PKUCycleAmerica Press Information

PKU Grandfather cycling across America to raise awareness about PKU and $100,000 for PKU research

Tia is a five year old with PKU, which means she can’t eat a normal meal like most five year olds.  Tia is missing an enzyme that breaks down the amino acid Phenylalanine (Phe) so the body can use it.  So Tia’s intake of Phe has to be controlled with a very restricted diet or she will suffer severe, irreversible consequences that could include delayed mental and motor skills, seizures, speech delays, hyperactivity, and behavioral abnormalities.  PKU is rare, only occurring in 1 in 10,000 births in the US.  Within 48hrs after birth, each child in America is tested for PKU.  If PKU is confirmed, the baby must start on a medical food formula within the first few days or severe mental retardation will occur.  This formula contains the other essential amino acids except Phe.  Currently Tia has to drink this formula three times per day to get her daily protein requirements.  Tia can eat some other foods with low levels of Phe like fruits and vegetables, but everything she eats must be measured so the amount of Phe is known down to the milligram.  Tia must maintain this restricted diet for the rest of her life since there is no cure for the enzyme deficiency and her tolerance for Phe in normal foods doesn’t increase with age.

Approximately 15,000 to 20,000 children and young adults in the US have PKU.  Their families are struggling with this restricted diet every meal.  If the diet is maintained throughout their lives the children grow up normally.  While it is never easy, young children adhere to the diet more readily when they are under the control of their parents each day.  In preteen and teen years well more than half drop off the diet for some time or totally with that number increasing to 60%–70% off diet once they become adults.  The PKU community has organized to form a new national organization, the National PKU Alliance (NPKUA), whose mission is to improve the lives of individuals and families associated with PKU through research, support, education and advocacy, while ultimately seeking a cure.  NPKUA will support promising research such as enzyme replacement therapies or low-Phe naturally occurring food products so PKU children can eat more normal diets. 

Dick Michaux from Vail, CO is Tia’s grandfather.  He and a family friend, Jim Burger from Encampment, WY, are cycling 3,800 miles across America to raise funds to support this research.  They are stopping in communities along their route to visit with PKU families.  They hope to raise awareness about little known PKU during these stops.

To donate to or learn more about Dick and Jim’s cross country cycling trip please visit www.PKUCycleAmerica.com. To learn more about PKU please visit www.PKU.com.

Dick Michaux   970 471 1417  or  dick@michauxfamily.com