Advancing the GI frontier for patients with CF

The care of patients with Cystic Fibrosis (CF) has seen amazing advances in the past few years, made in part through the development of CFTR modulators. However, the recognition of the frequency of gastrointestinal (GI) symptoms in our patients is just beginning to emerge. Only recently have publications noted the excessively high frequency of GI issues. Using a CF GI specific questionnaire, Tabori et al. recently reported that all 131 patients at their CF center had abdominal symptoms [[1]Tabori H. Arnold C. Jaudszus A. Mentzel H.-J. Renz D.M. Reinsch S. et al.Abdominal symptoms in cystic fibrosis and their relation to genotype, history, clinical and laboratory findings.PLoS One. 2017; 12e0174463Crossref PubMed Scopus (43) Google Scholar]. Abdominal pain or flatulence was seen in 79% and distention in 63%. Children experienced more abdominal pain (87%) than adults (70%). Heartburn was reported in 61% of adults. Nausea as well as constipation was common. It is clear that much work needs to be done to improve identification and treatment of the GI symptoms associated with CF. To address these needs, in 2014 the U.S. Cystic Fibrosis Foundation funded an initiative call DIGEST – Developing Innovative Gastrointestinal Specialty Training. Through a competitive application process, this three-year training program enrolled 16 pediatric and adult gastroenterologists across the U.S. with another 16 in 2016 that also included Canada. The program consists of monthly didactic/case based web seminars, a required clinic at least monthly within a CF clinic, and lastly a scholarly project. We are excited to announce that the latter has resulted in the recently published supplement in the Journal of Cystic Fibrosis of 10 articles representing GI CF best practice guidelines [2Maqbool A. Pauwels A. Cystic Fibrosis and gastroesophageal reflux disease.J Cyst Fibros. Nov 2017; 16: S2-S13https://doi.org/10.1016/j.jcf.2017.07.007Abstract Full Text Full Text PDF PubMed Scopus (25) Google Scholar, 3Dorsey J. Gonska T. Bacterial overgrowth, dysbiosis, inflammation, and dysmotility in the Cystic Fibrosis intestine.J Cyst Fibros. 2017 Nov; 16: S14-S23https://doi.org/10.1016/j.jcf.2017.07.014Abstract Full Text Full Text PDF PubMed Scopus (52) Google Scholar, 4Lusman S.S. Grand R. Approach to chronic abdominal pain in Cystic Fibrosis.J Cyst Fibros. 2017 Nov; 16: S24-S31https://doi.org/10.1016/j.jcf.2017.06.009Abstract Full Text Full Text PDF PubMed Scopus (13) Google Scholar, 5Sathe M. Houwen R. Meconium ileus in Cystic Fibrosis.J Cyst Fibros. 2017 Nov; 16: S32-S39https://doi.org/10.1016/j.jcf.2017.06.007Abstract Full Text Full Text PDF PubMed Scopus (58) Google Scholar, 6Abraham J.M. Taylor C.J. Cystic Fibrosis & disorders of the large intestine: DIOS, constipation, and colorectal cancer.J Cyst Fibros. 2017 Nov; 16: S40-S49https://doi.org/10.1016/j.jcf.2017.06.013Abstract Full Text Full Text PDF PubMed Scopus (37) Google Scholar, 7Leung D.H. Narkewicz M.R. Cystic Fibrosis-related cirrhosis.J Cyst Fibros. 2017 Nov; 16: S50-S61https://doi.org/10.1016/j.jcf.2017.07.002Abstract Full Text Full Text PDF PubMed Scopus (38) Google Scholar, 8Assis D.N. Debray D. Gallbladder and bile duct disease in Cystic Fibrosis.J Cyst Fibros. Nov 2017; 16: S62-S69https://doi.org/10.1016/j.jcf.2017.07.006Abstract Full Text Full Text PDF PubMed Scopus (29) Google Scholar, 9Singh V.K. Schwarzenberg S.J. Pancreatic insufficiency in Cystic Fibrosis.J Cyst Fibros. 2017 Nov; 16: S70-S78https://doi.org/10.1016/j.jcf.2017.06.011Abstract Full Text Full Text PDF PubMed Scopus (86) Google Scholar, 10Freeman A.J. Ooi C.Y. Pancreatitis and pancreatic cystosis in Cystic Fibrosis.J Cyst Fibros. Nov 2017; 16: S79-S86https://doi.org/10.1016/j.jcf.2017.07.004Abstract Full Text Full Text PDF PubMed Scopus (35) Google Scholar, 11Sullivan J.S. Mascarenhas M.R. Nutrition: prevention and management of nutritional failure in Cystic Fibrosis.J Cyst Fibros. 2017 Nov; 16: S87-S93https://doi.org/10.1016/j.jcf.2017.07.010Abstract Full Text Full Text PDF PubMed Scopus (23) Google Scholar]. Since there are very few evidence-based publications in these areas, these articles instead represent a summary of what is known and best practice. Each review represents a collaborative effort between a DIGEST awardee and a senior leader in that GI area across the globe. These are available for 12 months as open access through the Journal of Cystic Fibrosis and we encourage CF teams to embrace and disseminate them to their entire caregiver team. These articles span the entire GI tract given the multitude of GI symptoms and diseases involved in patients with CF. They describe the history and importance of GI issues in CF, what is known, but also what is unknown, in the approach to the GI aspects of CF. Importantly, they highlight the prevalence of GI symptoms which often are not reported by a patient to their pulmonary CF provider. They review the differential diagnosis, diagnostic workup, and treatment options including management of complications, highlighting differences that may be seen in children versus adults. Since we are only beginning to scratch the surface in our knowledge of the GI aspects of CF, each article describes potential endpoints for testing therapies in clinical trials and how we can take advantage of specific GI outcome measures for testing new CF treatment modalities. These reviews can be thought of as version 1.0 in our armamentarium to recognize, diagnose and treat the GI symptoms and diseases seen in our patients with CF. These are meant to be usable tools for all providers in the CF clinic, rather than just GI specialists. With new diagnostic modalities and treatments, in parallel with clinical trials focused on GI outcome measures, we can move the needle on this often neglected aspect that contributes to pain and poor quality of life in our patients with CF. This is a time of amazing advances and exciting opportunities where the tools now exist to extend what has been done with the pulmonary manifestations and apply them to the GI symptoms present in so many of our patients. But this will take a dedicated cadre of GI physicians who specialize in CF to lead this charge. The U.S. CF Foundation has had the vision to develop this next generation and spawn GI specific trials in our patients with CF, a model that can be expanded globally. No conflict of interest. The DIGEST program and the support to Drs. Freedman, Wilschanski, and Schwarzenberg and this Best Practice initiative, was through grant funding from the U.S. Cystic Fibrosis Foundation.

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