The foregut in cystic fibrosis

Area of Science:

• Aerodigestive tract embryology and physiology
• Cystic Fibrosis (CF) pathophysiology
• Gastrointestinal (GI) and respiratory system interactions

Background:

• Aerodigestive organs share a common embryologic origin, influencing their function in people with cystic fibrosis (pwCF).
• The interconnectedness of the GI tract and respiratory system is critical in understanding CF progression.
• Existing terminology like GER and GERD is insufficient to describe the complex pathology in pwCF.

Purpose of the Study:

• To introduce and define a new term, GI-related aspiration (GRASP), for the aspiration of foregut contents into the respiratory tract in pwCF.
• To explain how GRASP, encompassing GERD and gastroparesis, contributes to lung disease deterioration in pwCF.
• To highlight the need for standardized clinical management algorithms for GRASP in CF.

Main Methods:

• Review of aerodigestive tract embryology and neuroanatomic controls.
• Analysis of the pathophysiology linking foregut dysfunction to lung injury in pwCF.
• Discussion of the dual disease processes (GERD and gastroparesis) constituting GRASP.

Main Results:

• GRASP, a combination of GERD and gastroparesis, is identified as a significant barrier to normalcy in pwCF.
• Aspiration of foregut contents from the duodenum to the respiratory tree contributes to lung damage.
• Current management strategies for GI symptoms in CF are inadequate, lacking respiratory considerations.

Conclusions:

• Effective management of GRASP, by addressing both GERD and gastroparesis, is essential for improving lung health in pwCF.
• Standardized clinical management algorithms for GRASP are a critical research gap.
• Future research should focus on reducing the impact of GRASP to improve outcomes for pwCF, especially post-lung transplantation.