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Cystic Fibrosis

CF: Overview of Nutrition Topics in Cystic Fibrosis (2020)

This guideline focused primarily on the topics of Medical Nutrition Therapy provided by an RDN or international equivalent, methods of nutrition screening and assessment, and dietary intake. For topics outside the scope of the current guideline, please refer to recommendations in the external guidelines indicated below. This is not an exhaustive list of high-quality recommendations, but those referenced were from external guidelines assessed as having high quality using the AGREE II tool1 and were approved for use by the Academy’s Council on Research, and specific recommendations referenced were based on systematic review and reviewed, voted on, and approved by consensus by workgroup members.

Table: Recommendation Overview Table of Nutrition Topics for Individuals with Cystic Fibrosis

Topic Guideline Recommended
 Nutrition Screening
 Screening for Nutrition Risk; Frequency  Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Nutrition Assessment
 Frequency of Nutrition Assessment Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Nutrition Assessment Components Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Role of CFTR Modulators in Nutrition Assessment Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Assessment of Energy Expenditure Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
Assessment of Body Composition Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Assessment of Biochemical Values Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Nutrition Intervention: Medical Nutrition Therapy and Nutrition Counseling
 MNT Frequency and Dietitian FTEs Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 MNT Approach Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Behavioral Modification & Nutrition Education  Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 Dietary Intake
 Energy Requirements

Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 

ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016

 General Guidance on Food Intake Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Good Intake with CFRD Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Food Intake with Overweight/Obesity Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Macronutrient Distribution Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Fiber Intake Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Infant Feeding Academy of Nutrition and DIetetics Cystic Fibrosis Evidence-Based Nutrition Practice Guideline, 2019
 Nutritional Supplements: Macronutrients
 Oral Nutrition Supplements Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 Enteral Feeding Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines, 2016.
 Parenteral Nutrition ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Essential Fatty Acid Supplementation Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 Micronutrient Supplements
 Vitamin A ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Vitamin D 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, 2012
 Vitamin E ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Vitamin K Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 Calcium ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Iron Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 Magnesium Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 Selenium ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Sodium* ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Zinc ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Medications
 PERT

ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016

Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 

 Appetite Stimulants & Growth Hormones  Use of appetite stimulants and growth hormones should be considered only on an individual basis, if indicate, and in consultation with the CF physician.
 Colon Cancer Screening  Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendation, 2018
 Co-Morbidities
 Gastro-esophageal Reflux Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 DIOS and Constipation Use guidelines for the general population.
 Bone Health ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Pregnancy

No evidence-based guidelines found.

ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016 (refers to guidance on vitamin supplementation for women who are pregnant throughout the guideline)

 Liver Disease  No evidence-based guidelines found. Individuals with liver disease should receive individualized care and regular follow up and should be referred to a hepatologist.
 CFRD ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children and adults with cystic fibrosis, 2016
 Supplements
 Probiotics  Several recent systematic reviews have been published on probiotic use in individuals with CF and guidelines should be updated based on systematic review findings.
 Glutathione Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand, 2017 
 Coconut Oil  Evidence-based guidance was not available.
 Herbal Supplements  Evidence-based guidance was not available.

*No evidence-based recommendations were found for sodium supplementation. However, due to the importance of this topic, practitioners are referred to ungraded recommendations from the indicated evidence-based practice guideline.

References:

  1. AGREE Next Steps Consortium. The AGREE II Instrument [Electronic version]. http://www.agreetrust.org. Published 2017. Accessed February 3, 2020.
  2. Saxby et al PC, Kench A, King S, Crowder T, van der Haak N and the Australian and New Zealand Cystic Fibrosis Nutrition Guideline Authorship Group. Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand. In. Sydney: Thoracic Society of Australia and New Zealand; 2017.
  3. Turck D, Braegger CP, Colombo C, et al. ESPEN-ESPGHAN-ECFS guidelines on nutrition care for infants, children, and adults with cystic fibrosis. Clin Nutr. 2016;35(3):557-577.
  4. Schwarzenberg SJ, Hempstead SE, McDonald CM, et al. Enteral tube feeding for individuals with cystic fibrosis: Cystic Fibrosis Foundation evidence-informed guidelines. J Cyst Fibrosis. 2016;15(6):724-735.
  5. 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 Metabol. 2012;97(4):1082-1093.
  6. Hadjiliadis D, Khoruts A, Zauber AG, Hempstead SE, Maisonneuve P, Lowenfels AB. Cystic Fibrosis Colorectal Cancer Screening Consensus Recommendations. Gastroenterol. 2018;154(3):736-745.e714.

 

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