Change in Nutrient and Dietary Intake in European Children with Cystic Fibrosis after a 6-Month Intervention with a Self-Management mHealth Tool

Abstract

Cystic Fibrosis (CF) is a life-long genetic disease, causing increased energy needs and a healthy diet with a specific nutrient distribution. Nutritional status is an indicator of disease prognosis and survival. This study aimed at assessing the effectiveness of a self-management mobile app in supporting patients with CF to achieve the dietary goals set by the CF nutrition guidelines. A clinical trial was conducted in pancreatic insufficient children with CF, followed in six European CF centres, where the self-management app developed within the MyCyFAPP project was used for six months. To assess secondary outcomes, three-day food records were compiled in the app at baseline and after 3 and 6 months of use. Eighty-four subjects (mean 7.8 years old) were enrolled. Compared to baseline, macronutrient distribution better approximated the guidelines, with protein and lipid increasing by 1.0 and 2.1% of the total energy intake, respectively, by the end of the study. Consequently, carbohydrate intake of the total energy intake decreased significantly (−2.9%), along with simple carbohydrate intake (−2.4%). Regarding food groups, a decrease in ultra-processed foods was documented, with a concomitant increase in meat and dairy. The use of a self-management mobile app to self-monitor dietary intake could become a useful tool to achieve adherence to guideline recommendations, if validated during a longer period of time or against a control group.

The Relationship between Body Composition, Dietary Intake, Physical Activity, and Pulmonary Status in Adolescents and Adults with Cystic Fibrosis

“…In the past, this concern for malnutrition has often led to physicians recommending high-calorie diets without concern for diet quality [9]. This has led to a tendency for individuals with CF to overconsume energy-dense, nutrient-poor foods, particularly foods high in added sugars and refined carbohydrates that have a high glycemic index [9][10][11][12].…”

Needs of people with rare diseases that can be supported by electronic resources: a scoping review

“…Synthesised themes from the data extraction led to the identification of 4 domains and 23 subdomains of needs of people with a rare disease or parents of children with a rare disease. A range of unique needs arising from features of individual diseases were identified (eg, risk of life-threatening acute episodes in Addison’s disease in a context of few emergency health professionals being knowledgeable or confident to deal with adrenal crisis23), as well as more global needs that were common across all presentations (eg, the scarcity of high quality, relevant information about individual rare diseases) 19–21 23–51…”

Improvements in body mass index of children with cystic fibrosis following implementation of a standardized nutritional algorithm: A quality improvement project

“…We believe that the improvement was due to both better nutrition and the close monitoring of patients throughout the project. Previous studies also indicate that higher lung function outcomes are associated with better nutrition 22,23 …”

“…Previous studies also indicate that higher lung function outcomes are associated with better nutrition. 22,23 During the project period, Pa and MRSA colonization and hospitalization rates did not change compared to the previous year; this may be due to its relatively short duration. On the other hand, oral antibiotic usage for PEx increased, though this could be attributed to closer monitoring and the more aggressive treatment which may also have contributed to the observed increases in FEV1pp.…”