Introducing a granule based protein substitute to the diet of a child with phenylketonuria to address reluctance to ingest phenylalanine-free protein substitute: A case report.

Abstract

Phenylalanine (Phe)-free protein substitutes are used within the management of phenylketonuria (PKU). However, adherence to the Phe-restricted diet is often challenging. A child (age 4.5 years) with PKU rejected the Phe-free protein substitutes used within her therapeutic diet, causing stress for herself and family at mealtimes. Switching to a new Phe-free protein substitute that can be mixed into other foods [PKU GOLIKE^® (3-16)] provided an alternative strategy that was acceptable to the child. Good control of blood Phe was maintained. Newer Phe-free protein substitutes may provide a strategy for maintaining the therapeutic diet for PKU where the patient has difficulty doing so on standard substitutes. Here, the use of a Phe-free protein substitute with improved palatability and ease of use supported maintenance of the Phe-restricted diet for a child with PKU who struggled to maintain the diet on standard substitutes.