Posted: Jul 2, 2008  13:30

A Moyie Springs family whose son has a rare genetic disorder is not only learning to cope with a disease that will not allow children to eat a normal meal due to an enzyme their body lacks; they are hoping a grandfather's cross-country bicycle trip will raise awareness and research money to fight Phenylketonuria.

Tara Nelson's son, Bryce, is a victim of the inherited disorder, also known as PKU, which he was diagnosed with shortly after his birth.

"The the only thing I knew about PKU was that the initials described the test that is done on all babies when they are born," she Nelson, who works for the Beverly Kee CPA firm in Sandpoint. "I, like most people, had no idea how many genetic birth defects there were, how rare they are, or how they affect your life. I have since become somewhat of an expert on at least one of them."

Nelson - whose husband Del works for BF Redi Mix - said doctors in Sandpoint had never seen a case of PKU before.

"When I had him, it had been 19 years since the last child was born with PKU in North Idaho, in Post Falls," she said. "Needless to say, it is rare. However, with increasing population, it has become somewhat more common in our area. There was another baby born with it in Hayden a few years ago, and two children with it moved into Sandpoint some years back as well."

Nelson described her son a very bright, understanding and loving child, but has "classical PKU" in the most severe form.

PKU is a rare, inherited, metabolic genetic disorder characterized by the inability of the body to utilize the essential amino acid, phenylalanine. Those with PKU accumulate too much phenylalanine, one of the amino acids found in protein-containing foods. For unknown reasons, an excess of phenylalanine in an infant's body is harmful to the development of the brain, causing mental retardation unless treated during infancy.

She has since learned that a man whose granddaughter has PKU is bicycling 3,800 miles across the U.S. with a friend to raise awareness for the disease and money to fund research for a therapy starting in clinical trials.

Though they are riding through parts of Idaho, north Idaho is not on their route.

"I think it is a great thing that someone is doing something like biking across America for charity," said Nelson.

To learn more about the route the pair is travelling, PKU, what you can do to help, go to http://www.pkucycleamerica.com.