PKU Low Calorie Drink Study

Evaluating the Compliance, Acceptability, Safety and Tolerance of a Lower Calorie Protein Substitute for the Dietary Management of Phenylketonuria in Children and Adults - a Pilot Study

This study will evaluate the compliance, acceptability, gastrointestinal (GI) tolerance and safety of a lower calorie amino acid based liquid protein substitute for patients with Phenylketonuria (PKU) or hyperphenylalaninemia (HPA).

Inclusion Criteria:

• Male or female
• 3 years of age or above
• Diagnosed with classical or variant type phenylketonuria (PKU), or hyperphenylalaninemia (HPA)
• Have been compliant in taking at least one amino acid-based, Phe-free protein substitute, providing at least 20g protein equivalent daily, for at least 1 month prior to study commencement
• Have a prescribed daily Phe allowance
• Written informed consent from patient, or from parent / carer if applicable

Exclusion Criteria:

• Pregnant or lactating
• Requiring enteral tube or parenteral nutrition
• Major hepatic or renal dysfunction
• Participation in other studies within 1 month prior to entry to this study
• Allergy to any of the study product ingredients
• Investigator concern around the patient being underweight (e.g.: BMI lower than 18.5kg/m2 for adults) and/or having an eating disorder
• Investigator concern around willingness/ability of patient or parent/carer to comply with protocol requirements