FOX47 NEWS - Living With PKU

Jeff Angileri
Apr 30, 2008, 10:17 PM

Jessica Zimmerman has to track everything she eats throughout the day.

We always say that her diet is stricter than a vegan- to give people a sense of it- she can't have any commercial bakery products along with no meats, dairy, says Jessica's mother Ann Zimmerman.

She can have some proteins, but it has to be very carefully measured.

What we're trying to do is get her to that exact amount every single day, Zimmerman says.

Jessica has a disorder called Phenylketonuria or PKU.

It's a disorder that's an interference with the ability to mobilize Phenalanaline, says Denise Ney of the UW Department of Nutritional Sciences.

Penalanaline, or phee, is found in majority of proteins.

And because people with PKU can't metabolize it, the phee is left building up in the blood.

Experts say diagnosing a child with the disorder at birth is critical.

If they're not, and left on their mothers milk or regular infant formula they will be severely mentally retarded, Ney says.

People diagnosed with PKU follow a strict diet.

Just because they can't eat protein, doesn't mean they don't need it.

Jessica, like all PKU patients, gets the majority of her protein from an amino acid formula.

She has to drink 18 ounces of the mixture a day.

The problem is,

It smells really bad, says Jessica Zimmerman.

And according to most, it tastes even worse.

But there soon could be an alternative to the stinky formula.

A special task force of UW scientists and medical professionals at the Waisman Center in Madison are working on what can be considered a break through in the treatment of PKU.

It's called GMP, a protein found in whey which is plentiful here in Wisconsin.

Wisconsin makes the most cheese and there is nine pounds of whey for every pound of cheese, says Mark Etzel with the UW's Department of Food Sciences.

Unlike the amino acid formula, GMP can be used as an ingredient.

What we decided was that we can make some food products that were tastier than what they had to consume everyday to get their protein, says Kathy Nelson with The Wisconsin Department of Dairy Research.

The center has used the GMP protein to make crackers, fruit roll ups and the most popular product, strawberry pudding.

But GMP is still in the testing phase.

The UW conducted a study on mice with PKU.

Half the animals were put on the traditional amino acid formula diet and the others on GMP.

The results showed mice on the GMP diet had lower levels of phee in their brains.

This is important because the damage to an individual, to their nervous system occurs when the phenananiline accumulates in the brain, Ney says.

The UW is currently conducting a similar study on people with PKU.

Our data shows so far that there is no change in the blood phee levels, Ney says.

Which means the GMP can at least be used as an alternative.

Jessica was one of the first to try the GMP pudding.

She says the GMP products would do more than just give her more choices for food,

It will help her feel more like everyone else.

I always have to have special stuff, special food like when someone brings birthday treats to school I have to have a special birthday treat bag. It would be really helpful because I always feel like the oddball out, Jessica says.

Ney says she requested grant money for a national GMP study to test the diet in four locations across the US.

She says they'll find out if they're approved some time this summer.

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