No abstract

IntroductionFor the optimal management of children with cystic fibrosis, there are currently no efficient tools for the precise adjustment of pancreatic enzyme replacement therapy, either for advice on appropriate dietary intake or for achieving an optimal nutrition status. Therefore, we aim to develop a mobile application that ensures a successful nutritional therapy in children with cystic fibrosis.Methods and analysisA multidisciplinary team of 12 partners coordinate their efforts in 9 work packages that cover the entire so-called ‘from laboratory to market’ approach by means of an original and innovative co-design process. A cohort of 200 patients with cystic fibrosis aged 1–17 years are enrolled. We will develop an innovative, clinically tested mobile health application for patients and health professionals involved in cystic fibrosis management. The mobile application integrates the research knowledge and innovative tools for maximising self-management with the aim of leading to a better nutritional status, quality of life and disease prognosis. Bringing together different and complementary areas of knowledge is fundamental for tackling complex challenges in disease treatment, such as optimal nutrition and pancreatic enzyme replacement therapy in cystic fibrosis. Patients are expected to benefit the most from the outcomes of this innovative project.Ethics and disseminationThe project is approved by the Ethics Committee of the coordinating organisation, Hospital Universitari La Fe (Ref: 2014/0484). Scientific findings will be disseminated via journals and conferences addressed to clinicians, food scientists, information and communications technology experts and patients. The specific dissemination working group within the project will address the wide audience communication through the website (http://www.mycyfapp.eu), the social networks and the newsletter.

Background Despite a large number of clinical trials aiming at evaluating the digital self-management of chronic diseases, there is little discussion about users’ experiences with digital approaches. However, a good user experience is a critical factor for technology adoption. Understanding users’ experiences can inform the design of approaches toward increased motivation for digital self-management. Objective This study aimed to evaluate the self-management of cystic fibrosis (CF) with a focus on gastrointestinal concerns and the care of young patients. Following a user-centered design approach, we developed a self-management app for patients and parents and a web tool for health care professionals (HCPs). To evaluate the proposed solutions, a 6-month clinical trial was conducted in 6 European CF competence centers. This paper analyzes the user acceptance of the technology and the benefits and disadvantages perceived by the trial participants. Methods A mixed methods approach was applied. Data were collected through 41 semistructured qualitative interviews of patients, parents, and HCPs involved in the clinical trial. In addition, data were collected through questionnaires embedded in the self-management app. Results Support for enzyme dose calculation and nutrition management was found to be particularly useful. Patients and parents rapidly strengthened their knowledge about the treatment and increased their self-efficacy. Reported benefits include reduced occurrence of symptoms and enhanced quality of life. Patients and parents had different skills, requiring follow-up by HCPs in an introductory phase. HCPs valued obtaining precise information about the patients, allowing for more personalized advice. However, the tight follow-up of several patients led to an increased workload. Over time, as patient self-efficacy increased, patient motivation for using the app decreased and the quality of the reported data was reduced. Conclusions Self-management enfolds a collaboration between patients and HCPs. To be successful, a self-management approach should be accepted by both parties. Through understanding behaviors and experiences, this study defines recommendations for a complex case—the demanding treatment of CF. We identify target patient groups and situations for which the app is most beneficial and suggest focusing on these rather than motivating for regular app usage over a long time. We also advise the personalized supervision of patients during the introduction of the approach. Finally, we propose to develop guidance for HCPs to facilitate changes in practice. As personalization and technology literacy are factors found to influence the acceptance of digital self-management of other chronic diseases, it is relevant to consider the proposed recommendations beyond the case of CF.