Development, implementation and evaluation of a nutrition education and behaviour program for children with cystc fibrosis.

Abstract

Background: Cystic fibrosis (CF) is a genetically inherited disease which adversely affects the respiratory and gastrointestinal systems. Malnutrition is a major clinical problem in individuals with the disease. Nutritional interventions are warranted as improvements in nutritional status could improve the rates of morbidity and mortality associated with the disease. The review of the literature indicated the need to develop a behavioural-based nutrition prevention program in order for children to achieve CF dietary requirements and appropriate pancreatic enzyme replacement therapy.Methods: The intervention program, Go and Grow with CF, and nutrition and pancreatic enzyme knowledge and self-management questionnaires were developed for children with CF and their carers as part of this thesis. Social learning theory constructs which particularly assist children in achieving desirable behaviours were applied during the development of the Go and Grow with CF program. The program consisted of workshops and a home-based course.Fifty eight children with cystic fibrosis, aged 2 to 11 years, and their carers participated in a clinical trial that was designed to assess the effects of the Go and Grow with CF pilot program on knowledge, self-management, behaviour, dietary intake and body composition, using anthropometry. Process evaluation was conducted on the pilot program and on the clinic-wide implementation of the revised Go and Grow with CF program. The revised program included the Australian Pancreatic Enzyme Replacement Therapy Guidelines and the effects of fat-based dosing were assessed with a cohort of 29 children with CF-related pancreatic insufficiency aged 1 to 13 years.Results: Similar to the process evaluation of the pilot program, 100% of carers who completed the revised home-based course indicated that they would recommend Go and Grow with CF to other families with a child who has CF. The 'objective assessment of knowledge indicated a significant m improvement in' children's knowledge in the short-term. There were no statistically significant improvements in any of the other parameters assessed. The lack of significant improvements in self-management, behaviour, dietary intake and anthropometry may have been because the program had no effect, the parameters assessed or the instruments used (particularly the questionnaires) were not sufficiently sensitive, the sample size (which was determined by the CF population available) was too small or the duration of the intervention and follow-up was too short.Conclusion: Carers' unanimous recommendation of Go and Grow with CF, together with high levels of perceived learning, reported increase in confidence and improvement in children's knowledge in the short-term, indicate the benefits of the program.Although there was no statistically significant improvement in the anthropometric measurements after the intervention, 'the extensive data obtained during this study suggest that measurements of height and weight may underestimate the presence of poor nutritional status. It is likely that comprehensive assessments of body composition of children with CF would be useful in detecting mild degrees of malnutrition and in providing information about the effects of nutritional status on morbidity and mortality associated with the disease.Fat-based pancreatic enzyme replacement therapy dosing warrants further investigation given that parents had a strong preference for this method and that fat absorption remains abnormal in the majority of individuals who have pancreatic insufficiency. Evaluation of all pancreatic enzyme replacement therapy dosing methods are needed and this research suggests that dose should be assessed on a meal and snack basis, rather than just on daily intake, in order for levels of adherence to be examined.The apparent absence of a long-term effect of a single exposure to the program on knowledge suggests that regular, ongoing education and counselling is required by families to reinforce aspects related to the child's current stage of development and disease status.