Brother and Sister with PKU

Almost every American baby at birth receives a needle stick to collect a few drops of blood to test for phenylketonuria, or PKU, a rare, inherited birth defect. A Mayo Clinic website reports that a person born with PKU is ‘missing or deficient in a specific enzyme needed to process an essential amino acid called phenylalanine.’

The gene defect occurs mostly in people from Northern European backgrounds. If having too much protein in their diets, these people at anytime in life can develop mental retardation and a host of physical problems. They have to monitor everything eaten.

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⤥ Summer Fun Starts Here ⤦

⤥ Summer Fun Starts Here ⤦

Jenny Barnes, from the Midwest, and her younger brother, were both born with PKU. ‘I remember in kindergarten,’ said 35-year-old Barnes in a telephone interview, ‘when we had snack time. I couldn’t eat what the other children had. Instead of milk, I drank Tang. For lunch, my mom would circle on the school menu the items I could eat.’ Her parents always stressed that she could never depart from her diet.

But when she was in second grade, doctors as an experiment allowed her to eat protein for a while, in order to monitor whether her system could handle it. She said, ‘I remember my parents feeding me a burger. At first, I took a bite and spit it out. I didn’t like the taste.’ The experiment didn’t last long.

In high school, she said only her best friends knew about her having PKU. Later in life, she said that on occasion while eating out with new friends she had ordered items containing protein, and not eaten much of it, because she didn’t want to go through all the hassle of having to explain PKU.

In 2006, doctors learned Barnes also had diabetes, and she began insulin shots four times a day. Because of PKU, she must drink each day a special formula with necessary nutrients.

Having PKU, and not being able to freely eat protein, seems odd given her occupation. She and her brother work at their family’s restaurant, one well-known in part for quarter-pound hamburgers.

‘It’s never bothered me,’ Barnes said of being around meat. ‘Working in a restaurant is my life, my livelihood, and my brother and I want to take over from our parents some day.’

One thing: She and her brother will never be accused of eating the profits.