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Life with PKU can be A-OK

By Tom Larson

Sun Tribune

Tajara Kill looks like any normal third-grader, shyly raising her hand when a question is asked and beaming when she delivers the correct answer.

And Tajara, 8, is like all the kids her age, except that they've noticed she doesn't always eat what they eat during lunch, that there are some snacks she has to turn down.

Tajara has phenylketonuria, a rare disorder commonly called PKU that is an inherited metabolic disease. It's caused by the absence of an enzyme that converts phenylalanine to tyrosine. Failure of the conversion to take place results in a buildup of phenylalanine, which is toxic to the central nervous system and can cause mental retardation and other neurological problems when treatment is not started within the first few weeks of life.

By adhering to a strict diet and with help from a special formula, children with PKU can expect normal development and a normal life span.

Tajara's family is particularly aware of what PKU is and what must be done to manage it. Tajara's younger sister, 3-year-old Trenity, also lives with PKU.

Tajara's life with PKU gave St. Mary's third-grade teacher Barb Spaulding a perfect teaching moment -- or in this case, 90 moments.

Beth-Ann Bloom and Amy Gaviglio, Genetic Counselors at the Minnesota Department of Health, met with Spaulding's class Wednesday afternoon to teach the kids about the disease that affects their friend and classmate.

Through a series of games, readings, quizzes and -- most popularly -- through treats, Bloom and Gaviglio brought home the lesson of PKU.

"People think people with PKU can't eat anything that tastes good," said Bloom, as she and students mixed up a snack that included Bugles, popcorn, sugary cereal and fruits. "But they eat stuff like this. This tastes pretty yummy, doesn't it?"

Of the 72,000 babies born annually in Minnesota, only about six are diagnosed with PKU. All babies are screened for it and other diseases and disorders as soon as they're born.

About three days after Tajara's birth in Morris, tests showed she had PKU, and she was sent to University of Minnesota hospitals for more tests and to determine her treatment. Aside from the formula, no other medicine is needed to maintain a normal life, said Tajara's grandmother, Cecilia Kill, who attended Wednesday's program.

Tajara's diet can't include foods such as steak, hamburger, chicken, dairy products, eggs, peanut butter, nuts or beans -- anything that contains high levels of protein.

She can eat certain pastas, breads, rice, corn, potatoes and just about any fruit, vegetable and juice. The family also buys a special flour mixture for things like cookies, Cecilia said.

While many types of candy are OK for Tajara, chocolate is not, and any products that contain artificial sweeteners are strictly forbidden.

The St. Mary's cafeteria works with the family to plan Tajara's school menu, she said.

But the formula -- which Gaviglio called "magic milk" -- also must be a staple of Tajara's daily diet and it is expensive, Cecilia said.

And while she has't grown up eating hamburgers and peanut butter sandwiches, it doesn't mean there isn't a longing.

"Probably more when she sees other people eating them," Cecilia said. "She knows she can't have them, but it's still tough."

Tajara, her family and others with PKU become quite good at reading labels and identifying the contents of foods she should avoid.

Bloom and Gaviglio also played a game in which Spaulding's third-graders were given small pictures of foods and candy and then were asked to decide which ones a person with PKU could eat.

The students got the hang of the game quickly and, for the most part, were able to give reasons why Tajara could or couldn't eat them.

One of Tajara's classmates, however, didn't need a label to know if the treat she was asked to talk about was A-OK for PKU.

"I've got Skittles, and I think they're OK," the girl said, turning to Tajara, "because I see you eating them all the time."

Tajara beamed again, just a normal girl with a sugar tooth.