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Full Guideline: Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand
Abstract
Optimising growth and nutrition in people with cystic fibrosis (CF) has been shown to positively influence lung function and survival. The ‘2017 Nutrition Guidelines for Cystic Fibrosis in Australia and New Zealand’ (herein referred to as ‘2017 Guidelines’ ) is a planned update of previously published guidelines - The ‘2006 Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis’1. This edition acknowledges and incorporates new topic areas including the nutritional implications of new genetic modulatory therapies, the emergence of overweight and obesity in CF, and complementary nutritional therapies. An overview of dietitian prescribing in New Zealand (NZ) is also provided. This document was developed by a group of Australian and NZ dietitians experienced in CF care, with the assistance of an interdisciplinary clinical expert committee representing various other health professions and CF consumers.
... 53,54 A serum retinol value below a cutoff of 20 mcg/dl represents biochemical vitamin A deficiency. 47 Although obtaining fasting annual laboratory values may be burdensome, nonfasting levels may reflect recent intake of vitamin A. Thus, serum retinol levels are ideally assessed in the fasting state. 36 Vitamin A is a negative acute-phase reactant and therefore can result in falsely low levels when assessed during acute illness. 55 Vitamin A is transported bound to retinol-binding protein (RBP), which is produced in the liver and often low in patients with liver disease and/or malnutrition. ...
... 56 Measurement of serum retinyl esters can be more useful for estimating vitamin A toxicity if >10% of total vitamin A is in the form of retinyl esters. 36,57 Zinc is necessary for synthesis of RBP, and therefore, zinc deficiency may lead to inadequate RBP available to circulate retinol. 58 Vitamin A absorption and conversion of beta-carotene take place in the small intestine; therefore, a history of small bowel resection may lead to low serum levels of vitamin A. CF-specific multivitamins are commonly the first choice for vitamin A supplementation with the content predominantly as beta-carotene, 59 which decreases the risk of toxicity. ...
... Evaluation and interpretation of serum levels 4,36,37 Daily vitamin and mineral recommendations 3 68 Supplemental cholecalciferol has been shown to be superior at improving serum 25(OH)D levels compared with ergocalciferol, 69 and current CFF guidelines recommend treatment with cholecalciferol and offer specific dosing guidelines. 38 Ergocalciferol may be considered for individuals who avoid the use of animal products, although higher doses may be required. ...
Advancements in respiratory and nutrition management have significantly improved the survival of patients with cystic fibrosis (CF). With the availability of several nutrition interventions such as oral/enteral nutrition supplements, enteric‐coated pancreatic enzymes, and water‐miscible CF‐specific vitamin supplements, frank vitamin deficiencies—with the exception of vitamin D—are rarely encountered in current clinical practice. Whereas they were previously considered as micronutrients, our current understanding of fat‐soluble vitamins and minerals as antioxidants, immunomodulators, and disease biomarkers has been evolving. The impact of highly effective modulators on the micronutrient status of patients with CF remains elusive. This narrative review focuses on the updates on the management of fat‐soluble vitamins and other micronutrients in CF in the current era and identifies the gaps in our knowledge.
... The results indicated that not all CPGs follow state-of-the-art standards of development. Among those evaluated, the Thoracic Society of Australia and New Zealand CPGs [40] scored the highest overall quality, while the Paediatric Gastroenterology Society/Dietitians Association of Australia CPGs [41] demonstrated the lowest quality score. The study indicates the need for the development of more robust, unbiased, state-of-the-art CPGs to aid everyday clinical practice. ...
Nutrition is a modifiable factor of paramount importance for the prevention and attainment of health and the development of youngsters [...]
... 24 Prokinetics are sometimes employed as an adjunct but there is a limited evidence base to support their use. 25 The efficacy of treating DIOS with high-dose pancreatic enzyme replacement is unproven. 20 In more severe cases, endoscopic approaches have been described with colonoscopic instillation of Gastrografin, which has been reported to eliminate the need for surgical treatment in over 30%. ...
Cystic fibrosis (CF) is a complex multiorgan disease, which often affects the gastrointestinal tract. With improved CF specific therapies and multidisciplinary management, patients with CF are now living longer with a median life expectancy of around 50 years. This increased life expectancy has resulted in corresponding increase in presentations of the CF patient with comorbid surgical conditions that were never important considerations. Investigations and management of these conditions, such as distal intestinal obstruction syndrome and colorectal cancer warrant good clinical understanding of the unique challenges that CF patients present including chronic immunosuppression, impaired respiratory function and their multi‐organ dysfunction. The purpose of this review is to provide general surgeons with a contemporary update on the CF related surgical issues as they are likely to become increasingly involved in the care of these complex patients and form an integral part of the multidisciplinary team. The improved life expectancy for patients with cystic fibrosis means general surgeons will increasingly be involved in the care of such patients. Herein we provide a narrative, contemporary review on the management of surgical issues in this patient cohort.
The population of people living with a Fontan circulation are highly heterogenous, including both children and adults, who have complex health issues and comorbidities associated with their unique physiology throughout life. Research focused on nutritional considerations and interventions in the Fontan population is extremely limited beyond childhood. This review article discusses the current literature examining nutritional considerations in the setting of Fontan physiology and provides an overview of the available evidence to support nutritional management strategies and future research directions. Protein-losing enteropathy, growth deficits, bone mineral loss, and malabsorption are well-recognised nutritional concerns within this population, but increased adiposity, altered glucose metabolism, and skeletal muscle deficiency are also more recently identified issues. Emergencing evidence suggets that abnormal body composition is associated with poor circulatory function and health outcomes. Many nutrition-related issues, including the impact of congenital heart disease on nutritional status, factors contributing to altered body composition and comorbidities, as well as the role of the microbiome and metabolomics, remain poodly understood.
Introduction
Gastrointestinal (GI) symptoms are common in individuals with Cystic Fibrosis (CF). International research has highlighted that GI care for this group of patients is lacking. Gastroenterology services to CF clinics across Australasia are yet to be examined. This study aimed to describe the current service delivery model and identify areas for improvement that may lead to positive patient outcomes.
Materials and methods
CF clinicians (dietitians, clinical nurse consultants, respiratory consultants), gastroenterologists (GE), and patients or their carers from Australia and New Zealand (NZ) were surveyed online to gather their opinions on CF gastroenterology services provided in their region. Data were analysed using descriptive statistics (frequencies and percentages). Likert scale questions were analysed by grouping responses 1–5 and 6–10, presented alongside the median and interquartile range (IQR). Mann–Whitney U and chi-square tests were used to look at differences between stakeholder groups.
Results
One hundred and fifty-six health professionals and 172 patients or their carers completed the survey. Results showed that the current GI model of care is predominantly a publicly funded service delivered outside of CF clinic time. GE are largely not integrated into the CF team and report a lack of training opportunities. There is a higher level of dissatisfaction with the current service model in NZ than Australia.
Discussion
No stakeholder group deemed the current CF gastroenterology service model as adequate, leaving opportunity for transformations in this field. Ideally this study will invigorate the need for promotion and integration of GI services that would ultimately benefit the whole CF community.
Background:
Treatment advancements have improved life expectancy and nutritional status of people with cystic fibrosis (CF). Alongside reductions in malnutrition, incidences of overweight, obesity and risk factors for diet-related chronic diseases have increased in recent years. This study aimed to synthesise the available literature on diet quality, macronutrient and micronutrient intakes compared to the recommended guidelines in adults with CF, an essential step in deducing the optimal dietary pattern and intakes for CF adults.
Methods:
A systematic search of five electronic databases from inception until April 2023 was conducted using keywords related to CF, diet quality and nutrient intakes.
Results:
Twenty-one studies were included comprising 18 cross-sectional, one cohort and two case control studies, reporting data from 724 adults with CF. Energy and / or macronutrient intake data was reported across 17 cohorts, eight studies provided micronutrients data, and diet quality was determined for four CF cohorts by using a diet quality score, and / or categorising food intake into servings per day for food groups and comparing findings to national dietary guidelines. Although energy intake recommendations were met, and most micronutrient requirements were achieved through supplementation, total energy intake from fat was above recommendations and diet quality was poor.
Conclusion:
This is the first systematic review comprehensively evaluating literature on dietary intakes of adults with CF. Energy-dense, nutrient-poor foods contribute to intakes which pose risk in developing diet-related chronic diseases. Revision of dietary guidelines and practice change in CF nutritional therapy is warranted to optimise nutrition and health outcomes.
Background:
Historically, body mass index (BMI) >50th percentile has represented optimal nutritional status in children with cystic fibrosis (CF) due to its positive association with lung function. Body composition parameters including fat-free mass index (FFMI) have been suggested as a more physiological nutrition benchmark.
Aims:
(1) describe changes in body composition with age and gender; (2) assess the correlation between measures of nutritional status (FFMI-z, FMI-z, BMI-z) and lung function (forced expiratory volume in one second predicted; FEV1pp).
Methods:
This retrospective, mixed cross-sectional and serial measures study consisted of children with CF (8 to 18 years) attending Sydney Children's Hospital (2007-2020). FFMI and fat mass index (FMI) were taken from biennial dual energy x-ray absorptiometry (DXA) scans. Z-scores were derived using Well's reference population [1]. Repeated measures correlation analyses assessed correlations between FFMI-z, FMI-z, and BMI-z with FEV1pp.
Results:
339 DXA reports were analysed from 137 patients. There were slight downwards trends in BMI-z and FMI-z, and an upwards trend in FFMI-z with increasing age and across both genders. Females had higher FMI-z and FFMI-z than males from 12.5 years. There was a weak, positive correlation between FEV1pp and BMI-z (r = 0.14, p = 0.04), and FFMI-z (r = 0.25, p<0.001). FMI-z had no correlation with FEV1pp (r=-0.06, p = 0.41).
Conclusion:
Deficits in FFMI exist despite increasing trends with age. FFMI-z and BMI-z had a weak, positive correlation with FEV1pp. In contemporary cohorts, nutritional status (reflected by surrogate markers such as FFMI and BMI) may be less influential upon lung function than in previous decades. [1]: Wells, J.C., et al. Body-composition reference data for simple and reference techniques and a 4-component model: a new UK reference child. Am. J. Clin. Nutr.96, 1316-1326 (2012).
Cystic fibrosis is a disease that affects quality of life and life span as a result of mutation in the transmembrane conductivity regulator gene. The aim of this study is to determine the nutritional status of 2–18-year-old children with cystic fibrosis.75 children with cystic fibrosis between the ages of 2-18 were included in the study. The data were obtained online with the "Sociodemographic Form", "Nutrition Evaluation Form" and "24-Hour Food Consumption Record". Dietary quality, energy intake, macro and micronutrient intakes of the participants were determined by taking a 24-hour food consumption record. IBM Statistical Package for Social Sciences Version 24 statistical program and BEBIS 8.2 program were used to analyze the data obtained through the questionnaire. In this study 32 girls and 43 boys were included. It was determined that the daily energy intake of the girls was 1368 ± 394 kcal, and the boys were 1496 ± 494 kcal. When the z-score values of the participants are examined, the z-score value according to the height is minimum -4.16, maximum 3.05; It has been determined that the z-score value for weight is minimum -3.79 and maximum 2.60. The mean z-score of the participants for height was -0.25; The mean z score according to weight was determined as -0.40. The average weight of the boys is 30 kg, and the girls are 28.4 kg. More studies are needed to determine nutritional status, new nutritional therapies, and supplemental doses in children with cystic fibrosis.
Objective:
The energy demands of individuals with cystic fibrosis (CF) vary depending on pancreatic function, body composition, lung function, and clinical status. In clinical practice, predictive equations are used to determine energy requirements yet do not reliably account for these factors. Research regarding energy requirements during CF pulmonary exacerbation (CFPEx) and clinical stability is conflicting. The aim of this study was to investigate potential within-individual changes in measured resting energy expenditure (mREE) using indirect calorimetry (IC) at the commencement and completion of intravenous antibiotic treatment (IVABx) for CFPEx and during clinical stability. Secondary aims were to investigate potential differences between predicted resting energy expenditure (pREE) using Schofield equation and correlations between clinical factors with mREE.
Methods:
Body composition using bioimpedance analysis and mREE were conducted at three time points: commencement of IVABx; completion of IVABx; and clinically stable period thereafter.
Results:
Twenty-eight adults with CF completed at least one valid IC measurement. No significant within-person changes in mREE and body composition parameters were observed across time points. The mREE was positively correlated with fat-free mass (kg; r = 0.6; P = 0.008). The mREE was significantly higher than pREE at all time points with the mREE/pREE ratio elevated at time point 1:118% ± 19.5%; time point 2: 112% ± 13.2%; and time point 3: 122 ± 14.3%.
Conclusion:
The mREE remained stable during CFPEx and clinical stability. The pREE underestimated mREE and application of injury factor adjustment of 110% to 130% could potentially account for this discrepancy. The potential role of IC and body composition in individualizing CF nutritional assessment and prescription requires further exploration.
Attainment and maintenance of good nutrition has been an important aspect of management in cystic fibrosis (CF) for decades. In the era of highly effective modulator therapy for CF, the quality of the nutrients we recommend is increasingly important. Our therapy must support our patients' health for many years beyond what we previously thought. Preventing cardiovascular disease, reducing hyperlipidemia, and optimizing lean body mass for active, longer lives now join the long-standing goal of promoting lung function through nutrition. This chapter summarizes recent developments in nutrition in people with CF, with an eye to the evolution of our practice.
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