Nutrition in Children and Adults Clinical Care Guidelines

Nutritional support is a vital component of care for patients with cystic fibrosis. These recommendations cover caloric intake, dosing for pancreatic enzyme replacement therapy, and monitoring growth in childhood and weight status in patients of all ages. 

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Summary
  • This set of recommendations resulted from the review of evidence in three areas: dietary energy (caloric) intake, dosing for pancreatic enzyme replacement therapy, and monitoring growth in childhood and weight status in patients of all ages.
  • The CF Foundation worked with investigators at Johns Hopkins University to conduct a systematic review to assist in making recommendations.

Nutrition in Children and Adults Clinical Care Guidelines

Stallings VA, Stark LJ, Robinson KA, Feranchak AP, Quinton H, Clinical Practice Guidelines on Growth and Nutrition Subcommittee, Ad Hoc Working Group. 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 May;108(5):832-9.PMID: 18442507

This set of recommendations resulted from the review of evidence in three areas: dietary energy (caloric) intake, dosing for pancreatic enzyme replacement therapy (PERT), and monitoring growth in childhood and weight status in patients of all ages. It was a partial update of the 2002 consensus report recommendations for nutrition for pediatric patients with cystic fibrosis. Increased resting energy expenditure (patients require more dietary calories than healthy peers) is common in patients with cystic fibrosis and often is combined with poor absorption of dietary fat secondary to pancreatic exocrine insufficiency (PEI). This results in increased risk for both macro- and micronutrient deficiencies. Malnutrition in CF has been shown to affect important clinical outcomes including pulmonary function, rate of pulmonary decline, frequency of hospital admission, and quality of life

Due to a paucity of high-quality, prospective studies and clinical trials, most of the 2008 recommendations were based on the results of retrospective studies of the Cystic Fibrosis Foundation Patient Registry, census-based practice guidelines, and historical practice patterns. Since the 2008 guidelines, new evidence shows that optimization of patient nutritional status is a key component of clinical care and should be addressed in the care of all patients. Future work will likely focus on identifying more informative indicators of nutritional status, such as body composition (fat mass, fat distribution, fat-free or lean mass), in addition to weight and stature. Identification of early nutritional biomarkers that predict disease progression or support of individualized nutrition regimes may be required to optimize the use of newer therapies.

Since its initial publication, the 2008 evidence-based nutritional guidelines have served as an invaluable resource to CF dietitians, pulmonologists, gastroenterologists, endocrinologists, and primary care providers. Expanded and successful implementation of nutritional therapy, combined with advances in respiratory care, has led to increased life expectancy and improved quality of life. At its core, these guidelines have provided a framework for non-nutrition experts to provide optimal nutritional care for all patients with CF.

Methodology

The CF Foundation worked with investigators at Johns Hopkins University to conduct a systematic review. This review assisted the subcommittee in making recommendations. They looked at articles published from January 1988 to February 2005 to inform the development of clinical care guidelines and encourage evidence-based practice. The Subcommittee on Growth and Nutrition reviewed the evidence in two areas: energy intake and dosing for PERT. Evidence-based recommendations are presented here. Also, an ad hoc working group conducted a review of the literature and performed new analyses using the CF Patient Registry to update the recommendations for growth and weight-status monitoring. The evidence base for each recommendation was graded using the system developed by the U.S. Preventive Services Task Force. Find additional information about the USPSTF grading definitions.

Recommendation

Recommendation Evaluation of the Evidence
1. For patients older than 2 years, energy intakes of 110–200 percent of requirements for healthy patients of similar age, sex, and size results in improved weight gain. Grade: B
2. Maintenance of a normal weight- and stature-for- age in children, and a normal weight-for-height in adults, was associated with better forced expiratory volume (FEV1) and survival. Grade: B
3. Children and adolescents 2–20 years old should maintain a body mass index (BMI) at or above the 50th percentile. Children younger than 2 years should reach a weight-for-length ≥50th percentile by 2 years. For adults, women should maintain a BMI at or above 22 while men should maintain a BMI at or above 23. These recommendations were based on results showing that maintenance of these growth parameters was associated with higher FEV1 measurements. No grade
4. For children and adults, PERT dosing should be 500–2,500 units lipase per kilogram body weight per meal; or <10,000 units lipase per kilogram body weight per day; or <4,000 units lipase per gram dietary fat.   No grade

Unanswered Questions

Several areas had insufficient evidence to make a recommendation and additional research is needed to:

  • Identify new informative measures of nutritional status (not only BMI) in children and adults
  • Develop methods to optimize PERT effectiveness in individual patients
  • Determine the effect of CF-related diabetes on nutritional outcomes
  • Understand if nutritional requirements and optimal management vary with new drug therapies (e.g., potentiators and correctors)

Further Reading 

Relevant manuscripts published after the original guidelines are listed below. These manuscripts have not been reviewed or endorsed by the guidelines committee.

  1. Schwarzenberg SJ, Hempstead SE, McDonald CM, Powers SW, Wooldridge J, Blair S, Freedman S, Harrington E, Murphy PJ, Palmer L, Schroeder AE, Shiel K, Sullivan J, Wallentine M, Marshall BC, Leonard AR. Enteral Tube Feeding for Individuals With Cystic Fibrosis: Cystic Fibrosis Foundation Evidence-Informed Guidelines. J Cyst Fibros. 2016. Nov;15(6):724-735.PMID: 27599607
  2. Sheikh S, Zemel BS, Stallings VA, Rubenstein RC, Kelly A. Body composition and pulmonary function in cystic fibrosis. Front Pediatr. 2014 Apr 15;2:33.PMID: 24783186
  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):530-535.e1.PMID: 23062247
  4. Solomon M, Bozic M, Mascarenhas MR. Nutritional issues in cystic fibrosis. Clin Chest Med. Mar;37(1):97-107. Epub 2015 Dec 24.PMID: PMID: 26857771
  5. Heltshe SL, Borowitz DS, Leung DH, Ramsey B, Mayer-Hamblett N. Early attained weight and length predict growth faltering better than velocity measures in infants with CF. J Cyst Fibros. 2014 Dec;13(6):723-9. Epub 2014 Jun 7.PMID: 24917114
  6. Alicandro G, Bisogno A, Battezzati A, Bianchi ML, Corti F, Colombo C. Recurrent pulmonary exacerbations are associated with low fat-free mass and low bone mineral density in young adults with cystic fibrosis. J Cyst Fibros. 2014 May;13(3):328-34. Epub 2013 Nov 28.PMID: 24291530
  7. Borowitz D, Gelfond D, Maguiness K, Heubi JE, Ramsey B. Maximal daily dose of pancreatic enzyme replacement therapy in infants with cystic fibrosis: a reconsideration. J Cyst Fibros. 2013 Dec;12(6):784-5. Epub 2013 Jun 26.PMID: 23809508
  8. Fogarty AW, Britton J, Clayton A, Smyth AR. Are measures of body habitus associated with mortality in cystic fibrosis? Chest. 2012;142:712-17.PMID: 22362875
  9. Tangpricha V, Kelly A, Stephenson A, Maguiness K, Enders J, Robinson KA, Marshall BC, Borowitz D, for the Cystic Fibrosis Foundation Vitamin D Evidence-Based Review Committee. 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(4):1082-1093.PMID: 22399505
  10. Stark LJ, Opipari-Arrigan L, Quittner AL, Bean J, Powers SW. The effects of an intensive behavior and nutrition intervention compared to standard of care on weight outcomes in CF. ˆ. 2011 Jan;46(1):31-5. Epub 2010 Sep 1.PMID: 20812240

This executive summary was prepared by:

A. Jay Freeman, MD, MSc (Children's Healthcare of Atlanta/Emory University School of Medicine) and Virginia A. Stallings, MD, MS (Children's Hospital of Philadelphia/Perelman School of Medicine)

The guidelines were published in May 2008, they were reviewed in April 2019 and it was determined that no update is needed at this time.

 

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Maintaining Healthy Weight With Cystic Fibrosis Download PDF
Early Nutrition and Growth for Your Young Child With Cystic Fibrosis Download PDF
Eating Healthy as a Teenager With Cystic Fibrosis Download PDF
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