A preliminary study of telemedicine for patients with hepatic glycogen storage disease and their healthcare providers: from bedside to home site monitoring

Irene J Hoogeveen,Rob Burghard,Francjan J Van Spronsen,Terry G J Derks,Robert‐Jan Zandvoort,Charlotte M A Lubout,Sebastiaan Te Boekhorst,Tom J De Koning,Foekje De Boer,Fabian Peeks

doi:10.1007/s10545-018-0167-2

Irene J Hoogeveen, Rob Burghard + Show 8 more

Open Access

https://doi.org/10.1007/s10545-018-0167-2

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Abstract

BackgroundThe purpose of this project was to develop a telemedicine platform that supports home site monitoring and integrates biochemical, physiological, and dietary parameters for individual patients with hepatic glycogen storage disease (GSD).Methods and resultsThe GSD communication platform (GCP) was designed with input from software developers, GSD patients, researchers, and healthcare providers. In phase 1, prototyping and software design of the GCP has occurred. The GCP was composed of a GSD App for patients and a GSD clinical dashboard for healthcare providers. In phase 2, the GCP was tested by retrospective patient data entry. The following software functionalities were included (a) dietary registration and prescription module, (b) emergency protocol module, and (c) data import functions for continuous glucose monitor devices and activity wearables. In phase 3, the GSD App was implemented in a pilot study of eight patients with GSD Ia (n = 3), GSD IIIa (n = 1), and GSD IX (n = 4). Usability was measured by the system usability scale (SUS). The mean SUS score was 64/100 [range: 38–93].ConclusionsThis report describes the design, development, and validation process of a telemedicine platform for patients with hepatic GSD. The GCP can facilitate home site monitoring and data exchange between patients with hepatic GSD and healthcare providers under varying circumstances. In the future, the GCP may support cross-border healthcare, second opinion processes and clinical trials, and could possibly also be adapted for other diseases for which a medical diet is the cornerstone.

Highlights

Hepatic glycogen storage diseases (GSDs) are a group of inborn errors of carbohydrate metabolism, for which a strict diet is the cornerstone of management
The GSD application (GSD App) can be accessed by going to https://app.gsdapp.nl in the internet browser on a mobile phone
The GSD clinical dashboard can be accessed via a desktop browser: https://clinicaldashboard.gsdapp.nl

Summary

Introduction

Hepatic glycogen storage diseases (GSDs) are a group of inborn errors of carbohydrate metabolism, for which a strict diet is the cornerstone of management. A case report on two patients with GSD IXa presented improvement in liver cirrhosis on ultrasound after improving metabolic control (Tsilianidis et al 2013). The purpose of this project was to develop a telemedicine platform that supports home site monitoring and integrates biochemical, physiological, and dietary parameters for individual patients with hepatic glycogen storage disease (GSD). The GCP may support cross-border healthcare, second opinion processes and clinical trials, and could possibly be adapted for other diseases for which a medical diet is the cornerstone

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