Nutritional Considerations for a New Era: A CF Foundation Position Paper

The Cystic Fibrosis Foundation organized a multidisciplinary committee to develop a nutrition position paper to provide interim advice and considerations to the CF community around CF nutrition based on the rapidly changing nutrition landscape in CF.

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Summary
  • The introduction of highly effective CFTR modulator therapy (HEMT) has led to improved nutrition among people with CF and has been associated with increasing rates of overweight and obesity.
  • As a result, nutrition-focused care for people with CF has undergone significant changes with new emphasis on overweight and obesity.
  • The Cystic Fibrosis Foundation convened a workgroup to provide nutrition guidance to the CF community in a rapidly changing landscape. 

Nutritional Considerations for a New Era: A CF Foundation Position Paper: Executive Summary

Leonard A, Bailey J, Bruce, A, et al. Nutritional considerations for a new era: A CF foundation position paper. J Cyst Fibros. 2023 Sep;22(5):788-795.  doi: 10.1016/j.jcf.2023.05.010. Epub 2023 May 23.

Purpose and Background 

A high-fat, high-calorie diet has historically been recommended for people with cystic fibrosis. This recommendation was based on significant calorie loss caused by pancreatic insufficiency and increased caloric expenditure related to chronic lung disease. The introduction of highly effective CFTR modulator therapy (HEMT) has led to improved nutrition among people with CF and has been associated with increasing rates of overweight and obesity. As a result, nutrition-focused care for people with CF has undergone significant changes with new emphasis on overweight and obesity. The Cystic Fibrosis Foundation convened a workgroup to provide nutrition guidance to the CF community in a rapidly changing landscape. 

Methodology 

A multidisciplinary workgroup, including adult and pediatric physicians, dietitians, a psychologist, a social worker, people with CF, and representatives from the CF Foundation was formed. Surveys were distributed by email to determine topics important to people with CF, their families, and healthcare professionals. Four subgroups were formed and provided a focused review of the literature on six topics: 

  • Weight management
  • Obesity comorbidities
  • Medical/surgical management of obesity
  • Eating behavior and food insecurity
  • Salt homeostasis/hypertension
  • Pancreatic enzyme use
Key Point Why/What Has Changed?

1. For people with CF taking HEMT, the legacy high-fat, high-calorie diet may have a negative impact on cardiovascular health and weight gain on this population as it ages.

People with CF who are taking HEMT are living longer and may be at risk for comorbidities related to the traditional CF diet. Intake of calorie dense (energy dense) foods for weight gain is no longer a priority for some people with CF as the CF community recognizes the importance of nutritionally rich foods for long-term health. This translates to following an age-appropriate healthy diet rich in fruits, vegetables, whole grains, and legumes, and adjusting caloric intake to meet the individual needs of people with CF.

Lipid screening should follow guidelines for the general population until additional CF-specific data become available. 

2. Emerging data indicate increased prevalence of overweight/obesity in people with CF worldwide, with up to 40% of people with CF overweight or obese in 2021.  Overweight/obesity among people with CF may contribute to cardiovascular disease and other comorbidities (insulin resistance, sleep apnea, and hypertension) as people with CF live longer. HEMT may impact weight by several mechanisms and dietary intake may need to be adjusted.
3. Food insecurity remains a concern for people with CF from all socioeconomic sectors, and screening should be part of standard CF care. In 2019, up to 33% of people with CF in the U.S. were impacted by food insecurity, triple the national average. Screening and interventions can help identify and address food insecurity in the CF community. 
4. It is important for CF clinicians to feel comfortable with identifying, discussing, and referring people with CF for concerns related to eating attitudes and behavior. Previously, body image concerns among people with CF primarily involved low body weight, but recently began to include weight gain and larger body size.
5. Weight-neutral approaches place the focus on improving other health outcomes versus promoting weight loss to treat overweight or obesity. Weight stigma has been found to have a negative impact on overall health outcomes. Weight-neutral approaches can provide sustainable improvement in cholesterol levels, waist to hip ratios, physical activity, diet quality, and self-esteem while also leading to weight loss and a reduction in body mass index. 
6. Measurement of fecal pancreatic elastase after HEMT initiation among children up to age 5 may be helpful if a change in pancreatic status is clinically suspected.  New data suggest HEMT may improve or reverse exocrine pancreatic insufficiency among some children treated with pancreatic enzyme replacement therapy, leading to improvement or normalization of fecal pancreatic elastase. This phenomenon has not been common among adults with CF. 
7. The use of an inline enzyme cartridge with immobilized lipase for enteral feedings has been shown to be safe and effective for digesting nutrients and promoting weight gain. These data have become available since publication of the CF Foundation enteral feeding guidelines in 2016.
8. Salt intake: The recommendation for high salt intake needs to be revisited in adults taking HEMT given the reduced loss of sodium and chloride in the sweat of people with CF taking HEMT. With improved life expectancy, increasing prevalence of overweight and effect of HEMT on salt homeostasis, hypertension may be increasing in people with CF. Blood pressure should be monitored, and previous recommendations regarding salt intake should likely be modified for people with CF taking HEMT. 

Unanswered Questions 

  • The validity and reliability of CF-specific screening tools for eating attitudes and behaviors that are evidence-based and easy to implement are needed and the impact that HEMT, gender, race, and ethnicity have on body image in people with CF remains unclear.
  • The impact of CFTR modulators on pancreatic insufficiency, including evaluation for recovery and maintenance of pancreatic exocrine function, and the effect of HEMT on nutrition/vitamin absorption are unknown. Standards and/or algorithms, ideally evidence-based, for adjusting pancreatic enzyme replacement therapy among people with CF receiving CFTR modulator therapy, would be of benefit in nutritional care of people with CF.
  • Additional study is needed to better define the adverse cardiometabolic effects of obesity, optimal diet makeup, vitamin monitoring/supplementation, pancreatic enzyme replacement therapy, sodium needs, and medical/surgical treatment of overweight and obesity in the post-HEMT era.

Further Reading 

  • Snowball JE, Flight WG, Heath L, Koutoukidis DA. A paradigm shift in cystic fibrosis nutritional care: clinicians views on the management of patients with overweight and obesity. J Cystic Fibrosis. 2023 Sep; 22 (5): 836-842. PMID 36966039.
  • Flatt AJ, Sheikh S, Peleckis AJ, et al. Preservation of beta cell function in pancreatic insufficient cystic fibrosis with highly effective CFTR modulator therapy. J Clin Endocrinol Metab. 2023 Dec 21; 109 (1): 151-160. PMID: 37503734
  • Schwarzenberg S, Vu P, Skalland M, et al. Promise study group. Elexacaftor/tezacaftor/ivacaftor and gastrointestinal outcomes in cystic fibrosis: Report of Promise-GI. J Cyst Fibros. 2023 Mar;22(2):282-289. PMID: 36280527

Use of This Summary 

The CF Foundation intends for this executive summary of its position paper to summarize the published position paper on nutritional consideration. Care decisions regarding individual patients should be made using a combination of this guidance, the associated benefit-risk assessment of treatment options from the clinical team, the patient’s individual and unique circumstances, as well as the goals and preferences of the patients and families that the team serves, as a part of shared decision making between the patient and clinician.

This executive summary was prepared by:

Emily DiMango, MD, (Columbia University) and Clarissa Morency, RD, (Baylor Medicine)

The position paper was published in 2023. It will be reviewed in 2025 and updated at that time, as appropriate.

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