• Skip to main content
  • Skip to primary sidebar
Pediatric Pulmonology: The Remaining Barriers to Normalcy in Cystic Fibrosis

The Remaining Barriers to Normalcy in Cystic Fibrosis

  • Issues
  • Resources
  • About
  • Contact
  • Search

The foregut in cystic fibrosis

By Christopher Vélez MD; Isabel Neuringer MD; Sarah Jane Schwarzenberg MD

The aerodigestive organs share a kindred embryologic origin that allows for a more complete explanation as to how the foregut can remain a barrier to normalcy in people with cystic fibrosis (pwCF). The structures of the aerodigestive tract include the nasopharynx, the oropharynx, the hypopharynx, the esophagus, the stomach, as well as the supraglottic, glottic, and subglottic tubular airways (including the trachea). Additional gastrointestinal (GI) luminal/alimentary organs of the foregut include the duodenum. Extraluminal foregut structures include the liver, the gall bladder, the biliary tree, and the pancreas. There are a variety of neurologic controls within these complicated anatomic compartments to separate the transit of food and liquid from air. These structures share the same origin from the primitive foregut/mesenchyme. The vagus nerve is a critical structure that unites respiratory and digestive functions. This article comments on the interconnected nature of cystic fibrosis and the GI tract. As it relates to the foregut, this has been typically treated as simple “reflux” as the cause of worsened lung function in pwCF. That terms like gastroesophageal reflux (GER), gastroesophageal reflux disease (GERD), heartburn, and regurgitation are used interchangeably to reflect pathology further complicates matters; we offer a more physiologically accurate term called “GI-related aspiration” or “GRASP.” Broadly, this term reflects that aspiration of foregut contents from the duodenum through the stomach to the esophagus, into the pharynx and the respiratory tree in pwCF. As a barrier to normalcy in pwCF, GRASP is fundamentally two disease processes—GERD and gastroparesis—that likely contribute most to the deterioration of lung disease in pwCF. In the modulator era, successful GRASP management will be critical, particularly in those post-lung transplantation (LTx), only through successful management of both GERD and gastroparesis. Standardization of clinical management algorithms for GRASP in CF-related GRASP is a key clinical and research gap preventing normalcy in pwCF; what exists nearly exclusively addresses surgical evaluations or offers guidance for the management of GI symptoms alone (with unclear parameters for respiratory disease considerations). We begin first by describing the result of GRASP damage to the lung in various stages of lung disease. This is followed by a discussion of the mechanisms by which the digestive tract can injure the lungs. We summarize what we anticipate future research directions will be to reduce the impact of GRASP as a barrier to normalcy in pwCF.

Read the Full Article  

Journal Issue: Remaining Barriers to Normalcy in Cystic Fibrosis: Advances in Gastroenterology, Hepatology, and Nutrition

Primary Sidebar

PP Cover - September 2024

Pediatric Pulmonology

Remaining Barriers to Normalcy in Cystic Fibrosis: Advances in Gastroenterology, Hepatology, and Nutrition

September 2024 · View Articles

The Remaining Barriers to Normalcy in Cystic Fibrosis VII

February 2022 · View Articles

The Remaining Barriers to Normalcy in Cystic Fibrosis VI

February 2021 · View Articles

List all issues »

Most Viewed Articles

  • Rapid therapeutic advances in CFTR modulator science

  • Reevaluating approaches to cystic fibrosis pulmonary exacerbations

  • Breakthrough therapies: Cystic fibrosis (CF) potentiators and correctors

  • Recent advances in the early treatment of cystic fibrosis: Bridging the gap to highly effective modulator therapy

  • Inflammation in cystic fibrosis: An update

Quiz and Survey

Test your knowledge and help shape future editions of "The Remaining Barriers to Normalcy." Participate in our Quiz and Survey »

This website is made possible by an educational grant from Vertex Pharmaceuticals Incorporated.

Vertex

  • Help & Support
  • About Us
  • Cookies & Privacy
  • Wiley Job Network
  • Terms & Conditions
  • Advertisers & Agents
Copyright © 2018-2025 John Wiley & Sons, Inc. All Rights Reserved.
The content on this site is intended for health professionals.
Wiley