A Study To Evaluate The Acceptability Of the Express Plus Range, Foods For Special Medical Purposes (FSMP), In Children And Adults With Inborn Errors of Metabolism (IEM)
A prospective, open label, acceptability study to evaluate PKU, MSUD, HCU, TYR and GA express plus in the dietary management of 40 patients with IEM. The following parameters will be assessed: adherence to prescribed dietary intakes, palatability, usability, gastrointestinal tolerance, clinically relevant routine biochemical parameters, timeframe to transition and contribution of the express plus range to overall protein substitute intake over a 28 day period.
• Diagnosis of PKU or associated disorder for example, Tetrahydrobiopterin (BH4) deficiency, requiring a low protein diet and Phe-free L-amino acid supplements (protein substitute).
• Established on PKU express or an equivalent protein substitute
• In the opinion of the study investigator, the participant is able to take the study product and meets at least one of the following criteria:
‣ taking an age inappropriate protein substitute
⁃ struggling with adherence to current protein substitute
⁃ delayed in transitioning
• Aged 3 years and above
• Willingly given, written, informed consent from patient or parent/guardian.
• Willingly given, written assent (if appropriate).