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 |
| 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 |
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:
- AGREE Next Steps Consortium. The AGREE II Instrument [Electronic version]. http://www.agreetrust.org. Published 2017. Accessed February 3, 2020.
- 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.
- 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.
- 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.
- 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.
- 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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