Cystic Fibrosis- Nutrition and Exercise Considerations

Hosts:

• Medical Student: Brooke Pace Quertermous
• Faculty: Katie McKie, MD
• Faculty: Ryan Harris, Ph.D

Peer Review: Rebecca Yang, MD; Janelle McGill, MD

During this episode, you will learn that Cystic Fibrosis is more than just a lung disease. Individuals with CF are also at risk for malabsorption, maldigestion, intestinal obstruction, exercise intolerance, and other systemic abnormalities.

Dr. Katie McKie, Director of Pediatric Pulmonology at Augusta University, joins Dr. Ryan Harris, Founder and Director of the LIVEP and CF researcher, and Medical Student Brooke Quertermous to discuss the impact of nutrition and exercise on children with CF.

Specifically, they will: 

1. Explain the mechanism by which CF affects digestion and absorption of nutrients
2. Acknowledge the importance of frequent growth monitoring for children with CF.
3. Understand the role of nutrient supplementation for children with CF, and when enteral nutrition is required.
4. Describe why exercise intolerance occurs in CF and the necessity of regular exercise for these patients.

FREE CME Credit (requires sign-in): https://mcg.cloud-cme.com/course/courseoverview?P=0&EID=8631

Thank you for listening to this episode from the Department of Pediatrics at the Medical College of Georgia. If you have any comments, suggestions, or feedback- you can email us at mcgpediatricpodcast@augusta.edu

Remember that all content during this episode is intended for informational and educational purposes only. It should not be used as medical advice to diagnose or treat any particular patient. Clinical vignette cases presented are based on hypothetical patient scenariosWe look forward to speaking to you on our next episode of the MCG Pediatric Podcast.  

References:

1. Gajbhiye, R., et al., Cystic fibrosis transmembrane conductance regulator (CFTR) gene abnormalities in Indian males with congenital bilateral absence of vas deferens & renal anomalies. Indian J Med Res, 2016. 143(5): p. 616-23. AND Elborn, J.S., Cystic fibrosis. Lancet, 2016. 388(10059): p. 2519-2531.)
2. (Kuk, K. and J.L. Taylor-Cousar, Lumacaftor and ivacaftor in the management of patients with cystic fibrosis: current evidence and future prospects. Ther Adv Respir Dis, 2015. 9(6): p. 313-26.)
3. (Farrell et al, Siret el al, Sims et al).
4. Sullivan, J. S., & Mascarenhas, M. R. (2017). Nutrition: Prevention and management of nutritional failure in cystic fibrosis. Journal of Cystic Fibrosis, 16. doi:10.1016/j.jcf.2017.07.010
5. Committee On Practice And Ambulatory Medicine, & Workgroup, B. (2020, March 01). 2020 recommendations for Preventive Pediatric health care. Retrieved March 22, 2021, from https://pediatrics.aappublications.org/content/145/3/e20200013
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7. (Nutrition Prevention and Management Article)
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9. Ameet Daftary, James Acton, James Heubi, Raouf Amin, Fecal elastase-1: Utility in pancreatic function in cystic fibrosis, Journal of Cystic Fibrosis, Volume 5, Issue 2, 2006, Pages 71-76,ISSN 1569-1993,
10. Walkowiak, D. Sands, A. Nowakowska, R. Piotrowski, K. Zybert, K.H. Herzig, et al. Early decline of pancreatic function in cystic fibrosis patients with class 1 or 2 CFTR mutations J Pediatr Gastroenterol Nutr, 40 (2) (2005), pp. 199-201
11. Witt, H. (2003). Chronic pancreatitis and cystic fibrosis. Gut, 52(90002), 31ii-41. doi:10.1136/gut.52.suppl_2.ii31
12. Fielding, J., Brantley, L., Seigler, N., McKie, K. T., Davison, G. W., & Harris, R. A. (2015). Oxygen uptake kinetics and exercise capacity in children with cystic fibrosis. Pediatric Pulmonology, 50(7), 647-654. doi:10.1002/ppul.23189
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15. Pastré, J., Prévotat, A., Tardif, C., Langlois, C., Duhamel, A., & Wallaert, B. (2014). Determinants of exercise capacity in cystic fibrosis patients with mild-to-moderate lung disease. BMC pulmonary medicine, 14(1), 1-8.
16. Hulzebos, Erik H. J.1; Bomhof-Roordink, Hanna1,3; van de Weert-van Leeuwen, Pauline B.2; Twisk, Jos W. R.3; Arets, H. G. M.2; van der Ent, Cornelis K.2; Takken, Tim1 Prediction of Mortality in Adolescents with Cystic Fibrosis, Medicine & Science in Sports & Exercise: November 2014 - Volume 46 - Issue 11 - p 2047-2052 doi: 10.1249/MSS.0000000000000344
17. Ding S., Zhong C. (2020) Exercise and Cystic Fibrosis. In: Xiao J. (eds) Physical Exercise for Human Health. Advances in Experimental Medicine and Biology, vol 1228. Springer, Singapore. https://doi.org/10.1007/978-981-15-1792-1_26
18. Moorcroft AJ, Dodd ME, Morris J, Webb AK. Individualised unsupervised exercise training in adults with cystic fibrosis: a 1 year randomised controlled trial. Thorax. 2004 Dec;59(12):1074-80. doi: 10.1136/thx.2003.015313. PMID: 15563708; PMCID: PMC1746905.
19. Pianosi P, Leblanc J, Almudevar A. Peak oxygen uptake and mortality in children with cystic fibrosis. Thorax. Jan 2005;60(1):50-54.

LIVEP Contact: Reva Crandall at 706-721-5483.