For 28 days, 20 patients with tyrosinaemia will take TYR sphere as part of the usual dietary management of their condition. Patients will record their adherence to recommended intakes each day, gastrointestinal symptoms (if any) experienced each day of weeks one and four, and the palatability of the product at the end of the trial. Metabolic control will be evaluated by dried blood spots collected during the Baseline and End of Study visits, and weekly during the 28 days of the study itself. Trial data will be used in an application to make TYR sphere available via the NHS.
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Adherence to prescribed intake of TYR sphere
Timeframe: Days 1 to 28
Patient record of gastrointestinal tolerance, week 1
Timeframe: Days 1 - 7
Patient record of gastrointestinal tolerance, week 4
Timeframe: Days 21 - 28
Patient assessment of palatability of TYR sphere
Timeframe: Day 28
Change in metabolic control (tyrosine) over study period
Timeframe: One at Baseline Visit (Day 1) one per week during Evaluation Period (Days 1 - 28) and one at End of Study visit (Day 28)
Change in metabolic control (phenylalanine) over study period
Timeframe: One at Baseline Visit (Day 1) one per week during Evaluation Period (Days 1 - 28) and one at End of Study visit (Day 28)