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Recommendations Summary

CF: MNT: Suggested Approach and Dietitian Full Time Equivalents (FTEs) 2020

Click here to see the explanation of recommendation ratings (Strong, Fair, Weak, Consensus, Insufficient Evidence) and labels (Imperative or Conditional). To see more detail on the evidence from which the following recommendations were drawn, use the hyperlinks in the Supporting Evidence Section below.

  • Recommendation(s)

    CF: MNT Approach

    RDNs or international equivalents should collaborate with all individuals with cystic fibrosis (CF), their families, and interdisciplinary healthcare teams to co-produce individualized Medical Nutrition Therapy (MNT) based upon the individual’s personal preferences, psychological and psychosocial factors, physiological needs, health status, and pharmacological interventions. MNT for individuals with CF should include comprehensive nutrition assessment and appropriate interventions, including individualized modification of diet, dietary supplements including micronutrient supplements, and pancreatic enzymes, in order to maintain or improve nutrition status and symptoms over time.

    Rating: Fair

    CF: MNT Full Time Equivalents (FTEs)

    It is reasonable for one FTE RDN or international equivalent to provide care for 75-150 individuals with cystic fibrosis (CF). A caseload at the lower end of this range is appropriate for dietitians who work primarily with the pediatric population or adults with advanced disease and/or co-morbidities in order to deliver continuous, high-quality nutrition care that effectively manages nutrition challenges and prevents disease decline.

    Rating: Consensus

    • Risks/Harms of Implementing This Recommendation

      Potential harms (time spent at clinic visits, time and money implementing MNT suggestions, psychological concerns or anxiety regarding food and nutrition) are relatively minimal compared with the potential benefits of improved nutrition status and decreased disease progression.

    • Conditions of Application

      Individualize Approach. MNT approach will vary and should be individualized according to the client with cystic fibrosis (CF) and family. Management of CF requires considerable dedication and may demand that individuals with CF and their families prioritize the most pressing health care challenges at any given time.   

      CF Conditions Necessitating Increased Care: The recommended caseload for CF RDNs should be considered as general guidance, since some individuals with CF may require more intensive nutrition care in order to manage nutrition status and co-morbidities. These populations include: individuals with a new CF diagnosis; pediatric individuals including those transitioning to adult care; those with advanced lung disease or pulmonary exacerbations; those who are pre- or post- lung transplant; those who have CFRD, liver disease, unintentional weight loss or decline in nutrition status; and those on enteral or parenteral nutrition or other significant co-morbidities. Individuals with CF who have lower socioeconomic status may require more RDN effort due to higher risk of nutrition concerns, more barriers with implementing healthy changes in nutrition behaviors and greater need for resources that can address these barriers (ex: food insecurity). The proportion of time dedicated to in-patient vs out-patient care will vary according to RDN and CF facility, with a trade-off between increased continuity of care, but decreased productivity, for dietitians working in both settings. Thus, RDNs working in both in- and out-patient settings should maintain caseloads at the lower end of the range recommended. As triple combination CFTR modulation therapy further changes the landscape of nutrition care, RDNs will have to take an even more individualized approach to nutrition care planning. It is likely that the lower end of the case range may be appropriate for all RDNs in all care settings moving forward.

      Additional RDN Responsibilities. Caseload assignments should also consider the RDNs additional duties, including time spent on clients without CF and contributions to research, including quality improvement (QI) studies. Additionally, while reimbursement coverage for pediatric CF care is more robust, there are considerable gaps in reimbursement with Medicare and certain state Medicaid programs for RDNs who care for adults with CF. Lack of reimbursement may require RDNs to manage additional duties, thus decreasing time available for MNT.

      Socioeconomic Status. Coordinated care provided by the interdisciplinary healthcare team at accredited CF centers is crucial to managing disease and should be encouraged for all individuals with CF. However, individuals living in rural areas or who have low socioeconomic status may have decreased access to insurance or challenges with transportation to medical care, and therefore, MNT appointments. In these cases, RDNs should work with the individual with CF and their families and the interdisciplinary CF healthcare team to provide feasible solutions for the person to receive nutrition care, including telephone or virtual appointments, when necessary.

    • Potential Costs Associated with Application

      Cost of MNT depends on the RDN salary, clinic/office space or overhead and insurance reimbursement, but this cost is much less than the cost of a complications associated with malnutrition in CF, which can include the initiation of enteral feeding, hospitalization or frequent CF pulmonary exacerbations. As described above, insurance reimbursement for CF care may be a challenge in many states, particularly for adults with CF, and this may lead to sub-optimal nutrition care and, consequently, health inequities and disparities. MNT is usually administered along with physician or medical visits, imposing a minimal additional time burden on the individual with CF.

    • Recommendation Narrative

      Background. CF is a life-long, progressive disease, currently without a cure. Maintaining or improving nutrition status is a cornerstone in slowing disease progression, due to the longitudinal, inverse association between weight and growth parameters and improved respiratory function (Academy of Nutrition and Dietetics 2019). RDNs in partnership with individuals and families living with CF and their health care providers must consider treatment needs throughout the lifespan, including risk of chronic diseases in adulthood. The pathology of CF is complex and is impacted heavily by lifestyle and environment (Corey et al 1988, Filigno et al 2017, Powers et al 2015, Stark et al 2009, Wheatley et al 2011, Shakkottai et al 2018). Over 2000 variants of the CF transmembrane conductance regulator (CFTR) gene have been identified, including approximately 350 disease-causing mutation.(Elborn 2016, Cystic Fibrosis Foundation 2011) Specific mutations can result in varying degrees of disease severity and progression. Nutrition status and needs are affected by medications, including but not limited to CFTR modulators, pancreatic enzyme replacement therapy (PERT), appetite stimulants and growth hormones. Individuals with CF have a variety of goals and values and diverse incomes, educations and social environments (Oates et al 2016). These complex conditions and interactions require an adaptable and individualized approach to MNT for individuals with CF.

      Evidence for Pediatrics. There was little evidence describing optimal MNT frequency identified in the systematic review, and there was no evidence examining effects of MNT on outcomes for infants with CF. Only four studies were included that examined the relationship between MNT and nutrition-related outcomes in children and adolescents with CF, and three of these were observational, quality improvement (QI) studies (Ledger et al 2013, Stark et al 2011, Savant et al 2014, Ramirez et al 2015). In these studies, MNT was one aspect of broader interventions, which compared increased or intensified CF care to standard care, either before implementation of a QI project or from reference participant data from the Cystic Fibrosis Foundation Patient Registry (CFFPR). Translation of the literature into guidance for clinical practice was complicated, since baseline or standard care was not described in available studies. Nevertheless, included studies generally demonstrated that increased or intensified MNT, as part of a greater intervention, was associated with improved growth outcomes in children and adolescents with CF (Stark et al 2011, Savant et al 2014, Ramirez et al 2015). In one study, researchers provided weekly, 90-minute counseling sessions which may not be applicable to clinical practice long term (Stark et al 2011). In the remaining studies, participants with CF received monthly-to-quarterly clinic-based assessments from an RDN with interventions as appropriate, which generally resulted in improved BMI percentiles over 2-7 years (Savant et al 2014, Ramirez et al 2015). One study demonstrated no effect of the intervention on growth outcomes, but the intervention did result in decreased in-patient and out-patient IV antibiotic requirements.(Ledger et al 2013).

      While very limited evidence examining the effects of MNT provided by an RDN showed no effect on lung function (Ledger et al 2013, Stark et al 2011, Ramirez et al 2015), the evidence regarding effect on anthropometric measures that was available along with conclusions regarding the importance of weight/growth in preventing pulmonary decline in both children and adults with CF (Academy of Nutrition and Dietetics 2019, Darrah et al 2016, Emerson et al 2002, Goss et al 2018, Keller et al 2003, Konstan et al 2007, Lai et al 2009, Machogu et al 2015, McPhail et al 2008, Sanders et al 2014, Sanders et al 2015, Sanders et al 2015, Sanders et al 2018, Usatin et al 2017, Welsh et al 2014, Woestenenk et al 2014, Yen et al 2013, Amadori et al 2009, Ramos et al 2017, George et al 2011, Chamnan et al 2010, Stephenson et al 2015, Vieni et al 2013, McColley et al 2017) demonstrate the critical role of an adequate level of care delivered by an RDN.

      Components of successful MNT interventions included:

      • specified frequency of nutrition screening and conditions for assessment;
      • individualized energy and supplement prescriptions;
      • twice weekly-to-quarterly monitoring of weight and growth, with specified cut-points for oral nutrition supplementation;
      • individualized nutrition education sessions regarding PERT, energy requirements, vitamin and mineral needs.

      Evidence for Adults: There was no evidence available examining the efficacy of MNT provided by an RDN or international equivalent in adults with CF.

      MNT scope and skills for CF RDNs. The aim of this section is to provide guidance on the overall approach for MNT provided by an RDN or international equivalent for an individual with CF and family. This section does not contain detailed guidance or description of specific assessment methods or interventions, which are addressed in other sections of this guideline or, when outside of the scope of the current guideline, in external guidelines (please see Recommendation Overview Table).

      CF RDNs are trained to provide specialty care for the unique needs of each person with CF as well as the family. RDNs should develop relationships with, and understand the nutrition goals of, individuals with CF and their families in order to deliver patient- and family-centered care that results in the most optimal nutrition-related outcomes for each individual with CF (Smyth et al 2017, Bryon et al 2011). CF RDNs provide expertise on topics including but not limited to malnutrition and nutrient deficiencies, vitamin and mineral (including sodium) supplementation, PERT, oral and enteral nutrition supplementation as well as CF-related diabetes (CFRD) and other comorbid conditions. RDNs provide nutrition education, including self-care, and anticipatory guidance of nutrition therapies into daily life.

      MNT for individuals with CF follows the Nutrition Care Process. CF RDNs screen for nutrition risk; assess and diagnose nutrition status and needs; intervene accordingly; and regularly monitor and evaluate nutrition status in individuals with CF (Swan et al 2017). Nutrition screening and assessment methods should be appropriate for the CF population (see screening and assessment sections). The course of nutrition intervention will be highly dependent on the individuals with CF and their families, including their cultural values and individualized goals. Evidence-based recommendations addressing specific approaches to behavioral modification and nutrition education are addressed in Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand.(Saxby et al 2017) RDNs must work with the entire interdisciplinary CF team and family to facilitate implementation of nutrition prescriptions that:

      • are feasible;
      • foster long-term enjoyment of and a healthy relationship with food;
      • increase intake of essential nutrients;
      • decrease intake of high-energy, low nutrient density foods, especially those that may contribute to impaired glucose or lipid homeostasis;
      • optimize nutrition status while aligning with each individual’s personal health goals

      Anticipative, proactive nutritional management is essential to daily, preventive care and treatment in CF.  Consistent contact between individuals with CF, their families and the RDN is essential for developing rapport and long-term relationships that facilitate addressing nutrition challenges and preventing adverse nutrition-related outcomes throughout the lifespan. The RDN is a critical member of a interdisciplinary team that includes the individual with CF, family, physicians, nurses, respiratory therapists and other healthcare professionals such as pharmacists, psychologists, social workers, research coordinators and/or physical therapists (Cystic Fibrosis Foundation 2019).

      Providing optimal nutrition care for individuals with CF requires advanced level practice skills. CF dietitians should attain experience and expertise in CF and diabetes and ideally will have or obtain specialty certifications in the areas of diabetes, pediatrics (when applicable), enteral and parenteral nutrition and/or MNT. Becoming a proficient CF RDN requires supervised training and practice with experienced CF dietitians, followed by ongoing continuing education in order to stay abreast of current research and dynamic changes to CF care. CF dietitians must be able to interpret markers of nutritional status for the CF population, provide advice tailored to the unique needs of those with CF, work within a specialized interdisciplinary team in the in-patient and out-patient settings, and demonstrate critical and clinical reasoning skills to deliver evidence-based practice reflecting current literature, including for complex cases (Saxby et al 2017, Cystic Fibrosis Trust 2016). The CFF provides resources including educational opportunities and training at national and regional conferences and consortiums, peer mentoring programs, and online resources to help practitioners achieve high level practice skills (Cystic Fibrosis Foundation 2010, Cystic Fibrosis Collaboration 2020).

      International standards for CF RDN effort. The CFF recognizes the need for nutrition care delivered by an RDN and requires a minimum of contact with and documentation from an RDN per patient per year for CFF accreditation of adult or pediatric CF Centers (Cystic Fibrosis Foundation, Yankaskas et al 2004, Cystic Fibrosis Foundation Patient Registry 2017). The CFF additionally specifies one FTE RDN is needed for care of 100-200 individuals with CF (Cystic Fibrosis Foundation 1997). Recommended dietitian staffing in Australia is one FTE dietitian per 75-150 patients, and in New Zealand, one half FTE per 50 patients, if RDNs are providing sole care (Saxby et al 2017). Minimum dietitian staffing requirements in Canada include one FTE for 151-249 individuals with CF (McIntosh 2016). In the U.K., recommendations are for one FTE dietitian per 150 patients (Cystic Fibrosis Trust 2016). Providing appropriate, proactive, individualized nutrition care in the modern age of CF, in which 90% of individuals will be on CFTR modulation therapy, will require increased involvement and care from an RDN. Many individuals with CF will require increased effort from the RDN (see conditions of application). Therefore, based on clinical experience, anticipation of increasing heterogeneity in the CF population and external reviewer feedback, the workgroup elected to corroborate the guidance from Australia and recommend a caseload of 75-150 CF clients per dietitian FTE.

      Regular collaboration with the RDN, as appropriate for the individual with CF, ensures continuity of care and development of relationships within the interdisciplinary CF team, including the individual with CF, their families and support systems, physicians, nurses, respiratory therapists, social workers and pharmacists.

    • Recommendation Strength Rationale

      The recommendations for children and adolescents with CF was based on Grade III evidence from the systematic review examining the relationship between MNT and anthropometric outcomes and antibiotic use. There was no available evidence examining the role of MNT in infants and adults with CF, thus, recommendations were based on expert consensus/opinion. The workgroup drafted consensus recommendations based on clinical expertise, literature outside of the systematic review, and nutrition principles and growth goals for the general population, with specifications that all practice decisions should be individualized according to the client. All consensus recommendations were discussed and approved unanimously by the workgroup. Grades I and II evidence demonstrated the prospective relationship between nutrition status and lung function in infants and adults with CF. The recommendation describing the suggested case load for RDNs is based on standards from the US and other comparable countries as well as expert consensus/opinion.

    • Minority Opinions

      Consensus reached.