The Colgate Scene
March 2004

Saving Jonah
Alumnus starts fund to fight rare disorder afflicting son

Because his liver cannot properly process glycogen into glucose, two-year-old Jonah Feldman must be fed a mixture of cornstarch and formula through a feeding tube.

At first glance, Jonah Feldman appears to be a normal two-year-old; a pint-sized bundle of energy and charm with sparkling eyes that seem to laugh on their own.

But what sets Jonah apart from other children becomes quickly apparent a few moments later when his parents prepare to feed him. While David Feldman '89 tries to hold the restless, fidgeting child still, his wife, Wendy Becker Feldman, attaches a large syringe to a feeding tube protruding from their son's abdomen. Once the attachment is secure, she slowly compresses the piston and a pale yellow mixture of cornstarch and formula flows through the tube into Jonah's stomach. Within a minute or two, she detaches the syringe, cleans the feeding tube and closes it, and walks into the kitchen to clean up and prepare for Jonah's next feeding. While Jonah is awake, the Feldmans will repeat the process every two and half hours, and every four hours when he is asleep. A missed feeding could have tragic consequences, which is why the family's babysitters have to be trained how to feed Jonah.

"I would much rather talk about being in business with my father and brother," said David Feldman, a dentist who shares an orthodontic practice with his father Barry '62 and brother Jonny '90 in their hometown of Cheshire, Conn. "But this story is more important right now."

Jonah is one of a handful of children worldwide suffering from glycogen storage disease (GSD) type 1a (there are 11 different forms of the disease), a genetic condition in which the liver is unable to properly metabolize glycogen into glucose. Glucose powers every cell in the human body and is an essential fuel for brain function, the respiratory system, and for maintaining phosphate balance. Children afflicted with GSD type 1a cannot properly release stored sugars when blood glucose levels start to dwindle. This in turn creates severe side effects, including hypoglycemia (dangerously low blood glucose levels), a condition known as acidosis or acidotic shock, respiratory distress, and enlargement of the liver. More than half of the children with GSD type 1a develop liver and kidney tumors. Hypoglycemia can cause brain seizures, which can lead to coma and death.

The Feldmans must closely monitor their son for hypoglycemia through needle sticks with a glucometer. Hypoglycemia is kept at bay through the administration of large doses of complex carbohydrates (uncooked cornstarch is a natural glucose polymer) through that surgically placed feeding tube. The tube is necessary for all feedings. Children with GSD must be fed so often through the feeding tube they can have problems ingesting food by mouth. Jonah must undergo intensive therapy to relearn sucking, swallowing, and even speech patterns.

What did I do?
The Feldmans learned of Jonah's condition a few days after he was born in October 2001.

"It was Jonah's low birth weight, which was related to Wendy's high blood pressure during the last trimester, which eventually led to his diagnosis," David Feldman said. "They did a glucose test, which is standard for every low-birth-weight baby, and the numbers just didn't jibe with what the baby was doing. They just kept ordering different tests and narrowing the list down of what could be a problem or just a potential anomaly. They finally transferred us over to Yale-New Haven Hospital because they suspected Jonah had glycogen storage disease, which was confirmed later by DNA tests."

Because of their health training (Wendy Feldman is also a dentist), the Feldmans understood what was at stake for their son. Their initial reaction was one of sadness, David Feldman said.

"I stood over his crib and wondered `What did I do?' I blamed myself for giving this child not a leg up, but a leg down," he recalled. "I felt bad knowing what he faced for the rest of his life. I cried to him and said, `I'm sorry I did this to you.'"

The Feldmans subsequently discovered that they each carried the genetic trait for GSD, which is carried by one out of 68 Ashkenazi Jews. (The Ashkenazim are descended from Jews who settled in Germany during the ninth century.) The Feldmans also learned there wasn't much research being conducted into this extremely rare disorder. GSD is regarded as an "orphan" disease, the Feldmans said, because it is so rare there is almost no corporate sponsorship of research. Jonah's long-term prognosis is uncertain due to a lack of data and because so few GSD children reach adulthood.

Undaunted, the Feldmans decided to take matters into their own hands and established a foundation in 2002 with the assistance of family, friends, and others impacted by the disease, The Children's Fund for Glycogen Storage Disease Research, Inc. (www.cureGSD.org).

"We set up the foundation to be proactive," said David Feldman. "Hopefully, GSD is closer to being cured today than it was yesterday."


David Feldman '89 with his sons Jonah and Ryan, who does not have glycogen storage disease. Despite the fact that Jonah must be fed every two and a half to four hours, Feldman and his wife, Wendy, want to make their son's life as normal as possible.

Exciting times ahead
From the start, friends, relatives, and the families of GSD children stepped forward to contribute. Wendy's aunt produced an 18-minute documentary, Life by the Clock, which looked at the impact of GSD on four families, including the Feldmans, to spread awareness of what life with the disease is like. David's parents turned their fortieth wedding anniversary party into a fundraiser with the theme "What a Match!" In lieu of gifts, the senior Feldmans asked the invitees to donate to the Children's Fund for Glycogen Storage Disease Research and promised to match every donation. The party raised more than $55,000.

Eight friends and relatives, including Steve Metro '90, started a racing team called Team CureGSD, whose members raised more than $85,000 (including $36,000 raised by Wendy Feldman) by running in three marathons last year. As of mid-January, the foundation had raised more than $600,000.

The Children's Fund for Glycogen Storage Disease Research also issued its first grants in 2003, awarding $50,000 each to researchers at Children's Hospital in Boston and the Duke University Medical Center. (The foundation hopes to award at least one $50,000 grant annually.) Meanwhile, Alexion Pharmaceuticals (a Cheshire-based company that works with orphan diseases) has agreed to begin working on GSD type 1. In the spring, the foundation is co-sponsoring a one-day GSD symposium that will include medical experts in areas such as gene therapy, stem cells, and hepatocyte (liver cell) transplantation to discuss potential treatments and cures.

"We are very excited about the possibilities that can come out of an event like this," said David Feldman. "I am also currently in contact with doctors at Hadassah Hospital in Jerusalem about a [potential] joint venture. We are working with a Washington, D.C. law firm to help obtain government funding, and many other individuals and families have contacted us to take a more active role. There are exciting times ahead."

While getting the foundation off the ground, the Feldmans added to their family with the birth of a second son, Ryan, who does not have GSD.

"David and I aren't moping around; we're going about our lives. Having another baby definitely helped the situation, so that we're not just focused on Jonah," said Wendy Feldman. "I don't want him to be treated differently than any other kid. He is doing so well developmentally. As he gets older he will know exactly what he is going through, which is a good thing, but it could also be hard for him. I worry a lot about what is going to happen when Jonah does go to school. Are the kids going to make fun of him, because he has a tube in his stomach or because he has to drink his cornstarch? David and I will make sure we send him to school with as much self-esteem and confidence as possible, so that he can function successfully in the world."

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