Rare Disease Report

PKU Sphere Introduced by Vitaflo USA

APRIL 25, 2017
Mathew Shanley
The launch of PKU Sphere – a powdered, low phenylalanine medical food used in the dietary management of Phenylketonuria (PKU) – was announced by Vitaflo USA this morning.

Vitaflo, a Nestle Health Science Company, is a developer of innovative specialized products for areas of disease-specific nutrition, and PKU Sphere is their first Glycomacropeptide (GMP)-based medical food containing a balanced mix of GMP and amino acids. GMP, a protein derived from milk, is naturally very low in phenylalanine, and has the potential to help patients meet their necessary levels of both protein and general nutrition.  

PKU, an inherited genetic disorder that reduces the ability to metabolize the amino acid phenylalanine, affects an estimated 1 in every 15,000 people in the United States. In these patients, an untreated phenylalanine buildup can cause intellectual disability and other serious health-related issues.

"For a healthy life, patients living with PKU must follow a specific diet that limits phenylalanine for the rest of their lives, which can be a great challenge," said Jennifer Szymanski, General Manager of Vitaflo USA. "Launching PKU Sphere is the next step in our mission to create medical foods that combine clinically-supported benefits with the lifestyle needs of every patient we serve."

By offering low levels of phenylalanine and a balanced combination of GMP, amino acids, carbohydrate fat, vitamins, minerals, and DHA, the new food addresses the unique nutritional needs of patients living with this rare disease.

"There is emerging evidence in PKU linking GMP with potential benefits in improved protein utilization, bone health and gastrointestinal wellbeing," said Anita MacDonald, RD, Honorary Professor in Dietetics, Birmingham Children's Hospital.

"With more than two years of experience using PKU Sphere in children and teenagers in the UK, we have assessed its acceptability and tolerance, plus its effects on plasma amino acid profiles, micronutrient levels and growth when compared with conventional supplements. Children are able to maintain good adherence with PKU Sphere, and with careful introduction and regular monitoring, it is a safe and effective alternative medical food for the management of PKU,” Dr. MacDonald continued.

PKU Sphere is suitable for patients six years and older, and currently available in two flavors: red berry and vanilla. More information can be found by visiting the Vitaflo website.

About Phenylketonuria (PKU):

Phenylketonuria is an inherited disorder that increases the levels of a phenylalanine – a building block of proteins – in the blood. It is caused by mutations in the gene that assist in making an enzyme called phenylalanine hydroxylase. Symptoms of PKU include skin rashes, intellectual disability, delayed social skills, seizures, hyperactivity, musty odor, and microcephaly.


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