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Beyond the lungs: The new GI frontier
JCF-01343; No of Pages 2
Journal of Cystic Fibrosis xx (2016) xxx – xxx www.elsevier.com/locate/jcf
Editorial
Beyond the lungs: The new GI frontier Steven D. Freedman a,⁎,1 , Sarah Jane Schwarzenberg b,1 a
Division of Gastroenterology, Beth Israel Deaconess Medical Center and Division of Respiratory Diseases, Boston Children's Hospital; Harvard Medical School, Boston, MA, United States b Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, United States
The decades-long emphasis on managing the pulmonary complications of cystic fibrosis (CF) has resulted in both improved life span and improved quality of life for these patients. The development of dedicated programs to train pulmonologists as well as the Program in Adult Care Excellence (PACE) through the U.S. CF Foundation and the implementation of a core curriculum for trainees in adult respiratory medicine by the European Respiratory Society/ European CF Society task force on the care of adults with CF play a critical role in achieving these goals across the age spectrum [1]. However, as people with CF live longer, they have heightened expectations of normal life cycle events (e.g. college graduation, careers, marriage, parenthood). The burden of personal care and the limitations of pulmonary disease continue to be factors preventing the realization of these long-term ambitions. So too are the non-pulmonary manifestations of CF. CF is a multi-organ disease affecting not only the lungs, but also the gastrointestinal system as well as the reproductive tract and endocrine system. Many lines of evidence show that the complications of the gastrointestinal tract directly affect quality of life and survival through the genesis of symptoms and its impact on nutritional status. Not only has pancreatic enzyme replacement therapy been lifesaving in our patients with CF, the data is clear linking nutrition with lung function [2]. Weight for age percentile at age 4 is predictive of lung function, risk for diabetes, and mortality at age 18 [3].
⁎ Corresponding author at: Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Dana 501, Boston, MA 02215, United States. Tel: +1 617 667 5576; fax: +1 617 667 5826. E-mail address: [email protected] (S.D. Freedman). 1 Co-Directors, DIGEST (Developing Innovative Gastrointestinal Training) Program funded through the U.S. Cystic Fibrosis Foundation.
The three articles in this issue of JCF illustrate relatively common problems that our patients with CF face throughout the age spectrum [4,5,6]. However, they also highlight the challenge: if gastrointestinal disease is of such importance in the lives of our patients with CF, it suggests that we need a cadre of gastroenterologists dedicated to CF. Ideally, these specialists would be familiar with CF disease and management as a whole, but also experts in the diagnosis and management of the gastrointestinal and nutritional aspects of CF. Because patients with CF are surviving much longer than ever before (the current estimated lifespan of a child born today with CF exceeds 50 years), gastroenterologists from both pediatric and adult programs are needed to function as integral members of the CF Care Team and provide continuous care with age-appropriate transitions. To address these needs, in 2014 the U.S. CF Foundation created a training program called DIGEST (Developing Innovative Gastrointestinal Training). This three-year on-the-job educational program focuses on training pediatric and adult gastroenterologists across the U.S. in the management of the GI aspects of CF. These junior faculty gastroenterologists must have the commitment of their division chiefs to provide time for educational and clinical training, and the commitment of their local CF Center Director (usually a Pulmonologist) to provide training in CF care, and access to CF patients with gastrointestinal disease. Monthly webinars, both didactic and case-based, provide training from senior gastroenterologists with expertise in CF. Importantly, the CF Foundation provides financial support to offset the likely loss of revenue associated with this cognitive training that deemphasizes endoscopic procedures. As our first cohort of trainees approaches graduation, we believe that we offer their institutions a group of pediatric and adult gastroenterologists uniquely trained to partner in the CF clinic in the management of these complex patients across the age spectrum. The European Respiratory Society/European CF Society task force on the care
http://dx.doi.org/10.1016/j.jcf.2016.05.008 1569-1993© 2016 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. Please cite this article as: Freedman SD, Schwarzenberg SJ, Beyond the lungs: The new GI frontier, J Cyst Fibros (2016), http://dx.doi.org/10.1016/j.jcf.2016.05.008
2
Editorial
of adults with CF has similarly incorporated gastroenterologists and other specialists into a core curriculum [1]. Each of the 3 articles in this issue of JCF highlights how the program goals of DIGEST and nascent published data can be readily incorporated into CF patient care. For example, the prospective observational longitudinal study of DIOS by Munck et al. examined data from pediatric and adult CF centers across Europe and Israel and found that a prior history of meconium ileus was a risk factor for DIOS and associated with a higher surgery rate [4]. Additionally, a delay in receiving medical assistance and weight loss were associated with poorer outcomes. Thus, early recognition and treatment to avoid unnecessary surgery is likely to improve patient outcomes. Hauser et al. examined whether the increased prevalence of gastroesophageal reflux disease is associated with delayed gastric emptying. Similar to other studies, gastroparesis was present in 21% [5]. Importantly this diagnosis was not necessarily related to acid reflux. Thus the routine evaluation of gastroparesis in patients with acid reflux is unlikely to be beneficial in alleviating reflux symptoms. Lastly, Niccum et al. demonstrated through a prospective study that 49% of adults with CF with a mean age of 46 years had adenomatous polyps on their first colonoscopy with 32% having advanced pathology or N 3 polyps and of concern, 3% were found to have colonic adenocarcinoma [6]. These results have major implications in changing the current paradigm for colorectal screening including defining optimum bowel preps, ensuring safe sedation techniques given the pulmonary disease, and coordinating screening prior to lung transplantation. Taken together, these articles emphasize the need for dedicated, knowledgeable CF trained gastroenterologists who are integrated with the care team, to provide efficacious and timely treatments. There remain risks. Success requires commitment, time, and money. Sponsoring institutions must continue to provide opportunities for these young pediatric and adult CF gastroenterologists to expand their experience, develop best practices, and foster GI focused research programs in CF. Pulmonologists (and the broader CF care team) need to engage with these young experts, incorporating them into CF Centers and CF research, to
promote and maintain bidirectional engagement that will optimize the management of the gastrointestinal aspects in CF. DIGEST serves as a scalable model that can be implemented outside of the U.S. to train gastroenterologists as well as other needed specialists to foster the goals of multidisciplinary, expert care in the management of CF. In fact, based on the success of DIGEST, a similarly structured program called EnVision to train emerging leaders in CF endocrinology, was recently been launched by the U.S. CF Foundation. Lastly, it is critical that we provide the same evidence-based guidance for diagnosis and management of gastrointestinal disease in CF that we have traditionally demanded for the pulmonary disease. The articles in this issue provide a start for defining best practice guidelines that will serve as the base for continuous quality improvement and for the development of clinical research questions to improve the health and lives of people with CF. References [1] Elborn JS, Bell SC, Madge SL, Burgel PR, Castellani C, Conway S, et al. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis. Eur Respir J 2016 Feb;47(2):420–8. [2] Pencharz PB, Durie PR. Pathogenesis of malnutrition in cystic fibrosis, and its treatment. Clin Nutr 2000 Dec;19(6):387–94. [3] Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr 2013 Mar;162(3):530–5. [4] Munck A, Alberti C, Colombo C, Kashirskaya N, Ellemunter H, Fotoulaki M, et al. International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: associated factors and outcome. J Cyst Fibros 2016;15(4). http://dx.doi.org/10.1016/j.jcf.2016.02.002 [in this issue]. [5] Hauser B, De Schepper J, Malfroot A, De Wachter E, De Schutter I, Keymolen K, et al. Gastric emptying and gastro-oesophageal reflux in children with cystic fibrosis. J Cyst Fibros 2016;15(4). http://dx.doi.org/10. 1016/j.jcf.2015.12.015 [in this issue]. [6] Niccum DE, Billings JL, Dunitz JM, Khoruts A. Colonoscopic screening shows increased early incidence and progression of adenomas in cystic fibrosis. J Cyst Fibros 2016;15(4). http://dx.doi.org/10.1016/j.jcf.2016.01. 002 [in this issue].
Please cite this article as: Freedman SD, Schwarzenberg SJ, Beyond the lungs: The new GI frontier, J Cyst Fibros (2016), http://dx.doi.org/10.1016/j.jcf.2016.05.008
Journal of Cystic Fibrosis xx (2016) xxx – xxx www.elsevier.com/locate/jcf
Editorial
Beyond the lungs: The new GI frontier Steven D. Freedman a,⁎,1 , Sarah Jane Schwarzenberg b,1 a
Division of Gastroenterology, Beth Israel Deaconess Medical Center and Division of Respiratory Diseases, Boston Children's Hospital; Harvard Medical School, Boston, MA, United States b Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of Minnesota Masonic Children's Hospital, Minneapolis, MN, United States
The decades-long emphasis on managing the pulmonary complications of cystic fibrosis (CF) has resulted in both improved life span and improved quality of life for these patients. The development of dedicated programs to train pulmonologists as well as the Program in Adult Care Excellence (PACE) through the U.S. CF Foundation and the implementation of a core curriculum for trainees in adult respiratory medicine by the European Respiratory Society/ European CF Society task force on the care of adults with CF play a critical role in achieving these goals across the age spectrum [1]. However, as people with CF live longer, they have heightened expectations of normal life cycle events (e.g. college graduation, careers, marriage, parenthood). The burden of personal care and the limitations of pulmonary disease continue to be factors preventing the realization of these long-term ambitions. So too are the non-pulmonary manifestations of CF. CF is a multi-organ disease affecting not only the lungs, but also the gastrointestinal system as well as the reproductive tract and endocrine system. Many lines of evidence show that the complications of the gastrointestinal tract directly affect quality of life and survival through the genesis of symptoms and its impact on nutritional status. Not only has pancreatic enzyme replacement therapy been lifesaving in our patients with CF, the data is clear linking nutrition with lung function [2]. Weight for age percentile at age 4 is predictive of lung function, risk for diabetes, and mortality at age 18 [3].
⁎ Corresponding author at: Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Dana 501, Boston, MA 02215, United States. Tel: +1 617 667 5576; fax: +1 617 667 5826. E-mail address: [email protected] (S.D. Freedman). 1 Co-Directors, DIGEST (Developing Innovative Gastrointestinal Training) Program funded through the U.S. Cystic Fibrosis Foundation.
The three articles in this issue of JCF illustrate relatively common problems that our patients with CF face throughout the age spectrum [4,5,6]. However, they also highlight the challenge: if gastrointestinal disease is of such importance in the lives of our patients with CF, it suggests that we need a cadre of gastroenterologists dedicated to CF. Ideally, these specialists would be familiar with CF disease and management as a whole, but also experts in the diagnosis and management of the gastrointestinal and nutritional aspects of CF. Because patients with CF are surviving much longer than ever before (the current estimated lifespan of a child born today with CF exceeds 50 years), gastroenterologists from both pediatric and adult programs are needed to function as integral members of the CF Care Team and provide continuous care with age-appropriate transitions. To address these needs, in 2014 the U.S. CF Foundation created a training program called DIGEST (Developing Innovative Gastrointestinal Training). This three-year on-the-job educational program focuses on training pediatric and adult gastroenterologists across the U.S. in the management of the GI aspects of CF. These junior faculty gastroenterologists must have the commitment of their division chiefs to provide time for educational and clinical training, and the commitment of their local CF Center Director (usually a Pulmonologist) to provide training in CF care, and access to CF patients with gastrointestinal disease. Monthly webinars, both didactic and case-based, provide training from senior gastroenterologists with expertise in CF. Importantly, the CF Foundation provides financial support to offset the likely loss of revenue associated with this cognitive training that deemphasizes endoscopic procedures. As our first cohort of trainees approaches graduation, we believe that we offer their institutions a group of pediatric and adult gastroenterologists uniquely trained to partner in the CF clinic in the management of these complex patients across the age spectrum. The European Respiratory Society/European CF Society task force on the care
http://dx.doi.org/10.1016/j.jcf.2016.05.008 1569-1993© 2016 Published by Elsevier B.V. on behalf of European Cystic Fibrosis Society. Please cite this article as: Freedman SD, Schwarzenberg SJ, Beyond the lungs: The new GI frontier, J Cyst Fibros (2016), http://dx.doi.org/10.1016/j.jcf.2016.05.008
2
Editorial
of adults with CF has similarly incorporated gastroenterologists and other specialists into a core curriculum [1]. Each of the 3 articles in this issue of JCF highlights how the program goals of DIGEST and nascent published data can be readily incorporated into CF patient care. For example, the prospective observational longitudinal study of DIOS by Munck et al. examined data from pediatric and adult CF centers across Europe and Israel and found that a prior history of meconium ileus was a risk factor for DIOS and associated with a higher surgery rate [4]. Additionally, a delay in receiving medical assistance and weight loss were associated with poorer outcomes. Thus, early recognition and treatment to avoid unnecessary surgery is likely to improve patient outcomes. Hauser et al. examined whether the increased prevalence of gastroesophageal reflux disease is associated with delayed gastric emptying. Similar to other studies, gastroparesis was present in 21% [5]. Importantly this diagnosis was not necessarily related to acid reflux. Thus the routine evaluation of gastroparesis in patients with acid reflux is unlikely to be beneficial in alleviating reflux symptoms. Lastly, Niccum et al. demonstrated through a prospective study that 49% of adults with CF with a mean age of 46 years had adenomatous polyps on their first colonoscopy with 32% having advanced pathology or N 3 polyps and of concern, 3% were found to have colonic adenocarcinoma [6]. These results have major implications in changing the current paradigm for colorectal screening including defining optimum bowel preps, ensuring safe sedation techniques given the pulmonary disease, and coordinating screening prior to lung transplantation. Taken together, these articles emphasize the need for dedicated, knowledgeable CF trained gastroenterologists who are integrated with the care team, to provide efficacious and timely treatments. There remain risks. Success requires commitment, time, and money. Sponsoring institutions must continue to provide opportunities for these young pediatric and adult CF gastroenterologists to expand their experience, develop best practices, and foster GI focused research programs in CF. Pulmonologists (and the broader CF care team) need to engage with these young experts, incorporating them into CF Centers and CF research, to
promote and maintain bidirectional engagement that will optimize the management of the gastrointestinal aspects in CF. DIGEST serves as a scalable model that can be implemented outside of the U.S. to train gastroenterologists as well as other needed specialists to foster the goals of multidisciplinary, expert care in the management of CF. In fact, based on the success of DIGEST, a similarly structured program called EnVision to train emerging leaders in CF endocrinology, was recently been launched by the U.S. CF Foundation. Lastly, it is critical that we provide the same evidence-based guidance for diagnosis and management of gastrointestinal disease in CF that we have traditionally demanded for the pulmonary disease. The articles in this issue provide a start for defining best practice guidelines that will serve as the base for continuous quality improvement and for the development of clinical research questions to improve the health and lives of people with CF. References [1] Elborn JS, Bell SC, Madge SL, Burgel PR, Castellani C, Conway S, et al. Report of the European Respiratory Society/European Cystic Fibrosis Society task force on the care of adults with cystic fibrosis. Eur Respir J 2016 Feb;47(2):420–8. [2] Pencharz PB, Durie PR. Pathogenesis of malnutrition in cystic fibrosis, and its treatment. Clin Nutr 2000 Dec;19(6):387–94. [3] Yen EH, Quinton H, Borowitz D. Better nutritional status in early childhood is associated with improved clinical outcomes and survival in patients with cystic fibrosis. J Pediatr 2013 Mar;162(3):530–5. [4] Munck A, Alberti C, Colombo C, Kashirskaya N, Ellemunter H, Fotoulaki M, et al. International prospective study of distal intestinal obstruction syndrome in cystic fibrosis: associated factors and outcome. J Cyst Fibros 2016;15(4). http://dx.doi.org/10.1016/j.jcf.2016.02.002 [in this issue]. [5] Hauser B, De Schepper J, Malfroot A, De Wachter E, De Schutter I, Keymolen K, et al. Gastric emptying and gastro-oesophageal reflux in children with cystic fibrosis. J Cyst Fibros 2016;15(4). http://dx.doi.org/10. 1016/j.jcf.2015.12.015 [in this issue]. [6] Niccum DE, Billings JL, Dunitz JM, Khoruts A. Colonoscopic screening shows increased early incidence and progression of adenomas in cystic fibrosis. J Cyst Fibros 2016;15(4). http://dx.doi.org/10.1016/j.jcf.2016.01. 002 [in this issue].
Please cite this article as: Freedman SD, Schwarzenberg SJ, Beyond the lungs: The new GI frontier, J Cyst Fibros (2016), http://dx.doi.org/10.1016/j.jcf.2016.05.008