Cystic Fibrosis

Cystic Fibrosis

Welcome to the Cystic Fibrosis Project

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 a multi-disciplinary, integrated approach to care. Nutrition care is central to this approach, since poor nutritional status of CF patients is associated with disease decline. Nutritional status is likely to be compromised in CF patients, because CF increases energy needs as well as nutrient malabsorption. Registered Dietitian Nutritionists (RDNs) are on the front lines of counseling patients with CF, their families and their health care teams regarding optimal, individualized, nutrition care to prevent malnutrition. Thus, it is crucial to improve understanding regarding methods by which RDNs can improve nutrition-related outcomes in CF patients, including nutritional status and lung function.
 
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 research of the topic. The results of the scoping review were used to guide the focus of the systematic review, including the development of the research questions.
 
Systematic Review
The aim of this systematic review project 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. The expert workgroup focused on these subtopics:
  • Medical Nutrition Therapy
  • Dietary Intake (macronutrient distribution; fat intake; fiber intake, food groups, dietary patterns and meal frequency; infant feeding; CFRD)
  • CFTR Modulation Therapy
  • Assessment 

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 limited for all other nutrition-related outcomes, including pulmonary function
  • While controlled trials provide the highest-quality evidence, it was clear from the 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. 
  • The workgroup examined if there was evidence supporting the validity and reliability of using specific nutrition parameters for nutrition screening and assessment. Accuracy of the specific nutrition parameters varied according to the CF population examined and the reference standard used. While BMI percentiles were an accurate indicator of nutrition status in pediatric participants with CF, accuracy of body composition measurements compared to a gold standard was variable according to the individuals’ sex, age and body composition. In pediatric participants, weight and growth parameters were longitudinally associated with pulmonary function (FEV1%). In adults, a low BMI was associated with hard outcomes, but there was no evidence examining BMI cut-offs currently recommended by the Cystic Fibrosis Foundation. In all participants with CF, CFRD was longitudinally associated with adverse hard outcomes. However, there was little or no evidence examining the validity, reliability or relationship with hard outcomes for other common nutrition assessment parameters such as nutrition focused physical exam, body composition measures, micronutrient or essential fatty acid levels and deficiencies or lipid profile. 

Guideline
Development of the guideline is still in progress. Estimated publication of the guideline is winter 2019. 

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.
 

  • 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, RD
      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, RD
      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
  • Systematic Review Project Team and Disclosures (2019)

    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


    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


    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


    Financial Contributors

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


    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.