A Diet Tracking, Monitoring and Optimization mHealth Tool for Patients with Inborn Errors of Metabolism

Inborn errors of metabolismIEMare a group of rare geneticinheriteddisorders that cause a block in a metabolic pathway leading to clinically significant consequencescausing developmental and intellectual disabilitiesseizuresandif untreatedcoma and deathThe majority of the IEM disorders are managed by manipulation and modification of diet alone to reduce toxic metabolitesor with a combination of dietary modification and medicationsThe complex dietary therapies used in IEM challenge routine dietary recommendations and render diet apps designed for the general public of little utility in these conditionsAlsono diet app is developed specifically for the IEM community and meets the rigor required for researchparticularly the need to measure specific and multiple amino acid content in the diet to facilitate more study on impact on brain neurochemistryWithin the field of IEMthe use of three day diet diaries is utilized to monitor food intakeThese diaries are frequently inadequate in capturing what is actually being consumed due to recall errors and other factorsNovel methods of assessing dietary intake and making individualized dietary recommendations for people with IEM are required to reduce the diet tracking burden and to improve the adequacy of dietary recommendationsWe propose to develop and validate MyRareDiet to address an unmet need in the IEM population to assist with dietary management designed to increase adherence and compliance that leads to improved outcomeswhile facilitating the collection of dietary data from individuals with IEM for research purposesMyRareDiet includes a Patient Portala Clinical Dietician Portaland a Researcher PortalThe Patient Portal is to help IEM patients track and monitor their own diet so they can meet their strict diet targets and restrictionsThe Clinical Dietician Portal is to help clinical dieticians manage diet modifications for individuals with IEMThe Researcher Portal is to facilitate the conduct of diet based studies and the analysis of study resultsThe aims for Phase I areCollect stakeholder feedback from stakeholdersincluding IEM patients caregiversdietitiansand researchers via focus groupsDevelop the prototype MyRareDiet system using user centered design and the latest mobile enabled e technologiesConduct prototype evaluation including usability and usefulness tests and validation study comparing patient caregiver recorded diets using MyRareDiet and those recorded by nutrition students interviewing patients caregivers with another diet recording toolOur success criteria are based on evaluations on system usabilityandgtperceived usefulnessandgtand the agreementandgtof diets recorded by patients caregivers and those by nutrition students Inborn errors of metabolismIEMare a group of rare geneticinheriteddisorders that cause a block in a metabolic pathway leading to clinically significant consequencescausing developmental and intellectual disabilitiesseizuresandif untreatedcoma and deathThe majority of the IEM disorders are managed by manipulation and modification of diet alone to reduce toxic metabolitesor with a combination of dietary modification and medicationsThe complex dietary therapies used in IEM challenge routine dietary recommendations and render diet apps designed for the general public of little utility in these conditionsWe propose to develop and validate MyRareDiet to address an unmet need in the IEM population to assist with dietary management designed to increase adherence and compliance that leads to improved outcomeswhile facilitating the collection of dietary data from individuals with IEM for research purposes