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Cystic Fibrosis

CF: Scope of Guideline (2020)

Guideline Scope Characteristics

Disease/Condition(s)

The purpose of this guideline is to add to current nutrition guidelines for individuals with cystic fibrosis (CF), by focusing on gaps in the evidence-based literature that are of particular importance to how Medical Nutrition Therapy (MNT) is provided by registered dietitian nutritionists (RDNs) working with individuals with CF. Another aim for this guideline is to address topics that may be confusing or controversial to RDNs working with individuals with CF, due to recent improvements in CF treatments, namely CFTR modulation therapy, and changing trends in nutrition status and lifespan. 

Recent evidence-based nutrition guidelines for individuals with CF do not include guidance on frequency of MNT or give recommendations for staffing of RDNs working in the United States. Evidence scoping revealed an absence of evidence-based recommendations describing valid and reliable nutrition screening and assessment methods to guide nutrition diagnosis and, consequently intervention for individuals with CF. Therefore, in this guideline, the authors sought to ask diagnostic and etiologic systematic review questions in order to provide evidence-based recommendations to guide nutrition assessment. Findings from the systematic review, along with clinical and research expertise and standard practice guidelines, primarily from the Cystic Fibrosis Foundation, were used to suggest a schedule for screening and assessment, or monitoring and evaluation once the individual has entered continuous care. Recent, high-quality nutrition guidelines for individuals with CF have provided recommendations on a wide range of intervention topics, including but not limited to: behavioral modification, fat-soluble vitamin supplementation and oral and enteral nutrition supplementation. However, due to the lack of literature available, there were very little evidence-based literature regarding food intake for individuals with CF. While managing nutrition status has long been a crucial tenant of CF care, the importance of diet quality has become increasingly significant, due to the increasing lifespans for individuals with CF and the potential detrimental effects of a low diet quality over time. 

These recommendations are meant to serve as a baseline for MNT approach when working with individuals with CF in the United States. However, due to the extreme heterogeneity of individuals who have CF as well as CF pathologies, all MNT must be individualized in order to be effective. The interventions in this guideline primarily focus on food intake. However, some forms of supplementation will be needed for many individuals with CF, such as fat-soluble vitamins for those who are pancreatic insufficient. This focus also does not ignore the expertise of RDNs in different areas of CF nutrition care, such as with pancreatic enzyme replacement therapy (PERT). In an effort to provide a comprehensive guideline, the team has provided a “Recommendation Overview Table” (please see appropriate tab), which is a fairly comprehensive list of nutrition topics for CF care, and lists where a practitioner can find evidence-based recommendations not covered in the current guideline. These recommendations have been reviewed by all workgroup members, guideline quality was assessed with the AGREE II tool, and guidelines were approved by the Academy’s Council on Research. 

Guideline Category

Assessment of Therapeutic Effectiveness, Counseling, Evaluation, Management, Treatment

Clinical Specialty

Nutrition, Cystic Fibrosis

Intended Users

This guideline is primarily intended for RDNs working with individuals with CF and their families in the United States.

Guideline Objective(s)

Overall Objective

To provide nutrition counseling and care recommendations for RDNs working with individuals with CF in the United States that fill gaps in current evidence-based guidelines on topics that are crucial for delivering Medical Nutrition Therapy to individuals with CF and their families that improves health and prevents disease progression.

Specific Objectives

  • To provide evidence-based, specific, action statements regarding how to screen for nutrition risk and assess nutrition status in individuals with CF; 
  • To provide RDNs with an approach to MNT that is based on evidence, clinical expertise and goals and values of those with CF and their families;
  • To guide dietary recommendations (food intake) for individuals with CF that allows for an individualized approach, accounts for current changes in treatment trends, and considers long-term health;
  • To describe how MNT should address changes in the CF population, including more individuals being treated with CFTR modulators and more individuals who may be categorized as overweight or obese. 

Target Population

Infants and toddlers (0-2 year), Pre-school children (2-5 years), children (6 to 12 years), Adolescent (13 to 18 years), Adult (19 to 44 years), Middle Age (45 to 64 years), Aged (65 to 79 years), Male, Female.

Target Population Description

All individuals with cystic fibrosis, pediatric and adult, living in the United States.

Interventions and Practices Considered

This guideline includes recommendations addressing all stages of the nutrition care process (Assessment, Diagnosis, Intervention, Monitoring and Evaluation), and also includes nutrition screening recommendations. Though monitoring and evaluation recommendations are not repeated separately, screening and assessment recommendations suggest a schedule of monitoring nutrition parameters for individuals with CF, along with indications as to when the individual should be re-assessed, or evaluated. To learn more about the Nutrition Care Process, visit www.ncpro.org 

 

 

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