Evaluation of PKU Sphere

Evaluation of the Acceptability, Tolerance, and Satiety of PKU Sphere, a Glycomacropeptide (GMP) Based Medical Food in Patients With Phenylketonuria (PKU).

A prospective, open-label study of PKU Sphere in patients with PKU following a phenylalanine restricted therapeutic diet.

The rationale for this study is to assess acceptability, adherence and metabolic control in individuals with PKU consuming PKU Sphere, a GMP-based medical food.

The sponsor developed PKU Sphere in response to growing interest in GMP medical foods for the purpose of improving adherence and quality of life by offering an alternative to amino acid based medical foods. PKU Sphere is a powdered, low phenylalanine medical food containing a balanced mix of casein glycomacropeptide (GMP) isolate, essential and non-essential amino acids, carbohydrate, fat, vitamins, minerals and the long chain polyunsaturated fatty acid (LCP); docosahexaenoic acid (DHA). PKU Sphere has been designed for use in the dietary management of Phenylketonuria. It is available in two flavors, Red Berry and Vanilla, packaged in individual serving sachets of 35g and containing 20g protein equivalent (PE).

Inclusion Criteria:

• Diagnosis of PKU, identified by newborn screening (NBS) and treated since birth.
• Ages 3 years and above (this includes pregnant women who will be assessed on an individual basis by the Co-Principal Investigators).
• Currently or previously consuming a low phenylalanine diet supplemented with a medical food designed for the dietary management of PKU.
• English as primary language.
• Willingly given, written, informed consent from the participant, 18 or more years, or from the parent/caregiver for participants < 18 years.
• Willingly given, written assent (if appropriate) for those < 18 years.

Exclusion Criteria:

• Currently consuming 100% of the medical food component of the diet as PKU sphere.
• An inability, in the opinion of the investigator, to comply with the requirements of the protocol.
• Any other type of inherited metabolic disease.