WHEN you tuck into turkey with all the trimmings this Christmas Day spare a thought for little Usman Baker, whose rare protein intolerance means turkey, mince pies and even chocolates are all off the menu.

Eight-year-old Usman, from Barton, suffers from the rare, inherited, metabolic disease Phenylketonuria (PKU), which means eating the wrong food could lead to tragic results.

A pupil at the estate’s Bayards Hill Primary School, he is a keen dancer, cook and gardener, and looks just like any other healthy boy of his age.

But a build-up of protein in his body could lead to permanent brain damage.

Mum Safia, 42, said: “Usman’s main diet is fruit and veg, so every day is challenging for him, but Christmas is especially difficult because yummy food is being enjoyed all around him.”

Roast potatoes are allowed, but have to be weighed as do Brussels sprouts. Any one helping can’t weigh more than 55 grammes before it becomes harmful.

She added: “But there is definitely no meat allowed, or stuffing or Yorkshire puddings and chocolates are also banned, which is especially hard for him when they are everywhere at Christmas.”

Only one in 10,000 people have PKU – a disorder which arises from the absence of a single enzyme called phenylalanine hydroxylase.

This enzyme normally converts the essential amino acid, phenylalanine, to another amino acid, tyrosine.

But in people with PKU this does not happen and a build-up of phenylalanine, which is toxic to the central nervous system, can eventually lead to braindamage.

Their diets are supplemented with artificial protein which allows normal growth and development.

Mrs Baker said: “Usman can often get really teary and upset as his condition is always going to be there. He asks questions like: “What does chicken taste like? and “When I eat food you eat does it taste the same in my mouth as it does yours?

“Thankfully, each year he attends the Great Ormond Street Hospital (GOSH) Christmas party for children with PKU and he enjoys a feast of foods which have been prepared for children like him.”

Usman said: “I wish I could have stuffing. The chocolate in shops and cakes look yummy and I would like to try the turkey, but I can’t.

“But the GOSH party is fun. I meet other kids, we have lots of fun and freebies and Santa comes with gifts.

“I can have whatever I want to eat and it’s all without protein. Even the drinks. It makes me feel normal because I eat the same as everyone else there.”


The only way to treat PKU is to restrict the amount of phenylalanine entering the blood.

A PKU diet avoids food and drink that contain large amounts of phenylalanine.

Depending on their degree of PKU, sufferers cannot eat meat, cheese, poultry, eggs and milk, plus many other foods such as bread, pasta, chocolate and many cereals.

Foods such as cereals, some vegetables and milk contain small amounts of protein. These foods are allowed in the diet in weighed amounts called “exchanges”.

This provides a controlled intake of phenylalanine needed for body tissues to grow and function normally.