Many people with cystic fibrosis (CF) need help to ensure they’re getting adequate nutrition and the correct amount of enzymes, as well as related reminders. As part of the European MyCyFAPP project, researchers are developing a digital support device to promote autonomy and proper nutrition among CF patients.
CF is a genetic disease, causing severe damage to the lungs and the digestive system. Patients can experience insufficient pancreatic activity, often resulting in maldigestion and malabsorption, leading to malnutrition and growth problems.
An individualized therapy based on enzyme replacement can relieve many of the life-shortening side effects of cystic fibrosis. The MyCyFAPP project incorporates this therapy in an innovative information and communication technology tool. The project’s aim is to develop an app for self-management of enzyme replacement therapy in children with cystic fibrosis through a personalized and interactive monitoring and learning process.
MyCyFAPP is a project funded by the European Commission under the Framework Programme for Research and Innovation HORIZON 2020. Twelve organizations from seven European countries added their expertise to successfully engineer a new Pancreatic Enzyme Replacement Therapy (PERT). They are making it accessible and user-friendly to CF patients and their families as a mobile app.
MyCyFAPP will replace traditional in-hospital and outpatient consultations (physicians and dietitians) by mobile consultations, allowing for continuous follow-up and fast interventions if needed, and avoiding tedious and expensive analytical determinations (stools collection).
The app will ultimately improve the nutritional status and quality of life of patients and families, and reduce medical costs for healthcare systems and families.
Developing this app is not an easy task.
The team of researchers leading the project conducted interviews with 71 people from seven European countries to get an indication of the needs that this app should meet. This included patients, caregivers, families, and health professionals, as well as health monitoring authorities.
“We knew that this work wasn’t going to be easy,” said Jacqueline Floch, who is leading the work being carried out at SINTEF, in a news release. “However, the complexity of the problem still surprised us.”
After considering the information gathered from the interviews, the researchers came up with 466 different and complex needs.
“Firstly, we observe that all CF sufferers are different,” Floch said. “Some are very organized when it comes to monitoring their illness, while others are far less scrupulous. Some children may also be in denial because they don’t want to appear different. Some get a lot of support from their parents, who lay out the medicines and teach the kids how to take them, while others get almost no help at all. This means that while some need daily reminders to take their medicines, others have a more pressing need simply to talk to like-minded people about their disease.”
Doctors participating in the MyCyFAPP project consider that it is important to measure a patient’s enzyme doses correctly, as nutrition affects all health aspects of CF patients. In contrast, adults with CF and parents of children with the disease consider that they are capable of controlling enzyme doses.
“However,” Floch said, “the project found that in Europe there are large variations in the enzyme dose recommendations provided by doctors, and there is reason to believe that overdosing is being carried out in some places. We’re also seeing that many CF sufferers are looking for more than just a support device to help them calculate their enzyme doses. For example, some want an intelligent system that can make recommendations about treatment based on their own specific symptoms.”
“The doctors taking part in the project are skeptical to this suggestion. They fear that such a system will deliver incorrect advice,” Floch added. “Our view is that the doctors’ knowledge and experience must be taken seriously and that it is important that they carry out quality assurance of the work linked to the project. At the same time, it’s important that the system we develop is perceived by its users as beneficial. If not, no one will use it.”
The study also made researchers realize that there are important differences between the countries taking part in the MyCyFAPP project. For example, Norway has a well-organized CF user association (the Norwegian Expert Centre for Cystic Fibrosis, or NFCF) that has created Facebook closed groups, and has a service that offers trained peer mentors to support others in the same situation. Norway is the only country in the project that provides such a system.
Results from the interviews also showed that there are common needs to all participants. Doctors consider that for patients with CF, a good understanding of their disease is critical for better treatment monitoring. Parents also consider that is important for their children to know more about CF, and the young people taking part in the project agree. Another important aspect is the need to record a daily log of symptoms and overall health status.
Researchers of the MyCyFAPP project have categorized the needs they found into a set of groups based on the calculation of enzyme doses; nutritional monitoring; a daily symptoms log; learning; reminders; treatment monitoring support; digital forums; and communication between CF patients and their doctors.
Participating doctors have been working to assign priorities between these groups, and high priority was given to the symptoms log and learning. Nevertheless, enzyme doses, correct nutrition, the intake of calorie-rich foods, and a balanced diet are the most important factors.
Based of these priorities, the researchers have organized “co-design workshops,” where adult CF patients, children ages 4 to 15, and their parents are working together to create ideas for games and make sketches for an app to help improve CF patients’ autonomy. Health professionals are also drafting ideas for a monitoring device.
“Software development is well underway, and an initial assessment is planned for this autumn,” Floch said. The goal is to create software prepared for a clinical assessment in the summer of 2017.
The MyCyFAPP project is expected to provide a digital platform to promote the autonomy of patients and their families, and to decrease the distance between patients and their health staff.