Nutrition In Cystic Fibrosis Research Articles

Collaboration between registered dietitians and gastroenterologists in cystic fibrosis care: Results of an international cross-sectional survey.

Collaboration between registered dietitians and gastroenterologists has not been evaluated in cystic fibrosis (CF). We surveyed registered dietitians and gastroenterologists regarding the current participation of gastroenterologists in CF centers and identified possible areas to enhance partnership between the two disciplines. An anonymous online survey was distributed targeting registered dietitians and gastroenterologists involved in CF care through three international listservs (CF Nutrition, CF DIGEST, and PEDGI) over a 6-week period. SurveyMonkey was used, and informed consent was obtained. A total of 131 respondents participated in this survey, including 80 registered dietitians and 51 gastroenterologists (41 pediatric and 10 adult gastroenterologists). Most respondents (82%) were from the United States, and two-thirds had ≥5 years of experience in CF. A significant number of registered dietitians reported the nonavailability of gastroenterologists for collaboration and there was greater availability of gastroenterologists in pediatric centers. Barriers to interdisciplinary collaboration included lack of CF expertise and dedicated time among the gastroenterologists and difficulties in coordinating the gastroenterology clinics. More gastroenterologists than registered dietitians perceived that they worked collaboratively with the other discipline in various domains (clinical care, quality improvement, research, presentations, and publications). Both disciplines had mutual respect and interest to further the collaboration. There is an increased need for gastroenterologist participation and collaboration (particularly in adult centers) in CF alongside registered dietitians to enhance comprehensive patient care. Future efforts should focus on training more gastroenterologists in CF and facilitating easier access to gastroenterologists for the CF population.

Nutritional status in the era of highly effective CFTR modulators.

Advances in cystic fibrosis (CF) diagnostics and therapeutics have led to improved health and longevity, including increased body weight and decreased malnutrition in people with CF. Highly effective CFTR modulator therapies (HEMT) are associated with increased weight through a variety of mechanisms, accelerating trends of overweight and obesity in the CF population. Higher body mass index (BMI) is associated with improved pulmonary function in CF, yet the incremental improvement at overweight and obese BMIs is not clear. Improvements in pulmonary health with increasing BMI are largely driven by increases in fat-free mass (FFM), and impact of HEMT on FFM is uncertain. While trends toward higher weight and BMI are generally seen as favorable in CF, the increased prevalence of overweight and obesity has raised concern for potential risk of traditional age- and obesity-related comorbidities. Such comorbidities, including impaired glucose tolerance, hypertension, cardiac disease, hyperlipidemia, fatty liver, colon cancer, and obstructive sleep apnea, may occur on top of pre-existing CF-related comorbidities. CF nutrition recommendations are evolving in the post-modulator era to more individualized approaches, in contrast to prior blanket high-fat, high-calorie prescriptions for all. Ultimately, it will be essential to redefine goals for optimal weight and nutritional status to allow for holistic health and aging in people with CF.

Developing a nutrition screening tool for children with cystic fibrosis ages 0 to 2 years: Children with cystic fibrosis nutrition screening tool

BackgroundFor children with cystic fibrosis (CF), achieving and maintaining optimal growth by the age of 2 years is critical for future health outcomes. A standardized nutrition screening is needed to identify growth problems, enable timely interventions, and improve nutritional outcomes for children (0 to 2 years) with CF. The purpose of this study was to develop a nutrition screening tool for children (0 to 2 years) with CF to identify nutrition risk at every clinical encounter. MethodsA retrospective cross-sectional study was used to develop a nutrition screening tool to determine if nutrition interventions needed to change (at-risk) or continue (not at-risk). Retrospective data for pertinent nutrition factors were collected for 99 children attending an accredited CF clinic. The nutrition factors were compared to a dietitian assessment. A stepwise discriminant analysis determined weight-for-age (WFA) and weight-for-length (WFL) z-scores were significant. Then anthropometric data and corresponding dietitian assessment results were collected for children with CF attending two other accredited CF clinics (n = 29, n = 30). Discriminant analysis was used to determine sensitivity and specificity of the nutrition factors and to create a nutrition screening tool equation. ResultsThe nutrition screening model that included WFA z-score, LFA z-score, WFL z-score, and weight change velocity adequacy determined nutrition risk the best. The sensitivity was 89.7 %, specificity 83.2 %, NPV 93.3 %, and PPV 75.4 % for this model. ConclusionThe nutrition screening tool equation developed in this study standardizes the process to identify children (0 to 2 years) with CF at nutrition risk. Further validation is needed.

Evolving nutritional needs, obesity, and overweight status in cystic fibrosis.

The nutritional landscape in cystic fibrosis has shifted dramatically in the era of CFTR modulator therapy. In this review, we will critically examine the literature on overweight and obesity in CF, current nutritional care unknowns and opportunities for further investigation or adaptation in clinical care. Results of clinical trial and real-world data reflect marked improvement in nutritional status and quality of life. Clinical outcomes including CF related diabetes and CF related liver disease appear positively impacted. Secondary impacts on cardiometabolic disease have been noted, especially in association with excessive weight gain. The prior approaches to optimizing nutrition in cystic fibrosis with caloric excess can likely be safely retired for many. As modulator access expands across the lifespan, a longitudinal focus on health maintenance should be considered.

Nutritional considerations for a new era: A CF foundation position paper

ObjectiveTo provide interim advice and considerations to the CF Community around CF nutrition in the current era. MethodsThe Cystic Fibrosis (CF) Foundation organized a multidisciplinary committee to develop a Nutrition Position Paper based on the rapidly changing nutrition landscape in CF, due in part to widespread use of cystic fibrosis transmembrane regulator highly effective modulator therapy (HEMT). Four workgroups were formed: Weight Management, Eating Behavior/Food Insecurity, Salt Homeostasis and Pancreatic Enzyme use. Each workgroup conducted their own focused review of the literature. ResultsThe committee summarized current understanding of issues pertaining to the four workgroup topics and provided 6 key take-aways around CF Nutrition in the new era. ConclusionPeople with CF (pwCF) are living longer, particularly with the advent of HEMT. The traditional high fat, high calorie CF diet may have negative nutritional and cardiovascular consequences as pwCF age. Individuals with CF may have poor diet quality, food insecurity, distorted body image, and an higher incidence of eating disorders. An increase in overweight and obesity may lead to new considerations for nutritional management, given potential effects of overnutrition on pulmonary and cardiometabolic parameters.

Transforming the nutrition care model for infants with cystic fibrosis: A qualitative study of clinicians' perspectives.

Clinician perspectives may inform health service strategies to meet optimal nutrition needs for infants with cystic fibrosis (CF). We conducted a qualitative study with CF-specialized dietitians (registered dietitians [RDs]) and physicians between July to December 2020 to characterize the current state of infant nutrition care delivery and organize input into a conceptual model to inform CF care program strategies. Among 42 participants, 36 completed survey responses and 6 completed interviews; 93% were RDs. Three global themes emerged in the current care model: nutrition management, family centered connections, and collaborative care delivery. Within nutrition management, clinicians emphasized providing education, setting goals, and maintaining adequate follow-up with families. Under family centered connections, clinicians expressed the need to foster relationships with families and link families to resources for assistance to social stressors such as food insecurity. Collaborative care delivery for clinicians interviewed was defined by sharing expertise from across the interdisciplinary team. Based on the timing of this study, clinicians reported compelling examples for various modes of telehealth and home weight monitoring to facilitate and support these domains of nutrition care, including potential advantages for education, supporting family needs, and communication. We integrate these themes to propose a conceptual model to organize complementary in-person and telehealth activities and enhance quality infant CF nutrition care delivery. Future implementation can refine this model through testing of practical telehealth interventions to optimize nutrition outcomes for infants with CF.

Brazilian Guidelines for Nutrition in Cystic Fibrosis.

ABSTRACTObjective To develop a scientific consensus on nutrition in cystic fibrosis.Methods Sixteen coordinators elaborated relevant questions on nutritional therapy in cystic fibrosis, which were divided into six sections: nutritional assessment, nutritional recommendations, nutritional intervention, dietary counseling, special situations and enzyme replacement, and gastrointestinal manifestations. Two to three specialists in the field were responsible for each section and obtaining answers formulated based on standardized bibliographic searches. The available literature was searched in the PubMed®/MEDLINE database, after training and standardization of search strategies, to write the best level of evidence for the questions elaborated. Issues related to disagreement were discussed until a consensus was reached among specialists, based on the current scientific literature.Results Forty-two questions were prepared and objectively answered, resulting in a consensus of nutritional therapy in cystic fibrosis.Conclusion This work enabled establishing a scientific consensus for nutritional treatment of cystic fibrosis patients.

The Changing Landscape of Nutrition in Cystic Fibrosis: The Emergence of Overweight and Obesity.

Cystic fibrosis has historically been characterized by malnutrition, and nutrition strategies have placed emphasis on weight gain due to its association with better pulmonary outcomes. As treatment for this disease has significantly improved, longevity has increased and overweight and obesity have emerged issues in this population. The effect of excess weight and adiposity on CF clinical outcomes is unknown but may produce similar health consequences and obesity-related diseases as those observed in the general population. This review examines the prevalence of overweight and obesity in CF, the medical and psychological impact, as well as the existing evidence for treatment in the general population and how this may be applied to people with CF. Clinicians should partner with individuals with CF and their families to provide a personalized, interdisciplinary approach that includes dietary modification, physical activity, and behavioral intervention. Additional research is needed to identify the optimal strategies for preventing and addressing overweight and obesity in CF.

The Impact of Highly Effective CFTR Modulators on Growth and Nutrition Status.

Patients with cystic fibrosis (CF) are at increased risk of malnutrition and growth failure due to multiple factors as a result of suboptimal or absent function of the CFTR chloride channel protein. Dysfunctional CFTR contributes to increased energy expenditure, exocrine pancreatic insufficiency causing impaired dietary macronutrient digestion and absorption, intestinal dysbiosis, and impaired bile acid homeostasis. Poor nutritional status as a result of these mechanisms is associated with decreased lung function, worse clinical outcomes, and ultimately, increased mortality. Nutritional interventions addressing these mechanisms, such as pancreatic enzyme-replacement therapy and enteral caloric supplementation, have improved nutritional status and, by association, clinical outcomes. In the last decade, the advent of medications targeting defective CFTR proteins has revolutionized the care of patients with CF by reducing the overall impact of CFTR dysfunction. Below, we summarize the effects of highly effective CFTR modulators on nutritional status overall as well as specific factors including bile acid metabolism, pancreatic function, energy expenditure, and intestinal dysbiosis. The future of CF nutrition care will require a paradigm shift away from focusing on methods addressing CFTR dysfunction such as excess calorie provision and toward an individualized, holistic approach in the context of specific mutations and CFTR-directed therapy.

Kistik Fibroziste Mikrobesin Ögelerinin Önemi

Kistik fibrozis, kistik fibrozis transmembran konduktans regulator (KFTR) geninde meydana gelen mutasyonlarin neden oldugu, solunum sistemi basta olmak uzere epitel dokuya sahip hucrelerin tumunu etkileyen klinik olarak ilerleyici akciger hastaligi ile karakterize otozomal ressesif gecisli kalitsal bir hastaliktir. Bireylerin yaklasik olarak %90’inda pankreatik yetersizlik ve bununla birlikte malabsorbsiyon durumu gorulmektedir. Hastaligin patogenezinde yer alan kronik enfeksiyon ve artmis enerji gereksinimi, malabsorbsiyonlar ile birlikte yetersiz beslenme icin risk olusturmaktadir. Malabsorbsiyonlar nedeniyle mikrobesin ogelerinin emiliminde azalma, gereksinimdeki artis, yetersiz alim ve bu durumun sonucu olarak hastaligin kotu prognozu, yasam kalitesinin ve suresinin azalmasina neden olmaktadir. Kistik fibroziste beslenme durumu degisikliklerinin belirlenmesi, buna yonelik erken ve uygun beslenme mudahalelerinin yapilmasi gerekmektedir. Kistik fibrozisli bireylerde yasam boyunca buyume ve beslenme durumunun takip edilmesi onerilir. Pankreatik enzim replasman tedavisi (PERT), hidrasyon ve elektrolit dengesi ile birlikte yasamin erken donemlerinde diyetisyen kontrolunde optimal beslenme durumu saglanmalidir. Kistik fibroziste optimal beslenme, dogru pisirme yontemleri ile birlikte diyet ile mikrobesin ogeleri aliminda etkili olmasina ragmen, bu hastalarda mikrobesin ogeleri yetersizligi onlenemeyebilmektedir. Bu durumda optimal beslenme ile birlikte rehberlerin onerileri goz onunde bulundurularak, bireylerin gereksinimine ve biyokimyasal degerlerine uygun vitamin-mineral destegine gereksinim duyulmaktadir.

Experience of using specialized mixture of enteral nutrition in children with nutritive deficiency in cystic fibrosis

Сystic fibrosis is an autosomal recessive hereditary multisystem disease which is characterized by severe exocrine pancreatic insufficiency, chronic lung disease, and protein-energy malnutrition. Some studies have indicated that the body mass index is an independent factor which adversely affected survival of patients with cystic fibrosis. There is a direct correlation between body mass index, lung function and life expectancy. Objective. To determine the effectiveness and tolerability of the product for children’s dietary nutrition ‘NUTRINIdrink with dietary fibre (neutral taste)’ for children over one year of age for enteral nutrition in cystic fibrosis complicated by protein-energy deficiency.

Prevention of malnutrition in cystic fibrosis

Malnutrition is one of the major burdens of disease in cystic fibrosis. The prevention of malnutrition remains a priority throughout the life of a patient with cystic fibrosis. Literature and guidelines on the management of nutrition in cystic fibrosis have been published; however, here we review updated findings in cystic fibrosis nutrition as well as the role of novel treatments. We review the latest studies on the importance and consequences of nutrition in cystic fibrosis. Novel findings on specific nutrients such as vitamin D and sodium can improve our care and thereby health and growth outcomes. The role of exercise has been further studied. In the field of new treatments, we review the role of cystic fibrosis transmembrane-conductance regulator potentiators and modulators in cystic fibrosis nutrition. A new feeding tube fat-digesting device has been developed and shows promise in cystic fibrosis enteral nutrition. Advances in the nutritional care of cystic fibrosis are forming and believed to further develop in the near future, adding to the recent progress in cystic fibrosis patients' health, survival, and quality of life.

Nutrition in cystic fibrosis: From the past to the present and into the future.

Nutritional management is an integral part of multidisciplinary care for persons with cystic fibrosis. This review will look at how nutrition care has evolved over time. In addition, we will look at how some newer therapies impact nutrition care.

Growth and Nutrition in Cystic Fibrosis.

Optimal nutrition support has been integral in the management of cystic fibrosis (CF) since the disease was initially described. Nutritional status has a clear relationship with disease outcomes, and malnutrition in CF is typically a result of chronic negative energy balance secondary to malabsorption. As the mechanisms underlying the pathology of CF and its implications on nutrient absorption and energy expenditure have been elucidated, nutrition support has become increasingly sophisticated. Comprehensive nutrition monitoring and treatment guidelines from professional and advocacy organizations have unified the approach to nutrition optimization around the world. Newborn screening allows for early nutrition intervention and improvement in short- and long-term growth and other clinical outcomes. The nutrition support goal in CF care includes achieving optimal nutritional status to support growth and pubertal development in children, maintenance of optimal nutritional status in adult life, and optimizing fat soluble vitamin and essential fatty acid status. The mainstay of this approach is a high calorie, high-fat diet, exceeding age, and sex energy intake recommendations for healthy individuals. For patients with exocrine pancreatic insufficiency, enzyme replacement therapy is required to improve fat and calorie absorption. Enzyme dosing varies by age and dietary fat intake. Multiple potential impediments to absorption, including decreased motility, altered gut luminal bile salt and microbiota composition, and enteric inflammation must be considered. Fat soluble vitamin supplementation is required in patients with pancreatic insufficiency. In this report, nutrition support across the age and disease spectrum is discussed, with a focus on the relationships among nutritional status, growth, and disease outcomes.

Late diagnosis and poor nutrition in cystic fibrosis diagnosed before implementation of newborn screening.

Denmark has a high standard cystic fibrosis care. However, newborn screening was not implemented until 2016. This article describes the clinical status of cystic fibrosis patients at time of diagnosis prior to newborn screening. Patients diagnosed with cystic fibrosis in Denmark in 2010-2014 were reviewed using the Danish Cystic Fibrosis Registry as well as patient files. Parameters collected were age at diagnosis, gender, weight, height, forced expiratory volume at 1second, cystic fibrosis transmembrane regulator-genotype, lung bacteriology at diagnosis and previous diagnoses. A total of 63 patients were diagnosed in the study period. The most typical pre-cystic fibrosis diagnoses were asthma and pneumonia. The median age at diagnosis was 1.4years for the pancreatic insufficient and 27.3years for the pancreatic sufficient patients. Of the pancreatic insufficient patients, 21% had moderate to severe malnutrition with BMI below minus 2 SD and 40% had moderate to severe stunting with height below minus 2 SD. Diagnosis was delayed considerably compared to diagnosis by newborn screening in other countries. Many cystic fibrosis patients diagnosed due to clinical symptoms were moderately to severely underweight or stunted at diagnosis.

P336 Pioneering a snack trolley for cystic fibrosis nutrition assessment and education in a paediatric cystic fibrosis clinic

P336 Pioneering a snack trolley for cystic fibrosis nutrition assessment and education in a paediatric cystic fibrosis clinic

P337 Handling cystic fibrosis nutrition during hospitalisation

P337 Handling cystic fibrosis nutrition during hospitalisation

Evaluating the impact of 2006 Australasian Clinical Practice Guidelines for nutrition in children with cystic fibrosis in Australia.

To determine the association between the implementation of the 2006 Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis (CF) and the nutritional status of children participating in the Australian Cystic Fibrosis Data Registry (ACFDR). This research consisted of a quantitative study using ACFDR data and a survey of clinicians and dietitians treating children with CF. Two independent cohorts of children (2-5 years and 6-11 years) were selected from ACFDR between 1998 and 2014 (N = 2304). Generalised estimating equation model was used to assess weight, height and body mass index (BMI) z-scores for each patient before and after the implementation of the nutrition guidelines. A nationwide online survey was sent to 48 clinicians to explore the enablers and barriers to implementation of the guidelines. Data analysis showed significant increase (p < 0.05) in mean weight, height and BMI z-scores ranging from 0.06 to 0.18 after implementation of the guidelines in both cohorts of children. Nineteen (39%) clinicians participated in the survey. The majority of the respondents adopted the recommendations into their practice and used the guidelines as part of their professional development. Structural barriers included a lack of adequate staff resources and clinic space for consultations, inappropriate staff classification, high staff turnover and lack of mentoring support. In children participating in the ACFDR, nutritional status improved after the implementation of the 2006 guidelines. Survey results revealed enablers and barriers to guideline implementation and will inform implementation strategies for the revised Australasian nutrition guidelines for CF, released in 2017.

Cystic Fibrosis Nutrition – Chewing the Fat

Survival data for successive birth cohorts of cystic fibrosis infants born in the twentieth century have shown consistent improvements. More recent UK and US data suggest a plateau in improvements for clinically relevant outcomes. Better treatment of malnutrition has arguably been the most important advance in CF care, but despite this nearly a half of the UK CF population has a sub-optimal BMI. Nutritional decline typically occurs in late childhood and early adult life. Addressing poor adherence and more targeted multi-disciplinary interventions to prevent or reverse this pattern are key to achieving better outcomes for CF patients in the future.

The Prospective Assessment of Nutrition in Children with Cystic Fibrosis

Aims: Patients with Cystic Fibrosis (CF) have increased risk of malnutrition. Early detection of nutritional deterioration enables prompt intervention and correction. The aims of this project were to define the nutritional status of CF patients in Iran and New Zealand, compare and contrast the McDonald Nutritional Risk Screening (NRS) tool with the Australasian Guidelines for Nutrition in Cystic Fibrosis, and validate these results with each patient’s evaluation by their CF clinical team. Methods: Children with CF (2 - 18 years) were assessed during routine outpatient visits over one year. Anthropometric measurements were obtained. Both tools were applied and the results compared to their clinical evaluation (as gold standard) with calculation of specificity and sensitivity. Results: Under-nutrition was seen more frequent in the 33 Iranian children than in the 36 New Zealand (NZ) patients (39% versus 0%, p=0.0001), whereas over-nutrition was more prevalent in NZ children (9% versus 17%, p=0.05). At the first visit, both guidelines were able to recognize 77% and 61% of under-nourished Iranian patients, respectively. The mean sensitivity and specificity for all visits for the McDonald tool were 83% &amp; 73% (Iran) and 65% &amp; 86% (NZ). Sensitivity and specificity for the Australasian guidelines were 79% &amp; 79% (Iran) and 70% &amp; 90% (NZ). Conclusions: Both tools successfully recognised patients at risk of malnutrition. The McDonald tool had comparable sensitivity and specificity to that described previously, especially in Iranian patients. This tool may be helpful in recognizing at risk CF patients, particularlyin developing countries with fewer resources.