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Nutritional Management of Cystic Fibrosis Related Diabetes Mellitus

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Nutrition in Cystic Fibrosis

Part of the book series: Nutrition and Health ((NH))

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Abstract

Cystic Fibrosis Related Diabetes Mellitus (CFRD) is a serious and common complication of cystic fibrosis. CFRD is a unique form of diabetes, which is different in cause and in approach to nutrition therapy from the more well-known type 1 and type 2 diabetes mellitus. CFRD is a silent disease and one of the first manifestations may be nutritional failure. CFRD leads to more rapid lung function decline and earlier death in patients with CF and therefore screening and treatment of this disorder are essential to the care of CF patients. As with all forms of diabetes, nutrition therapy is essential to the care of the CF patient with CFRD. However, the nature of CF nutritional needs and the nature and pathophysiology of CFRD itself means that typical diabetic nutrition education is not sufficient for the patient with CF and diabetic nutrition education must be tailored to the specific needs of the patient with CF and diabetes. Additional concerns can be alterations in insulin sensitivity with illness, need for supplemental feeding, the use of steroid therapy, and potential for disordered eating, as well as the overarching need for high caloric intake. The only acceptable treatment for CFRD is insulin, which means that carbohydrate counting and management are essential to the successful management of this disease. However, treatment of CFRD normally results in reversal of nutritional failure and improvement in overall health of the patient with CF.

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References

  1. Moran A, Doherty L, Wang X, Thomas W. Abnormal glucose metabolism in cystic fibrosis. J Pediatr. 1998;133:10–7.

    Article  CAS  PubMed  Google Scholar 

  2. Frohnert BI, Ode KL, Moran A, Nathan BM, Laguna T, Holme B, Thomas W. Impaired fasting glucose in cystic fibrosis. Diabetes Care. 2010;33:2660–4.

    Article  PubMed Central  PubMed  Google Scholar 

  3. Moran A, Dunitz J, Nathan B, Saeed A, Holme B, Thomas W. Cystic fibrosis-related diabetes: current trends in prevalence, incidence, and mortality. Diabetes Care. 2009;32:1626–31.

    Article  PubMed Central  PubMed  Google Scholar 

  4. Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H. Evidence-based practice recommendations for nutrition-related management of children and adults with cystic fibrosis and pancreatic insufficiency: results of a systematic review. J Am Diet Assoc. 2008;108:832–9.

    Article  PubMed  Google Scholar 

  5. Cystic Fibrosis Foundation (CFF). Patient Registry. 2012 Annual Data Report. Bethesda, MD; 2012.

    Google Scholar 

  6. Moran A, Brunzell C, Cohen RC, Katz M, Marshall BC, Onady G, Robinson KA, Sabadosa KA, Stecenko A, Slovis B. Clinical care guidelines for cystic fibrosis-related diabetes: a position statement of the American Diabetes Association and a clinical practice guideline of the Cystic Fibrosis Foundation, endorsed by the Pediatric Endocrine Society. Diabetes Care. 2010;33:2697–708.

    Article  PubMed Central  PubMed  Google Scholar 

  7. Borowitz D, Baker RD, Stallings V, et al. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr. 2002;35:246–59.

    Article  PubMed  Google Scholar 

  8. White H, Pollard K, Etherington C, Clifton I, Morton AM, Owen D, Conway SP, Peckham DG. Nutritional decline in cystic fibrosis related diabetes: the effect of intensive nutritional intervention. J Cyst Fibros. 2009;8:179–85.

    Article  CAS  PubMed  Google Scholar 

  9. Lanng S, Thorsteinsson B, Nerup J, Koch C. Influence of the development of diabetes mellitus on clinical status in patients with cystic fibrosis. Eur J Pediatr. 1992;151:684–7.

    Article  CAS  PubMed  Google Scholar 

  10. Lanng S, Thorsteinsson B, Nerup J, Koch C. Diabetes mellitus in cystic fibrosis: effect of insulin therapy on lung function and infections. Acta Paediatr. 1994;83:849–53.

    Article  CAS  PubMed  Google Scholar 

  11. Gougeon R, Pencharz PB, Marliss EB. Effect of NIDDM on the kinetics of whole-body protein metabolism. Diabetes. 1994;43:318–28.

    Article  CAS  PubMed  Google Scholar 

  12. Figueroa V, Milla C, Parks EJ, Schwarzenberg SJ, Moran A. Abnormal lipid concentrations in cystic fibrosis. Am J Clin Nutr. 2002;75:1005–11.

    CAS  PubMed  Google Scholar 

  13. Rhodes B, Nash EF, Tullis E, Pencharz PB, Brotherwood M, Dupuis A, Stephenson A. Prevalence of dyslipidemia in adults with cystic fibrosis. J Cyst Fibros. 2010;9:24–8.

    Article  CAS  PubMed  Google Scholar 

  14. Stephenson AL, Mannik LA, Walsh S, Brotherwood M, Robert R, Darling PB, Nisenbaum R, Moerman J, Stanojevic S. Longitudinal trends in nutritional status and the relation between lung function and BMI in cystic fibrosis: a population-based cohort study. Am J Clin Nutr. 2013;97:872–7.

    Article  CAS  PubMed  Google Scholar 

  15. Schwarzenberg SJ, Thomas W, Olsen TW, Grover T, Walk D, Milla C, Moran A. Microvascular complications in cystic fibrosis-related diabetes. Diabetes Care. 2007;30:1056–61.

    Article  PubMed  Google Scholar 

  16. Andersen HU, Lanng S, Pressler T, Laugesen CS, Mathiesen ER. Cystic fibrosis-related diabetes: the presence of microvascular diabetes complications. Diabetes Care. 2006;29:2660–3.

    Article  PubMed  Google Scholar 

  17. Van den Berg JMW, Morton AM, Kok SW, Pijl H, Conway SP, Heijerman HGM. Microvascular complications in patients with cystic fibrosis-related diabetes (CFRD). J Cyst Fibros. 2008;7:515–9.

    Article  PubMed  Google Scholar 

  18. Moran A, Diem P, Klein DJ, Levitt MD, Robertson RP. Pancreatic endocrine function in cystic fibrosis. J Pediatr. 1991;118:715–23.

    Article  CAS  PubMed  Google Scholar 

  19. Wilson DC, Kalnins D, Stewart C, Hamilton N, Hanna AK, Durie PR, Tullis E, Pencharz PB. Challenges in the dietary treatment of cystic fibrosis related diabetes mellitus. Clin Nutr. 2000;19:87–93.

    Article  CAS  PubMed  Google Scholar 

  20. Orenstein D. Cystic fibrosis. In: Rudolph A, Hostetter M, Lister G, Siegel N, editors. Rudolph’s pediatrics. New York: McGraw Hill; 2002. p. 1969–80.

    Google Scholar 

  21. Gaskin K. Exocrine pancreatic function. In: Walker W, Goulet O, Kleinman R, Sherman P, Schneider B, Sanderson I, editors. Pediatric gastrointestinal disease. Hamilton: B.C. Decker; 2004. p. 1607–23.

    Google Scholar 

  22. Tofé S, Moreno JC, Máiz L, Alonso M, Escobar H, Barrio R. Insulin-secretion abnormalities and clinical deterioration related to impaired glucose tolerance in cystic fibrosis. Eur J Endocrinol. 2005;152:241–7.

    Article  PubMed  Google Scholar 

  23. Blackman SM, Hsu S, Vanscoy LL, Collaco JM, Ritter SE, Naughton K, Cutting GR. Genetic modifiers play a substantial role in diabetes complicating cystic fibrosis. J Clin Endocrinol Metab. 2009;94:1302–9.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  24. Milla CE, Warwick WJ, Moran A. Trends in pulmonary function in patients with cystic fibrosis correlate with the degree of glucose intolerance at baseline. Am J Respir Crit Care Med. 2000;162:891–5.

    Article  CAS  PubMed  Google Scholar 

  25. Rosenecker J, Höfler R, Steinkamp G, Eichler I, Smaczny C, Ballmann M, Posselt HG, Bargon J, von der Hardt H. Diabetes mellitus in patients with cystic fibrosis: the impact of diabetes mellitus on pulmonary function and clinical outcome. Eur J Med Res. 2001;6:345–50.

    CAS  PubMed  Google Scholar 

  26. Moran A, Pekow P, Grover P, Zorn M, Slovis B, Pilewski J, Tullis E, Liou TG, Allen H. Insulin therapy to improve BMI in cystic fibrosis-related diabetes without fasting hyperglycemia: results of the cystic fibrosis related diabetes therapy trial. Diabetes Care. 2009;32:1783–8.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  27. Brodsky J, Dougherty S, Makani R, Rubenstein RC, Kelly A. Elevation of 1-hour plasma glucose during oral glucose tolerance testing is associated with worse pulmonary function in cystic fibrosis. Diabetes Care. 2011;34:292–5.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  28. Hameed S, Morton JR, Jaffé A, Field PI, Belessis Y, Yoong T, Katz T, Verge CF. Early glucose abnormalities in cystic fibrosis are preceded by poor weight gain. Diabetes Care. 2010;33:221–6.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  29. Ode KL, Frohnert B, Laguna T, Phillips J, Holme B, Regelmann W, Thomas W, Moran A. Oral glucose tolerance testing in children with cystic fibrosis. Pediatr Diabetes. 2010;11:487–92.

    Article  CAS  PubMed  Google Scholar 

  30. Bizzarri C, Lucidi V, Ciampalini P, Bella S, Russo B, Cappa M. Clinical effects of early treatment with insulin glargine in patients with cystic fibrosis and impaired glucose tolerance. J Endocrinol Invest. 2006;29:RC1–4.

    Article  CAS  PubMed  Google Scholar 

  31. Dobson L, Hattersley AT, Tiley S, Elworthy S, Oades PJ, Sheldon CD. Clinical improvement in cystic fibrosis with early insulin treatment. Arch Dis Child. 2002;87:430–1.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  32. Waugh N, Royle P, Craigie I, Ho V, Pandit L, Ewings P, Adler A, Helms P, Sheldon C. Screening for cystic fibrosis-related diabetes: a systematic review. Health Technol Assess. 2012;16. iii–iv, 1–179.

    Google Scholar 

  33. Jefferies C, Solomon M, Perlman K, Sweezey N, Daneman D. Continuous glucose monitoring in adolescents with cystic fibrosis. J Pediatr. 2005;147:396–8.

    Article  PubMed  Google Scholar 

  34. Khammar A, Stremler N, Dubus J-C, Gross G, Sarles J, Reynaud R. Value of continuous glucose monitoring in screening for diabetes in cystic fibrosis. Arch Pediatr. 2009;16:1540–6.

    Article  CAS  PubMed  Google Scholar 

  35. Franco WB, Brown RF, Bremer AA. Use of “glucola alternatives” for cystic fibrosis-related diabetes screening. J Cyst Fibros. 2011;10:384–5.

    Article  PubMed  Google Scholar 

  36. Holl RW, Buck C, Babka C, Wolf A, Thon A. HbA1c is not recommended as a screening test for diabetes in cystic fibrosis. Diabetes Care. 2000;23:126.

    Article  CAS  PubMed  Google Scholar 

  37. Godbout A, Hammana I, Potvin S, Mainville D, Rakel A, Berthiaume Y, Chiasson J-L, Coderre L, Rabasa-Lhoret R. No relationship between mean plasma glucose and glycated haemoglobin in patients with cystic fibrosis-related diabetes. Diabetes Metab. 2008;34:568–73.

    Article  CAS  PubMed  Google Scholar 

  38. Hardin DS, Rice J, Rice M, Rosenblatt R. Use of the insulin pump in treat cystic fibrosis related diabetes. J Cyst Fibros. 2009;8:174–8.

    Article  CAS  PubMed  Google Scholar 

  39. Sunni M, Bellin MD, Moran A. Exogenous insulin requirements do not differ between youth and adults with cystic fibrosis related diabetes. Pediatr Diabetes. 2013;14:295–8.

    Article  PubMed Central  CAS  PubMed  Google Scholar 

  40. Scheuing N, Thon A, Konrad K, et al. Carbohydrate intake and insulin requirement in children, adolescents and young adults with cystic fibrosis-related diabetes: a multicenter comparison to type 1 diabetes. Clin Nutr. 2015;34:732–8.

    Article  CAS  PubMed  Google Scholar 

  41. Marshall BC, Butler SM, Stoddard M, Moran AM, Liou TG, Morgan WJ. Epidemiology of cystic fibrosis-related diabetes. J Pediatr. 2005;146:681–7.

    Article  CAS  PubMed  Google Scholar 

  42. Rasouli N, Seggelke S, Gibbs J, et al. Cystic fibrosis-related diabetes in adults: inpatient management of 121 patients during 410 admissions. J Diabetes Sci Technol. 2012;6:1038–44.

    Article  PubMed Central  PubMed  Google Scholar 

  43. Metzger BE, Lowe LP, Dyer AR, et al. Hyperglycemia and adverse pregnancy outcomes. N Engl J Med. 2008;358:1991–2002.

    Article  PubMed  Google Scholar 

  44. Michel SH, Mueller DH. Nutrition for pregnant women who have cystic fibrosis. J Acad Nutr Diet. 2012;112:1943–8.

    Article  PubMed  Google Scholar 

  45. Hirsch IB, Janci MM, Goss CH, Aitken ML. Hypoglycemia in adults with cystic fibrosis during oral glucose tolerance testing. Diabetes Care. 2013;36:e121–2.

    Article  PubMed Central  PubMed  Google Scholar 

  46. Radike K, Molz K, Holl RW, Poeter B, Hebestreit H, Ballmann M. Prognostic relevance of hypoglycemia following an oral glucose challenge for cystic fibrosis-related diabetes. Diabetes Care. 2011;34, e43.

    Article  PubMed Central  PubMed  Google Scholar 

  47. Battezzati A, Battezzati PM, Costantini D, Seia M, Zazzeron L, Russo MC, Daccò V, Bertoli S, Crosignani A, Colombo C. Spontaneous hypoglycemia in patients with cystic fibrosis. Eur J Endocrinol. 2007;156:369–76.

    Article  CAS  PubMed  Google Scholar 

  48. Hameed S, Morton JR, Field PI, et al. Once daily insulin detemir in cystic fibrosis with insulin deficiency. Arch Dis Child. 2012;97:464–7.

    Article  PubMed  Google Scholar 

  49. Quick VM, Byrd-Bredbenner C, Neumark-Sztainer D. Chronic illness and disordered eating: a discussion of the literature. Adv Nutr. 2013;4:277–86.

    Article  PubMed Central  PubMed  Google Scholar 

  50. Powers MA, Richter S, Ackard D, Gerken S, Meier M, Criego A. Characteristics of persons with an eating disorder and type 1 diabetes and psychological comparisons with persons with an eating disorder and no diabetes. Int J Eat Disord. 2012;45:252–6.

    Article  PubMed  Google Scholar 

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Author information

Authors and Affiliations

  1. University of Iowa Children’s Hospital, 200 Hawkins Dr., 2861 JPP, Iowa City, IA, 52242-1083, USA

    Katie Larson Ode M.D., M.S.

  2. Diabetes Care Centers, University of Minnesota Health, Fairview, 516 Delaware Street SE, MMC 88, Minneapolis, MN, 55455, USA

    Carol Brunzell R.D., L.D., C.D.E.

Authors
  1. Katie Larson Ode M.D., M.S.
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  2. Carol Brunzell R.D., L.D., C.D.E.
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Correspondence to Katie Larson Ode M.D., M.S. .

Editor information

Editors and Affiliations

  1. Department of Pediatrics, Division of Gastroenterology, Hepatology and Nutrition, University of California, San Francisco, San Francisco, California, USA

    Elizabeth H. Yen

  2. Gastorenterology and Nutrition, The Johns Hopkins Children’s Center, Baltimore, Maryland, USA

    Amanda Radmer Leonard

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Ode, K.L., Brunzell, C. (2015). Nutritional Management of Cystic Fibrosis Related Diabetes Mellitus. In: Yen, E., Leonard, A. (eds) Nutrition in Cystic Fibrosis. Nutrition and Health. Humana Press, Cham. https://doi.org/10.1007/978-3-319-16387-1_13

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  • DOI: https://doi.org/10.1007/978-3-319-16387-1_13

  • Publisher Name: Humana Press, Cham

  • Print ISBN: 978-3-319-16386-4

  • Online ISBN: 978-3-319-16387-1

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