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.
 

 Cystic Fibrosis Nutrition Care
during the Novel Coronavirus Covid-19 Pandemic (pdf)


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. Expand the section below titled Articles and Resources.

9/17/2020

  • Articles and Guideline Resources (2020)
    • 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.
       
    • Academy of Nutrition and Dietetics: 2020 Cystic Fibrosis Evidence Analysis Center Evidence-Based Nutrition Practice Guideline. The Academy of Nutrition and Dietetics Evidence Analysis Center conducted a systematic review of the literature to develop an evidence-based practice guideline for primary nutrition issues in cystic fibrosis (CF). This guideline is designed to complement and build upon existing evidence-based CF nutrition guidelines. The objective of this guideline was to provide recommendations for registered dietitian nutritionists in the United States delivering medical nutrition therapy to individuals with CF and their families that fill gaps in current evidence-based guidelines on topics that are crucial in order to improve health and prevent disease progression. This guideline provides 28 nutrition recommendations to guide medical nutrition therapy, including nutrition screening, nutrition assessment, and dietary intake. For topics outside the scope of this guideline, practitioners are referred to external, evidence-based recommendations. The CF landscape is evolving rapidly with breakthroughs in cystic fibrosis transmembrane regulator modulators changing CF at a cellular level. Medical nutrition therapy for individuals with CF from infancy through advanced age requires novel and individualized approaches. The Academy Evidence Analysis Library CF guidelines provide a framework for expanding upon current knowledge to determine effective nutrition strategies for individuals with CF through long and healthy futures. McDonald CM, Alvarez JA, Bailey J, Bowser EK, Farnham K, Mangus M, Padula L, Porco K, Rozga M. J Acad Nutr Diet. 2020; S2212-2672(20)30302-6. doi: 10.1016/j.jand.2020.03.015 
       
    • Dietary Macronutrient Distribution and Nutrition Outcomes in Persons with Cystic Fibrosis: An Evidence Analysis Center Systematic Review. Cystic fibrosis (CF) increases risk for undernutrition and malabsorption. Individuals with CF traditionally have been counseled to consume a high-fat diet. However, a new era of CF care has increased lifespan and decreased symptoms in many individuals with CF, necessitating a re-examination of the high-fat CF legacy diet. A literature search was conducted of Medline (Ovid), Embase, and CINAHL (EBSCO) databases to identify articles published from January 2002 to May 2018 in the English language examining the relationships between dietary macronutrient distribution and nutrition outcomes in individuals with CF. Articles were screened, risk of bias was assessed, data were synthesized narratively, and each outcome was graded for certainty of evidence. The databases search retrieved 2,519 articles, and 7 cross-sectional articles were included in the final narrative analysis. Three studies examined pediatric participants and 4 examined adults. None of the included studies reported on outcomes of mortality or quality of life. Very low certainty evidence described no apparent relationship between dietary macronutrient distribution and lung function, anthropometric measures, or lipid profile in individuals with CF. The current systematic review demonstrates wide ranges in the dietary macronutrient intakes of individuals with CF with little to no demonstrable relationship between macronutrient distribution and nutrition-related outcomes. No evidence is presented to substantiate an outcomes-related benefit to a higher fat-diet except in the context of achieving higher energy intakes in a lesser volume of food. McDonald CM, Bowser EK, Farnham K, Alvarez JA, Padula L, Rozga M. Dietary Macronutrient Distribution and Nutrition Outcomes in Persons with Cystic Fibrosis: An Evidence Analysis Center Systematic Review. . J Acad Nutr Diet. 2020; S2212-2672(20)30303-8. doi: 10.1016/j.jand.2020.03.016  
       
    • Effect of CFTR Modulators on Anthropometric Parameters in Individuals with Cystic Fibrosis: An Evidence Analysis Center Systematic Review. There is a strong positive association between nutrition status and lung function in cystic fibrosis (CF). Improvements in clinical care have increased longevity for individuals with CF, and it is unknown how cystic fibrosis transmembrane regulator (CFTR) modulation therapy affects nutrition status over time. The objective of this systematic review of the literature was to examine anthropometric (height, weight, and body mass index [BMI; calculated as kg/m2]) and body composition outcomes of CFTR modulation therapy. A literature search of Medline (Ovid), Embase, and CINAHL (EBSCO) databases was conducted for randomized controlled trials examining the effect of CFTR modulation therapy on anthropometric and body composition parameters, published in peer-reviewed journals from January 2002 until May 2018. Articles were screened, data were synthesized qualitatively, and evidence quality was graded by a team of content experts and systematic review methodologists. Significant weight gain with ivacaftor was noted in children and adults with at least 1 copy of G551D mutation. In adults with at least 1 copy of R117H the effect of ivacaftor on BMI was not significant. Effects on BMI were mixed in adults with class II mutations taking ivacaftor with lumacaftor. There was no significant change in BMI in children homozygous for F508del who took ivacaftor with tezacaftor. Elexacaftor-tezacaftor-ivacaftor increased BMI and body weight in individuals 12 years of age and older who were hetero- or homozygous for the F508del mutation. The effect of CFTR modulation therapy on anthropometric parameters depends on the genetic mutation and the type of modulation therapy used. More research is needed to understand the long-term clinical impact of these drugs on nutritional status, including body composition and the role of dietary intake. Bailey J, Rozga M, McDonald CM, Bowser EK, Farnham K, Mangus M, Padula L, Porco K, Alvarez JA. J Acad Nutr Diet. 2020; S2212-2672(20)30301-4. doi: 10.1016/j.jand.2020.03.014    

     

  • 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