VERSAILLES (AP) - For more than a decade, the 200 Mennonite families in this tight-knit Central Missouri community have welcomed visiting university scientists with open arms.

The researchers are no cultural tourists. As gene hunters tracking a rare disorder, their help can mean the difference between life and death for Mennonite infants.

Cooperation from Missouri Mennonites helped University of Missouri-Columbia biochemistry Professor Charlotte Phillips develop a DNA cheek swab that allows for a 24-hour or quicker screening of newborns at risk of maple syrup urine disease, which can be fatal if not promptly treated.

Now Phillips and other university researchers hope to expand their efforts to Kansas, Iowa and six other border states with a grant from the Heartland Regional Genetics and Newborn Screening Collaborative in Oklahoma City.

"If you’re not suspecting maple syrup disease, it takes five days to a week before you get results" through normal state newborn screening tests, genetic counselor Dawn Peck said. "You’re not getting your answer in time to prevent symptoms."

Maple syrup urine disease, or MSUD, is an amino acid disorder that gets its name from the burnt sugar odor first detected in babies’ diapers by Mennonite midwives decades ago.

In the general population, the MSUD rate is one out of every 225,000 births. But among Mennonites, decades of intermarriage have increased that rate to one in 150 births.

Infants with the inherited disorder don’t process protein properly, which results in an excess of three amino acids that are toxic to the brain. Babies can die within two weeks to a month if not treated or suffer brain and neurological damage if the disorder isn’t promptly treated.

Early detection enables families to quickly use a light-protein liquid formula to stave off mental retardation and other developmental disabilities.

The few hours immediately after birth can make a difference. Phillips, Peck and their research team members can rush to Mennonite communities such as Versailles and Memphis in northern Missouri and then to their Columbia lab the moment they learn a susceptible infant has been born, regardless of the time of day.

For MSUD patients, the regimen that starts in infancy becomes a lifelong commitment to a low-protein diet free of meat and dairy.

"They’re going to drink formula for the rest of their lives," Peck said.

Mennonite dairy farmer Lawrence Martin and his wife Malinda have eight children, ages two to 15. Neither knew they were MSUD carriers until the birth of their second child, daughter Verna.

After three days, the child’s high-pitched cry and poor nursing habits prompted a call to their midwife, who suggested an appointment with a neurologist.

When another Mennonite family told them about MSUD, the Martins turned to a community member who drives - the couple uses a horse and buggy for transportation - and immediately caught a ride to the University Hospital emergency room 75 miles away in Columbia.

Verna, now 13, spent her first few years in special education classes at the local parochial school but has since advanced to her grade level, the Martins said. Every child born since then has been promptly screened for the disorder, including their 6-year-old son Phares, who also has the disease.

The special formula costs the family nearly $10,000 a year - money their neighbors and church members raise through an annual benefit auction and bake sale.

Copyright 2007 Associated Press. All rights reserved. This material may not be published, broadcast, rewritten, or redistributed.

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