Nutritional Management Of Cystic Fibrosis Research Articles

WS12.06 The changing landscape of tube feeding in the post modulator era

Introduction: The introduction of elexacftor/tezacaftor/ ivacaftor (ETI) therapy has resulted in a paradigm shift in nutritional management of cystic fibrosis (CF). ETI is associated with increased weight, improved nutritional status and reduced pulmonary exacerbations. As a result, the number of individuals requiring tube feeding has fallen and gastrostomy tubes (G-tubes) are being proactively removed.

Nutritional management of cystic fibrosis and cystic fibrosis‐related diabetes in Australia

AbstractAimThe cystic fibrosis (CF) dietitians' role is to determine individualised nutritional management plans for patients. The aims of the present study were: to (i) document nutritional management delivered by Australian dietitians to patients with CF and CF‐related diabetes (CFRD); (ii) examine if advice is consistent between CF centres within Australia; and (iii) determine if dietetic staffing is adequate for patient numbers.MethodsA 25 question web‐based survey was distributed via professional networks to dietitians working with adult patients with CF within Australia.ResultsSubtle differences in practice were reported. Energy requirements were most commonly determined by the Schofield equation. Various methods were used to calculate protein and fat requirements. Education provided to patients with CFRD was relatively consistent between centres, but notably different from that reported in previous surveys. There were variations in waiting times to see patients newly diagnosed with CFRD, and review time frames for these patients varied significantly. The majority (82%) of CF centres were under staffed when compared with staffing levels outlined in standards of care.ConclusionsThe results of the present study provide insight into the nutritional management delivered by Australian dietitians to patients with CF and CFRD, which is evolving over time. Standardising practice for the delivery of education and addressing inconsistencies between treating centres would assist in optimising care for CF patients. Dietetic staffing is inadequate at the majority of CF centres, which may limit the ability to provide effective management, especially in the context of increasing rates of comorbidites such as CFRD.

Nutritional Management of Cystic Fibrosis

Nutritional Management of Cystic Fibrosis

Nutritional management of cystic fibrosis in Australia and New Zealand

AbstractAim: To document current practice in the nutritional management of cystic fibrosis in Australia and New Zealand and to examine changes in practice since 1996.Methods: Thirty‐four cystic fibrosis services in Australia and New Zealand responded to a survey that examined current nutritional management practices and dietetic staffing levels. The questionnaire was based on a previous survey conducted in 1996.Results: Cystic fibrosis dietetic staffing levels were low. No service met the staffing level recommended by the UK Cystic Fibrosis Trust. Minor practice variations existed in nutrition assessment and monitoring: nutrition support and vitamin supplementation. Changes in the management of pancreatic enzyme replacement therapy and cystic fibrosis‐related diabetes were reported since 1996.Conclusion: This survey highlights that improvements in consistency of nutrition management have been achieved since 1996 and reflect adherence to available clinical guidelines for pancreatic enzyme replacement therapy. While there are many areas of agreement in nutrition care for people with cystic fibrosis around Australia and New Zealand, there are still practice differences, implying that Australian‐ and New Zealand‐specific clinical guidelines are warranted. The implementation of the Australasian Clinical Practice Guidelines for Nutrition in Cystic Fibrosis will be important in standardising and improving cystic fibrosis care.

Nutritional status, perceived body image and eating behaviours in adults with cystic fibrosis

Achieving and maintaining an ideal nutritional status is the primary aim of the nutritional management of cystic fibrosis (CF). It is unclear how nutritional interventions impact on patients' perceptions and behaviours concerning body image and eating. This work aimed to provide a psychosocial profile and compare CF patients receiving (a) enteral tube feeding, (b) nutritional supplements, (c) no nutritional interventions, and (d) healthy controls. A cross-sectional questionnaire design was employed. Age, gender, lung function, and body mass index were recorded. Subjects completed measures of eating attitudes, perceived and desired body shape, body image, self-esteem and quality of life (QoL). A minority of CF patients reported disordered eating. Those receiving nutritional interventions engaged in less dieting behaviour. All CF groups, especially intervention groups, received more pressure from others to eat. For females, control groups desired to be slimmer whereas intervention groups desired to be heavier. Healthy males were content with their body whereas CF males wished to be heavier. Patients receiving enteral tube feeding were less satisfied with their body image, reported lower self-esteem and poorer QoL. Body image and eating behaviours are important considerations of nutritional interventions for maintaining QoL.

Pancreatic enzyme supplements and vitamins in cystic fibrosis

The management of pancreatic insufficiency and the maintenance of satisfactory fat‐soluble vitamin status are major issues in the nutritional management of cystic fibrosis (CF). This article discusses the pancreatic enzyme replacement preparations currently used in the United Kingdom. It also provides an overview of the importance of fat‐soluble vitamins in CF, the supplements currently used, and assesses where future research might be directed. We conclude that dietitians play an important role in helping CF patients to achieve optimal growth and nutritional status, and should therefore be involved in management from diagnosis. Monitoring of pancreatic enzyme replacement therapy (PERT) and vitamin therapy is an integral part of the dietetic assessment, and should be carried out on a regular basis to ensure optimal nutritional management for each individual. Future research needs to concentrate on the efficacy and safety of high lipase enzyme preparations. Research is required to assess the absorption, metabolism and losses of fat‐soluble vitamins amongst today's CF population, and to determine requirements according to age group.

Nutritional management of cystic fibrosis.

Nutritional support is an integral part of the management of cystic fibrosis patients. It is arguably best provided by a qualified dietitian and nutritional care sister working in conjunction with the rest of the cystic fibrosis team. The patient's nutritional needs should be assessed, regularly reviewed, and nutritional treatment tailored to meet the changing clinical and psychosocial needs of the patient. Nutritional intervention is not without complications, and in particular attention to normal feeding behaviour and vigilance when instituting supplementary nutrition may prevent many feeding difficulties.

Nutritional management of cystic fibrosis.

OVERVIEW OF NUTRITIONAL PROBLEMS IN CYSTIC FIBROSIS ....... . PATHOGENESIS OF ENERGY IMBALANCE Energy Losses . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . . .. . . . . . Energy Intake . ... . . . .. . . . .. . . .... . . .. . . . ... . . . .. . . . .. . . Energy Expenditure and Metabolism . . . ... . . ... . ..... . .... . . . . . . PathogeneSis of an Energy Dejicit . . . . . ... . . . . . . . . . .. . .. . . . . ... .

Nutritional Management of Cystic Fibrosis

Nutritional Management of Cystic Fibrosis

Effect of taurine supplementation on fat and energy absorption in cystic fibrosis.

In 10 children with cystic fibrosis and persisting steatorrhoea, supplementation with taurine (30-40 mg/kg/day) was given for two months as an adjunct to the usual pancreatic enzyme treatment. A three day fat and energy balance was performed in patients with cystic fibrosis, before and after the supplementation, and in seven healthy controls who did not receive taurine. Faecal fat was measured by a gravimetric method and stool energy was determined using a bomb calorimeter. Patients with cystic fibrosis, before and after taurine, and healthy controls received the same fat and energy intake (calculated by a dietitian). In patients with cystic fibrosis taurine did not produce any improvement of steatorrhoea (mean (SD) faecal fat 8.7 (3.3) v 11.2 (7.0) g/day, respectively before and after the supplementation), of faecal energy loss (0.978 (0.468) v 1.133 (0.539) MJ/day), of faecal fat expressed as percent of fat intake (13.4 (5.6) v 15.1 (9.8)%), and of faecal energy expressed as percent of energy intake (9.9 (3.6) v 11.2 (5.7)%). Healthy controls had significant lower fat (3.5 (2.3) g/day) and energy 0.576 (0.355) MJ/day faecal losses. In conclusion, taurine failed to decrease significantly fat and energy losses. Our study does not support the use of taurine supplementation in the nutritional management of cystic fibrosis.

Nutritional management of cystic fibrosis.

Cystic fibrosis patients have an increased requirement for calories and probably for all the major nutrients. The newer, enteric-coated granular preparations of pancreatic enzyme are more effective than preceding preparations and should permit a normal fat intake. Recent work has emphasized the interdependence of respiratory disease and nutrition.