Doctor Declares One-Man War on Deadly Disease That Preys Upon Amish Children

Amid the rolling fields of rural Lancaster County, Dr. Holmes Morton is waging a one-man war on a deadly disease that preys upon Amish children.

It is a war the Philadelphia-area pediatrician knows he cannot easily win, a struggle in which victories are measured by the number of lives saved and youngsters spared from lifetimes of torment and disability.

The enemy is glutaric aciduria, an inherited disorder that strikes swiftly, silently and selectively.

The disease, which strikes mainly children, is found almost exclusively among the 15,000 Amish whose descendants settled southeastern Pennsylvania’s fertile valleys in the 1700s.

Morton diagnosed the disease about a year ago in the children of Amos and Susan Miller. Two of their children died. The others, while permanently crippled, are responding to a treatment Morton prescribed.

“This is a vicious disease,” said Morton, who commutes 45 minutes from his home in suburban Philadelphia to Lancaster to make his rounds. “And it’s an epidemic for the Amish.”

The Amish are peaceful people whose religion forbids the use of automobiles, electricity and other products of modern technology. But they have welcomed Morton and his war on disease with open arms.

There is no cure for glutaric aciduria. But if detected and treated early enough, it can be controlled and its effects diminished.

The disease manifests itself when a child comes down with a common ailment like measles or diarrhea and the kidneys are unable to properly dispose of glutaric acid. The acid buildup affects the liver and central nervous system and often causes the child to lapse into a coma.

Once the disease takes hold, it can kill within days. Children who survive often suffer progressive paralysis.

Morton says diet, not drugs, is the key to treating the disease. And a low-protein diet rich in riboflavin, a substance found in Vitamin B, seems to be effective in controlling the disease.

“By changing the amount of riboflavin they take in, you can alter the course of the disease and prevent them from suffering that damage,” Morton said.

The biggest challenge is identifying the children who have the disease before it strikes. Morton’s task was made considerably easier recently when Hewlett-Packard Co. donated an $80,000 mass spectrometer that can detect the presence of glutaric acid in urine.

The company donated the machine after an article about Morton’s work appeared in the Wall Street Journal. The 38-year-old doctor credits the article with sparking a large number of donations to the nonprofit organization he established to fight the disease.

Morton hopes to open a temporary clinic next month in space donated by Lancaster General Hospital. By next year, he expects to have a permanent site for the clinic.

To make it more convenient to people who get around in horse and buggies, the clinic will be among the Amish farms in western Lancaster County. It will be called the Clinic for God’s Special Children, which is what Amish and Mennonites call children with disabilities.

“We want a place with a view off the main road on about an acre of unfarmable land,” Morton said. “We want to have a horse barn for their buggies, and we plan to use Amish and Mennonite labor. It’s important that they identify it as their clinic.”

Newborns in families who have already had a child with glutaric aciduria have a 1-in-4 chance of contracting the disease, he said. Of the Amish population at large, one in every 200 to 300 newborns will develop the disease.

Using the mass spectrometer, Morton can conduct a $10 test that could save a family $100,000 in medical bills.

“We can save these children from a life of paralysis and give them back a life where they can work,” Morton said. “If we can salvage a half or three-quarters of them, we’ll have come a long way.”

Although Morton’s work has been confined to the Amish in Lancaster, he said it is likely the Amish who migrated to Ohio and other parts of the country have taken glutaric aciduria and other genetic diseases with them.

“Amish communities in Pennsylvania and around the country have genetic diseases that are unique to their group,” he said. “It’s a nationwide problem.”

He said the disease will also be spread by the 20% of the Amish population who leave the church and marry outside the order.