patients and their health care providers. The condition is especially dangerous for children with CF and has been associated with delayed development, as well as increased odds of developing severe lung disease. Beyond PERT and specialized feeding regimens, many are asking about the potential benefits of treatment with CFTR modulators. What does the evidence say? 

That’s the focus of this issue of eCysticFibrosis Review, authored by Dr. Vikesh Singh, Associate Professor of Medicine and Director of the Pancreatitis Center at Johns Hopkins Medical Institutions. 

Learning objectives:

  • Explain the mechanisms of malnutrition in patients with cystic fibrosis.  
  • Describe the impact of CFTR modulators on parameters of nutrition, exocrine function, and recurrent acute pancreatitis in patients with cystic fibrosis. 

Author:

Vikesh K. Singh, MD, MSc
Vikesh K. Singh, MD, MSc

Associate Professor of Medicine, Division of Gastroenterology, Department of Medicine 
Johns Hopkins University School of Medicine 
Director of Endoscopy 
Johns Hopkins Hospital 
Director, Pancreatitis Center 
Medical Director, Pancreatic Islet Autotransplantation Program 
Johns Hopkins Medical Institutions 
Baltimore, MD

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: March 19, 2021
Expiration date: January 8, 2023