
"When Dr. Morton told us that our daughter Barbie Ann had
glutaric aciduria, John and I thought he had made a mistake,"
says Lydia Stoltzfoos, daughter-in-law of Jacob and Naomi. But
Barbie Ann soon developed a fever, and Morton admitted her to
Lancaster General. Stressed by infection, a child with glutaric
aciduria does not metabolize certain amino acids normally. The
resulting buildup of glutarate attacks the nervous system and
damages the basal ganglia, a part of the brain that controls
body movement. Once brain injury occurs, a child never recovers.
"If it weren't for Dr. Morton and the clinic," Lydia says,
"Barbie Ann would have ended up like many of Amos and Suzie
Miller's children. Five of them died or are paralyzed because of
this disease."
Over the years, Morton has traced each family's genetic heritage
through 14 generations. He has determined a carrier frequency
for the disorder among the Amish of about 1 in 10 people.
Working with Dr. Richard Kelley, a pediatrician, Morton
diagnosed glutaric aciduria in 16 other Amish children. The
doctors' studies predicted that 50 more children born in the
next generation would inherit the two copies of the defective
gene needed to cause the disorder. According to the statistics,
without treatment nearly all would be disabled, and 12 of them
would die before age 5. "Glutaric aciduria is a treatable
disease, so we need to know who is at risk during the first few
days of life," says Morton.
Discovering early just who has glutaric aciduria is more than
half the battle. Once the disease is diagnosed, Morton's main
task is to put his young patients on a low-protein,
high-riboflavin diet to lessen the effects of the disorder and
prevent medical complications. If a stricken child can survive
to age 5 with this help, he or she typically becomes resistant
to the worst of the disease.
Meanwhile, Morton's clinic has become a model for rural health
care, reducing hospitalization for the disorders to one-tenth
their historic rates. The Amish and Mennonites who use the
clinic do not buy medical insurance or subscribe to Medicare,
but instead depend on family and community for help. Says Dr. C.
Everett Koop, former U.S. Surgeon General: "Holmes Morton has
forced his way into the hearts of the Amish people and, based on
that trust, has accomplished a remarkable service."
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