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Neurogastroenterology and Motility Disorders of the Gastrointestinal Tract in Cystic Fibrosis

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Abstract

Purpose of Review

To discuss all the various motility disorders impacting people with Cystic Fibrosis (PwCF) and provide diagnostic and management approaches from a group of pediatric and adult CF and motility experts and physiologists with experience in the management of this disease.

Recent Findings

Gastrointestinal (GI) symptoms coexist with pulmonary symptoms in PwCF regardless of age and sex. The GI manifestations include gastroesophageal reflux disease, esophageal dysmotility gastroparesis, small bowel dysmotility, small intestinal bacterial overgrowth syndrome, distal idiopathic obstruction syndrome, constipation, and pelvic floor disorders. They are quite debilitating, limiting the patients’ quality of life and affecting their nutrition and ability to socialize. This genetic disorder affects many organ systems and is chronic, potentially impacting fertility and future family planning, requiring a multidisciplinary approach.

Summary

Our review discusses the treatments of motility disorders in CF, their prevalence and pathophysiology. We have provided a framework for clinicians who care for these patients that can help to guide their clinical management.

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Data Availability

The manometry data is available deidentified upon request from our editors.

Our paper is original and has not been previously published elsewhere.

All authors have reviewed and agree with the contents of the review paper as provided.

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Acknowledgements

We all thank the cystic fibrosis foundation and the countless numbers of people with cystic fibrosis who have contributed to our careers and the contents of this review.

Funding

Cystic Fibrosis Foundation: Award #00451A121.

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Authors and Affiliations

  1. Department of Pediatrics, Pediatric Gastroenterology, Hepatology and Nutrition, SSM Cardinal Glennon Children’s Medical Center, Saint Louis University, St Louis, MO, USA

    Dhiren Patel

  2. Pediatric Gastroenterology, Hepatology, and Nutrition, Clinical Associate Professor, Levine Childrens Hospital, Carolina Pediatric Gastroenterology, Charlotte, NC, USA

    Folashade Jose

  3. Anx Robotics, Plano, TX, USA

    Jason Baker

  4. Division of Gastroenterology, Atrium Health Wake Forest Medical University, Charlotte, NC, USA

    Baha Moshiree

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Contributions

DP and FJ are both first coauthors and were responsible for writing, design and editing of the manuscript. JB contributed to the writing, design and editing of the manuscript BM also contributed to the writing of the manuscript, provided the patient case and tables being presented here, the abstract and conclusion. She serves as the corresponding author on the manuscript and is also mentor to both DP and FJ as part of the Cystic Fibrosis Foundations DIGEST Awardees (DP and FJ).

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Correspondence to Baha Moshiree.

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Competing interests

The authors declare no competing interests.

Ethical Approval

No animals or humans participated in this review paper. The one patient whose manometry was used consented to participation in this data but as no identifiers are used, our IRB at Atrium Health does not require informed consent as this is exempt from requiring IRB consent.

Human and Animal Rights

This review does not contain any studies of human or animal subjects performed by any of the authors.

Conflict of Interest

Dhiren Patel: None.

Folashade Jose: None.

Jason Baker: Anx Robotics.

Baha Moshiree: Ardelyx: Speaker and Advisory Board, Medtronic- Research grant support, Takeda: Advisory board, Ironwood Pharmaceuticals: Advisory board and speaker, Abbvie- Advisory board, Bausch/Salix pharmaceuticals: Speaker and Grant Support, Nestle Foundation: Speaker, Restalsis: Research Grant support, Coloplast: research grant support, Atmo- Research grant support, advisory board.

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Dhiren Patel and Folashade Jose shared first authorship.

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Patel, D., Jose, F., Baker, J. et al. Neurogastroenterology and Motility Disorders of the Gastrointestinal Tract in Cystic Fibrosis. Curr Gastroenterol Rep 26, 9–19 (2024). https://doi.org/10.1007/s11894-023-00906-4

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