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Cystic fibrosis: Nutritional issues

Authors
Robert D Baker, MD, PhD
Chris Coburn-Miller, MSRD, CSP
Susan S Baker, MD, PhD
Section Editors
Kathleen J Motil, MD, PhD
George B Mallory, MD
Melvin B Heyman, MD, MPH
Deputy Editor
Alison G Hoppin, MD

INTRODUCTION

Children and adolescents with cystic fibrosis (CF) frequently have growth failure caused by the combination of malabsorption, increased energy needs, and reduced appetite. Nutrient delivery and correction of maldigestion and malabsorption are essential to achieve normal growth to support optimal pulmonary function and to prolong life.

The CF Foundation (CFF) patient registry has documented substantial improvement in life expectancy of patients with CF (figure 1) [1]. To a large degree, the longer life achieved by patients with CF can be ascribed to improved treatment of lung disease, pulmonary toilet, potent and tailored antibiotics, dornase alfa (DNase), and lung transplantation. However, greater emphasis on CF nutrition is considered important to improve longevity and quality of life. As a result, the CFF created a consensus report on practical aspects of nutrition in pediatric CF [2], and a more theoretic report on gastrointestinal outcomes and confounders in CF [3].

The evaluation, monitoring, and treatment of nutritional problems will be addressed here. The diagnosis and management of CF-related pancreatic insufficiency, and screening for CF-related comorbidities that affect nutritional status will also be discussed briefly here and in detail in separate topic reviews:

(See "Cystic fibrosis: Assessment and management of pancreatic insufficiency".)

(See "Cystic fibrosis: Overview of gastrointestinal disease".)

                                   

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Literature review current through: Nov 2016. | This topic last updated: Fri May 27 00:00:00 GMT 2016.
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References
Top
  1. Cystic Fibrosis Foundation Annual Patient Registry Report, 2013. Available from the Cystic Fibrosis Foundation, at: http://www.cff.org/ (Accessed on December 15, 2014).
  2. Borowitz D, Baker RD, Stallings V. Consensus report on nutrition for pediatric patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 2002; 35:246.
  3. Borowitz D, Durie PR, Clarke LL, et al. Gastrointestinal outcomes and confounders in cystic fibrosis. J Pediatr Gastroenterol Nutr 2005; 41:273.
  4. Riordan JR, Rommens JM, Kerem B, et al. Identification of the cystic fibrosis gene: cloning and characterization of complementary DNA. Science 1989; 245:1066.
  5. Zemel BS, Jawad AF, FitzSimmons S, Stallings VA. Longitudinal relationship among growth, nutritional status, and pulmonary function in children with cystic fibrosis: analysis of the Cystic Fibrosis Foundation National CF Patient Registry. J Pediatr 2000; 137:374.
  6. Reilly JJ, Edwards CA, Weaver LT. Malnutrition in children with cystic fibrosis: the energy-balance equation. J Pediatr Gastroenterol Nutr 1997; 25:127.
  7. Stallings VA, Stark LJ, Robinson KA, et al. 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.
  8. Lahiri T, Hempstead SE, Brady C, et al. Clinical Practice Guidelines From the Cystic Fibrosis Foundation for Preschoolers With Cystic Fibrosis. Pediatrics 2016; 137.
  9. Kastner-Cole D, Palmer CN, Ogston SA, et al. Overweight and obesity in deltaF508 homozygous cystic fibrosis. J Pediatr 2005; 147:402.
  10. 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; 162:530.
  11. Cystic Fibrosis Foundation, Borowitz D, Robinson KA, et al. Cystic Fibrosis Foundation evidence-based guidelines for management of infants with cystic fibrosis. J Pediatr 2009; 155:S73.
  12. Grey V, Atkinson S, Drury D, et al. Prevalence of low bone mass and deficiencies of vitamins D and K in pediatric patients with cystic fibrosis from 3 Canadian centers. Pediatrics 2008; 122:1014.
  13. Aris RM, Merkel PA, Bachrach LK, et al. Guide to bone health and disease in cystic fibrosis. J Clin Endocrinol Metab 2005; 90:1888.
  14. Aris RM, Renner JB, Winders AD, et al. Increased rate of fractures and severe kyphosis: sequelae of living into adulthood with cystic fibrosis. Ann Intern Med 1998; 128:186.
  15. Henderson RC, Specter BB. Kyphosis and fractures in children and young adults with cystic fibrosis. J Pediatr 1994; 125:208.
  16. Paccou J, Zeboulon N, Combescure C, et al. The prevalence of osteoporosis, osteopenia, and fractures among adults with cystic fibrosis: a systematic literature review with meta-analysis. Calcif Tissue Int 2010; 86:1.
  17. Bhudhikanok GS, Wang MC, Marcus R, et al. Bone acquisition and loss in children and adults with cystic fibrosis: a longitudinal study. J Pediatr 1998; 133:18.
  18. Simpson DE, Dontu VS, Stephens SE, et al. Large variations occur in bone density measurements of children when using different software. Nucl Med Commun 2005; 26:483.
  19. Conwell LS, Chang AB. Bisphosphonates for osteoporosis in people with cystic fibrosis. Cochrane Database Syst Rev 2014; :CD002010.
  20. Marini JC. Do bisphosphonates make children's bones better or brittle? N Engl J Med 2003; 349:423.
  21. Zemel BS, Kawchak DA, Cnaan A, et al. Prospective evaluation of resting energy expenditure, nutritional status, pulmonary function, and genotype in children with cystic fibrosis. Pediatr Res 1996; 40:578.
  22. Peterson ML, Jacobs DR Jr, Milla CE. Longitudinal changes in growth parameters are correlated with changes in pulmonary function in children with cystic fibrosis. Pediatrics 2003; 112:588.
  23. Steinkamp G, Wiedemann B. Relationship between nutritional status and lung function in cystic fibrosis: cross sectional and longitudinal analyses from the German CF quality assurance (CFQA) project. Thorax 2002; 57:596.
  24. Roy CC, Darling P, Weber AM. A rational approach to meeting macro- and micronutrient needs in cystic fibrosis. J Pediatr Gastroenterol Nutr 1984; 3 Suppl 1:S154.
  25. Magoffin A, Allen JR, McCauley J, et al. Longitudinal analysis of resting energy expenditure in patients with cystic fibrosis. J Pediatr 2008; 152:703.
  26. Moudiou T, Galli-Tsinopoulou A, Nousia-Arvanitakis S. Effect of exocrine pancreatic function on resting energy expenditure in cystic fibrosis. Acta Paediatr 2007; 96:1521.
  27. Johnson MR, Ferkol TW, Shepherd RW. Energy cost of activity and exercise in children and adolescents with cystic fibrosis. J Cyst Fibros 2006; 5:53.
  28. Drumm ML, Konstan MW, Schluchter MD, et al. Genetic modifiers of lung disease in cystic fibrosis. N Engl J Med 2005; 353:1443.
  29. Novitsky S, Segal KR, Chatr-Aryamontri B, et al. Validity of a new portable indirect calorimeter: the AeroSport TEEM 100. Eur J Appl Physiol Occup Physiol 1995; 70:462.
  30. Maqbool A, Stallings VA. Update on fat-soluble vitamins in cystic fibrosis. Curr Opin Pulm Med 2008; 14:574.
  31. Graham-Maar RC, Schall JI, Stettler N, et al. Elevated vitamin A intake and serum retinol in preadolescent children with cystic fibrosis. Am J Clin Nutr 2006; 84:174.
  32. Maqbool A, Graham-Maar RC, Schall JI, et al. Vitamin A intake and elevated serum retinol levels in children and young adults with cystic fibrosis. J Cyst Fibros 2008; 7:137.
  33. Yankaskas JR, Marshall BC, Sufian B, et al. Cystic fibrosis adult care: consensus conference report. Chest 2004; 125:1S.
  34. Tangpricha V, Kelly A, Stephenson A, et al. An update on the screening, diagnosis, management, and treatment of vitamin D deficiency in individuals with cystic fibrosis: evidence-based recommendations from the Cystic Fibrosis Foundation. J Clin Endocrinol Metab 2012; 97:1082.
  35. Holick MF. Vitamin D deficiency. N Engl J Med 2007; 357:266.
  36. Green D, Carson K, Leonard A, et al. Current treatment recommendations for correcting vitamin D deficiency in pediatric patients with cystic fibrosis are inadequate. J Pediatr 2008; 153:554.
  37. Christen S, Woodall AA, Shigenaga MK, et al. gamma-tocopherol traps mutagenic electrophiles such as NO(X) and complements alpha-tocopherol: physiological implications. Proc Natl Acad Sci U S A 1997; 94:3217.
  38. Huang SH, Schall JI, Zemel BS, Stallings VA. Vitamin E status in children with cystic fibrosis and pancreatic insufficiency. J Pediatr 2006; 148:556.
  39. Conway SP, Morton AM, Oldroyd B, et al. Osteoporosis and osteopenia in adults and adolescents with cystic fibrosis: prevalence and associated factors. Thorax 2000; 55:798.
  40. Conway SP, Wolfe SP, Brownlee KG, et al. Vitamin K status among children with cystic fibrosis and its relationship to bone mineral density and bone turnover. Pediatrics 2005; 115:1325.
  41. Nicolaidou P, Stavrinadis I, Loukou I, et al. The effect of vitamin K supplementation on biochemical markers of bone formation in children and adolescents with cystic fibrosis. Eur J Pediatr 2006; 165:540.
  42. Maqbool A, Schall JI, Garcia-Espana JF, et al. Serum linoleic acid status as a clinical indicator of essential fatty acid status in children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2008; 47:635.
  43. Maqbool A, Shall J, Zemel B, et al. Essential fatty acid status and growth and pulmonary function in children with cystic fibrosis. Pediatr Pulmonol 2004; S27:337.
  44. Oliver C, Watson H. Omega-3 fatty acids for cystic fibrosis. Cochrane Database Syst Rev 2016; :CD002201.
  45. KESSLER WR, ANDERSEN DH. Heat prostration in fibrocystic disease of the pancreas and other conditions. Pediatrics 1951; 8:648.
  46. Guimarães EV, Schettino GC, Camargos PA, Penna FJ. Prevalence of hyponatremia at diagnosis and factors associated with the longitudinal variation in serum sodium levels in infants with cystic fibrosis. J Pediatr 2012; 161:285.
  47. Nussbaum E, Boat TF, Wood RE, Doershuk CF. Cystic fibrosis with acute hypoelectrolytemia and metabolic alkalosis in infancy. Am J Dis Child 1979; 133:965.
  48. Singer LT, Nofer JA, Benson-Szekely LJ, Brooks LJ. Behavioral assessment and management of food refusal in children with cystic fibrosis. J Dev Behav Pediatr 1991; 12:115.
  49. Stark LJ, Bowen AM, Tyc VL, et al. A behavioral approach to increasing calorie consumption in children with cystic fibrosis. J Pediatr Psychol 1990; 15:309.
  50. Stark LJ, Knapp LG, Bowen AM, et al. Increasing calorie consumption in children with cystic fibrosis: replication with 2-year follow-up. J Appl Behav Anal 1993; 26:435.
  51. Stark LJ, Jelalian E, Mulvihill MM, et al. Eating in preschool children with cystic fibrosis and healthy peers: behavioral analysis. Pediatrics 1995; 95:210.
  52. Jelalian E, Stark LJ, Reynolds L, Seifer R. Nutrition intervention for weight gain in cystic fibrosis: a meta analysis. J Pediatr 1998; 132:486.
  53. Powers SW, Jones JS, Ferguson KS, et al. Randomized clinical trial of behavioral and nutrition treatment to improve energy intake and growth in toddlers and preschoolers with cystic fibrosis. Pediatrics 2005; 116:1442.
  54. Rowland M, Broderick A, Bourke B. Behavioral and nutritional treatment to improve energy intake and growth in toddlers and preschool-aged children with cystic fibrosis. Pediatrics 2006; 118:432; author reply 432.
  55. Details of the intervention are available at www.oup.com/us/pediatricpsych (Accessed on October 30, 2009).
  56. Stark LJ, Opipari-Arrigan L, Quittner AL, et al. The effects of an intensive behavior and nutrition intervention compared to standard of care on weight outcomes in CF. Pediatr Pulmonol 2011; 46:31.
  57. Kalnins D, Corey M, Ellis L, et al. Failure of conventional strategies to improve nutritional status in malnourished adolescents and adults with cystic fibrosis. J Pediatr 2005; 147:399.
  58. Smyth RL, Rayner O. Oral calorie supplements for cystic fibrosis. Cochrane Database Syst Rev 2014; :CD000406.
  59. Schibler A, von der Heiden R, Birrer P, Mullis PE. Prospective randomised treatment with recombinant human growth hormone in cystic fibrosis. Arch Dis Child 2003; 88:1078.
  60. Stalvey MS, Anbar RD, Konstan MW, et al. A multi-center controlled trial of growth hormone treatment in children with cystic fibrosis. Pediatr Pulmonol 2012; 47:252.
  61. Thaker V, Haagensen AL, Carter B, et al. Recombinant growth hormone therapy for cystic fibrosis in children and young adults. Cochrane Database Syst Rev 2015; :CD008901.
  62. Chinuck R, Dewar J, Baldwin DR, Hendron E. Appetite stimulants for people with cystic fibrosis. Cochrane Database Syst Rev 2014; :CD008190.
  63. Marchand V, Baker SS, Stark TJ, Baker RD. Randomized, double-blind, placebo-controlled pilot trial of megestrol acetate in malnourished children with cystic fibrosis. J Pediatr Gastroenterol Nutr 2000; 31:264.
  64. Eubanks V, Koppersmith N, Wooldridge N, et al. Effects of megestrol acetate on weight gain, body composition, and pulmonary function in patients with cystic fibrosis. J Pediatr 2002; 140:439.
  65. Homnick DN, Homnick BD, Reeves AJ, et al. Cyproheptadine is an effective appetite stimulant in cystic fibrosis. Pediatr Pulmonol 2004; 38:129.
  66. Walker SA, Gozal D. Pulmonary function correlates in the prediction of long-term weight gain in cystic fibrosis patients with gastrostomy tube feedings. J Pediatr Gastroenterol Nutr 1998; 27:53.
  67. Williams SG, Ashworth F, McAlweenie A, et al. Percutaneous endoscopic gastrostomy feeding in patients with cystic fibrosis. Gut 1999; 44:87.
  68. Akobeng AK, Miller V, Thomas A. Percutaneous endoscopic gastrostomy feeding improves nutritional status and stabilizes pulmonary function in patients with cystic fibrosis. J Pediatr Gastroenterol Nutr 1999; 29:485.
  69. Steinkamp G, Rodeck B, Seidenberg J, et al. [Stabilization of lung function in cystic fibrosis during long-term tube feeding via a percutaneous endoscopic gastrostomy]. Pneumologie 1990; 44:1151.
  70. Morton A, Wolfe S. Enteral tube feeding for cystic fibrosis. Cochrane Database Syst Rev 2015; :CD001198.
  71. Levy LD, Durie PR, Pencharz PB, Corey ML. Effects of long-term nutritional rehabilitation on body composition and clinical status in malnourished children and adolescents with cystic fibrosis. J Pediatr 1985; 107:225.
  72. Erskine JM, Lingard CD, Sontag MK, Accurso FJ. Enteral nutrition for patients with cystic fibrosis: comparison of a semi-elemental and nonelemental formula. J Pediatr 1998; 132:265.
  73. Munck A, Malbezin S, Bloch J, et al. Follow-up of 452 totally implantable vascular devices in cystic fibrosis patients. Eur Respir J 2004; 23:430.