Cystic Fibrosis

Cystic Fibrosis

Welcome to the Cystic Fibrosis Guideline and Supporting Systematic Review

Cystic Fibrosis (CF) is a genetic disease in which affected individuals produce thick, sticky mucus in multiple organs, including the lungs and pancreas. The complexity of this disease requires an inter-disciplinary, integrated approach to care. Nutrition care is central to this approach, since poor nutritional status in individuals with CF is associated with disease decline. Nutritional status is likely to be compromised in individuals with CF, because CF increases energy needs as well as nutrient malabsorption. Registered Dietitian Nutritionists (RDNs) are on the front lines of counseling  individuals with CF, their families and their healthcare teams regarding optimal, individualized, nutrition care to prevent malnutrition and other nutrition-related concerns. Thus, it is crucial to improve understanding regarding methods by which RDNs can improve nutrition-related outcomes for individuals with CF, including nutritional status and lung function.

Guideline
This guideline is intended for use by the RDN or international equivalent working with individuals with CF. It is intended to address gaps in current evidence-based practice guidelines and to address key questions that have arisen in CF nutrition practice due to developments in CF treatment, particularly CFTR modulation therapy. It is based on the systematic review published here in 2019. Guideline topics addressed are:
  • Medical Nutrition Therapy
    • Approach and RDN FTEs
  • Nutrition Screening and Assessment
  • Dietary Intake
    • General Guidance
    • Dietary Intake with CFRD or Overweight/Obesity
    • Macronutrient Distribution
    • Fiber Intake
    • Infant Feeding
Systematic Review
The aim of the systematic review supporting the evidence-based guideline was to examine the current evidence regarding optimal nutrition screening and assessment methods and the relationships between MNT, dietary intake and nutrition-related outcomes for people with CF.
 
Systematic Review Key Findings: The evidence examining the relationship between MNT delivered by and RDN and nutrition-related outcomes in CF was sparse. Observational evidence described that MNT did improve anthropometric outcomes in pediatric participants with CF when delivered at least quarterly. However, evidence quality was very low for all other nutrition-related outcomes, including pulmonary function. 
 
While controlled trials provide the highest-quality evidence, it was clear from a prior scoping review that evidence regarding the relationships between dietary intake and nutrition-related outcomes was sparse and, thus, observational evidence was also examined in this systematic review. Though the workgroup searched for a wide variety of dietary exposures including specific food groups, dietary patterns and macronutrient distribution, only 17 observational studies met inclusion criteria, and most had a cross-sectional study design. This limited evidence described no clear relationships between dietary intake and nutrition-related outcomes for individuals with CF.
 
Scoping Review

The work on this project started in 2018 by conducting a thorough scoping review designed to examine the extent, range and nature of the available nutrition research in individuals with CF. The results of the scoping review were used to guide the focus of the systematic review, including the development of the research questions.
 

Use the links on the left navigation to access the content. Expand the section below titled Project Team and Disclosures for a listing of individuals who contributed to the development of the project, disclosures and project funding information.

2/5/2020

  • Guideline and Systematic Review Project Team and Disclosures (2020)

    The following individuals contributed their valuable time and expertise to this project:

    Workgroup Members

    • Catherine M. McDonald, PhD, MS, RDN, CSP, Chair
      Clinical Dietitian, Intermountain Healthcare – Primary Children’s Hospital, Salt Lake City, UT, USA
    • Jessica A. Alvarez, PhD, MS, RDN
      Assistant Professor, Emory University School of Medicine, Atlanta, GA, USA
    • Julianna Bailey, MS, RDN, LD
      Clinical Dietitian, University of Alabama at Birmingham, Birmingham, AL, USA
    • Ellen K. Bowser, MS, RDN, LDN, RN, FAND
      Associate in Pediatrics/Faculty Nutritionist, University of Florida Pediatric Pulmonary Division, Gainesville, FL, USA
    • Kristen M. Farnham, MSH, RD, CSP, LDN
      Clinical Pediatric Dietitian, Nemours Children’s Specialty Care, Jacksonville, FL USA
    • Laura Grande Padula, MS, RD, LDN, CSP
      Advanced Practice Dietitian, Children’s Hospital of Philadelphia, Philadelphia, PA, USA

    Patient Advocate Workgroup Members

    • Mark W. Mangus, Sr., BSRC, RPFT, RRT, RCP, FAARC
      Respiratory Care Practitioner, Self-Employed, San Antonio, TX, USA
    • Kathleen Q. Porco, MS, NBC-HWC
      Diabetes Educator, Attain-Health Foundation, Red Lodge, MT, USA

    Project Manager

    • Mary Rozga, PhD, RD
      Nutrition Researcher, Academy of Nutrition and Dietetics, Chicago, IL, USA

    Lead Analyst

    • Erica K. Gradwell, MS, RD (through 10/2018)
      Consultant, Arvada, CO, USA
    • Mary Katherine Hoy, EdD, RD (as of 11/1/2018)
      Nutritionist, ARS-USDA, Beltsville, MD, USA

    Methodologist

    • Deepa Handu, PhD, RD
      Senior Scientist, Academy of Nutrition and Dietetics, Chicago, IL, USA

    Evidence Analysts

    • Lyanne Chin, PhD candidate, RDN
      University of Wisconsin, Madison, WI, USA
    • Megan D. Baumler, PhD, RDN, LD
      DPD Director/Assistant Professor, St. Catherine University, St. Paul, MN, USA
    • Erika M. Carrasquilla, MS, RD, LDN
      Analyst, Huron Consulting Group, Chicago, IL, USA
    • Alanna Clark, MPH
      Public Health Nutritionist (WIC), Mecklenburg County, Carolinas Healthcare Systems, Charlotte, NC, USA
    • Krista Engen, MS, RD
      Clinical Research Coordinator, Rush University Medical Center, Chicago, IL, USA
    • Sharon P. Foley, PhD, RDN
      Assistant Professor, Rush University, Chicago, IL, USA
    • Christiane L. Meireles, PhD, RDN, LD
      Clinical Asstiant Professor, University of Texas Health, San Antonio, TX, USA
    • Jessica West Schueler, MS, RDN
      Clinical Dietitian, St. Charles Health System, Bend OR, USA
    • Jinyu Xu, PhD, MPH, RD
      Research Scientists, Nationwide Children’s Hospital, Columbus, OH USA
    • Amy Yahiro, MS, RD, LDN
      Manager, Evidence-Based Medicine, North American Spine Society, Burr Ridge, IL USA

    Guideline Reviewers

    • Nicole Benesh, RD, LD
      Clinical Dietitian, University of Minnesota Medical Center, Minneapolis, MN, USA
    • Zachari Breeding, MS, RDN, LDN, FAND
      Advanced Clinical Dietitian, Cancer Treatment Centers of America, Philadelphia, PA, USA
    • Candace H. Chemtob, MS, RDN, LD
      Clinical and Research Dietitian, Central Florida Pulmonary Group, Adult Cystic Fibrosis Center, Orlando, FL, USA
    • Rachel Driscoll, MS, RD, CD-N
      Outpatient Dietitian, Bristol Hospital, Hartford, CT, USA
    • Aimee R.  Goyette, RD
      Dietitian, Atlantic Health, Morristown, NJ, USA
    • Shannon N. Gregg, MS, RD, LD
      Senior Clinical Nutritionist, University of New Mexico, Albuquerque, NM, USA
    • Angel M. Jones, MS, RD
      Clinical Dietitian, University of California Los Angeles, Los Angeles, CA, USA
    • Susannah J. King, MSc(Hon), MNutDIet, MPH, PhD, Grad Cert Health Prof Educ, Accredited Practicing Dietitian (Australia)
      Senior Dietitian, Alfred Health, Melbourne, Victoria, Australia
    • Mallory R. Lacey, MS, RD, LDN, CNSC
      Clinical Dietitian, Penn Medicine Lancaster General Hospital, Lancaster, PA, USA
    • Beth Ogata, MS, RD, CSP
      Lecturer, University of Washington, Seattle, WA, USA
    • Sobhana Ranjan, PhD, RD, LD
      Dietitian and Adjunct Faculty, Claxton Dietetic Solutions, LLC & Bluegrass Community and Technical College, Lexington, KY
    • Elizabeth K. Reid, MS, RDN, LDN
      Clinical Dietitian, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
    • Emily H. Russell, MS, RD, LD, CSP, IBCLC
      Senior Dietitian, Baylor Pediatric CF Center, Texas Children’s Hospital, Houston, TX, USA
    • Kayle J. Skorupski, MS, RDN-AP, CSG, CNSC, FANDAssistant Professor of Practice, University of Arizona, Tucson, AZ, USA
    • Valerie E. Tarn, MS, RDN, LD
      Assistant Professor of Pediatrics, Training Director of Pediatric Pulmonary Center, Birmingham, AL, USA
    • Cystic Fibrosis Foundation
      • Maria R. Mascarenhas, MBBS, 
        Section Chief, Nutrition, Division of Gastroenterology, Hepatology and Nutrition, University of Pennsylvania – The Children’s Hospital of Philadelphia, Philadelphia, PA, USA 
      •  Asim Maqbool, MD
        Medical Director, Center for Pancreatic Disorders, Attending Physician, GI, Hepatology and Nutrition, Associate Professor of Clinical Pediatrics, The Children’s Hospital of Philadelphia/University  of Pennsylvania Perelman School of Medicine, Philadelphia, PA, USA
      • Virginia A. Stallings, MD
        Professor of Pediatrics, Director Nutrition Center, Children’s Hospital of Philadelphia, Philadelphia, PA, USA
      • Sarah Hempstead, MS
        Practice Guidelines Manager, Cystic Fibrosis Foundation, Bethesda, MD, USA

    Financial Contributors

    • Academy of Nutrition and Dietetics
    • Medical Nutrition Dietetic Practice Group DPG

    The views or interests of the funding bodies did not influence the development of this systematic review.

    Disclosures of Potential Conflicts of Interest: In the interest of full disclosure, the Academy has adopted the policy of revealing relationships workgroup members have with companies that sell products or services that are relevant to this topic. Workgroup members are required to disclose potential conflicts of interest by completing the Academy Conflict of Interest Form. It should not be assumed that these financial interests will have adverse impact on the content, but they are noted here to fully inform readers:

    • Alvarez, J: received funding as grant principal investigator from the National Institute of Health.
  • Scoping Review Project Team and Disclosures (2018)

    The following individuals contributed their valuable time and expertise to the review:

    Project Manager

    • Mary R. Rozga, PhD, RDN
      Nutrition Researcher, Academy of Nutrition and Dietetics, Chicago, IL, USA

    Methodologist

    • Deepa Handu, PhD, RDN
      Senior Scientist, Academy of Nutrition and Dietetics, Chicago, IL, USA

    Content Advisors

    • Jessica Alvarez, PhD, RDN
      Assistant Professor of Medicine, Division of Endocrinology, Metabolism and Lipids, Department of Medicine, Emory University School of Medicine, Atlanta, GA, USA
    • Ellen K. Bowser, MS, RD, LD, RN, FAND
      Faculty Nutritionist, Pediatric Pulmonary Division, University of Florida, College of Medicine, Gainesville, FL, USA

    Financial Contributors

    • Medical Nutrition Practice Group
    • Academy of Nutrition and Dietetics

    Disclosures of Potential Conflicts of Interest: In the interest of full disclosure, the Academy has adopted the policy of revealing relationships workgroup members and content advisors have with companies that sell products or services that are relevant to this topic. Workgroup members and content advisors are required to disclose potential conflicts of interest by completing the Academy Conflict of Interest Form. It should not be assumed that these financial interests will have adverse impact on the content, but they are noted here to fully inform readers:

    • Alvarez, J: received funding from the National Institute of Health for a grant
  • Guideline Resources

    Cystic FIbrosis (2020) Guideline Presentation - includes all of the recommendations and reatings of the Cystic FIbrosis 2020 Evidence-Based Nutrition Practice Guideline in a PowerPoint presentation format. Idead for in-service presentations, staff training sessions and classess (not designed for patient or client use).  Click to order.