10 participants aged 3 years and over with tyrosinaemia or alkaptonuria will be prescribed Tyr sphere following an assessment of their individual needs by their dietitian. All participants will enter a 4-week evaluation period, assessing adherence and gastrointestinal tolerance. Evaluations of Tyr sphere's palatability are made at the end of the evaluation period. Dried blood spots are taken on days 1 and 28 and once per week in between. Participants who continue to take the product at the end of their evaluation period will enter a follow-up period during which metabolic control, anthropometric and nutritional status data will be collected during the yearly standard of care routine visits.
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Change in adherence to recommended amounts of Tyr sphere
Timeframe: Recorded daily on days 1-28
Change in gastrointestinal tolerance
Timeframe: Recorded daily on days 1-28
Patient evaluation of Tyr sphere's palatability
Timeframe: Day 28, end of acceptability phase
Change in metabolic control: tyrosine levels
Timeframe: Day 1, week 1, week 2, week 3, week 4, day 28
Change in metabolic control: phenylalanine levels
Timeframe: Day 1, week 1, week 2, week 3, week 4, day 28