BIRMINGHAM, Ala. - Chris and Katherine Key have owned Steel City Fitness for 10 years, and up until 13 months ago had never heard of the condition phenylketonuria (PKU). That's when their children Cole and Grace, twins, were born with it.
PKU is a rare inherited metabolic disease that arises from the absence of a single enzyme (phenylalanine hydroxylase). This enzyme normally converts the essential amino acid, phenylalanine, to another amino acid, tyrosine. Failure of the conversion to take place results in a buildup of phenylalanine, which is toxic to the central nervous system and causes severe mental retardation unless a strict diet is followed.
The diet consists of eliminating all high-protein foods, since all protein contains phenylalanine. Except in rare circumstances, the diet does not allow the consumption of meat, fish, poultry, milk, eggs, cheese, ice cream, legumes, nuts or many products containing regular flour. A synthetic formula is used as a nutritional substitute for the eliminated foods. It costs the Keys $2,000 a month for the formula alone. Only a few states have mandated insurance coverage for the cost.
If you call that a burden, don't let the Keys hear you. "We feel that we were blessed with our babies, and that God gave them to us because we are in the health and fitness industry and we know a lot about nutrition and are able to handle it," said Chris.
Not only are the Keys handling their situation, they also have started the PKU Foundation, a nonprofit foundation with three main purposes: 1) to raise money to help families who can't afford the synthetic formula, 2) to get legislation passed that forces insurance companies to pay for the formula, 3) to raise money for research to develop better-tasting formulas and to find a cure.
The first fund-raiser the foundation is holding is the Bod Pod Challenge. It's a giveaway for a Jeep Cherokee, a Harley Davidson and $1 million.
The way the contest works is that the contestants have to get tested on the Bod Pod - a body composition system. To become a contestant you must first donate $50, which gives you two free Bod Pod tests. The 14 people who have the biggest total body composition change over a three-month period - and a 15th contestant, who will be picked from a drawing that costs $5 a ticket - get to participate in a half-court basketball shot contest or a putting competition. Whoever wins the competition wins the prize. The first giveaway contest for the Jeep Cherokee will take place in February.
"We're hoping that the contest will motivate people to get into better shape and raise a lot of money for a wonderful cause," said Chris.
For more information on the Bod Pod Challenge, call (888) 282-5110.