Transfusion of hope

June 23, 2003|by Chris Copley

At first glance, a visitor can tell Jordan Lehman is no ordinary child. A plastic tube snakes out of his chest. His eyes close when bright lights are near. His speech is a series of grunts. He can't walk or crawl.

"Anything you can imagine a toddler doing, Jordan cannot do," says Bob Lehman, Jordan's father and pastor of Trinity Lutheran Church in Hagerstown.

Despite his obvious challenges, Jordan is considered a miracle baby. He was born with Tay-Sachs disease, a fatal condition that kills children within a few years of birth. Yet Jordan is alive and improving today because of an experimental treatment involving stem cells.


And because his parents and community joined hands to form a network of care and hard work.

An inherited disease

Tay-Sachs is a hereditary metabolic disorder, most commonly associated in North America with two ethnic groups; Jewish people of Eastern European ancestry; and Arcadians from Louisiana and the St. Lawrence River valley in Quebec.

When children are born with Tay-Sachs, their bone marrow will not produce an enzyme called hexosaminidase A, which the body uses to break down certain fatty acids in brain and nerve cells. These fatty acids accumulate and then destroy brain and nerve cells. Eventually, the entire central nervous system stops working, killing the child.

Bob Lehman says life expectancy for children with Tay-Sachs is 2 1/2 to 3 years. Jordan is 2 1/2.

He was born in December 2000 and joined his family in Charles Town, W.Va. Lehman says his son appeared typical after birth. He developed like most babies. But his progress took a dive after six or eight months.

"We knew there was something wrong, but we didn't know what," Lehman says.

When Jordan was examined a year after birth, his pediatrician had some concerns. Bloodwork and an MRI at Johns Hopkins Bayview Medical Center returned a definitive diagnosis: Tay-Sachs. Lehman says he and his wife, Jacqueline, were devastated.

Experimental treatment

At first, the Lehmans found no treatment. They searched the Web, contacted prominent children's hospitals, but found nothing.

Then, expectedly, Jacqueline Lehman's mother learned of another child with a metabolic disorder in Washington County who was receiving treatment from pediatrician Dr. Joanne Kurtzberg at Duke Children's Hospital and Health Center in Durham, N.C.

After days and days of tests, Jordan qualified for Kurtzberg's experimental stem cell transfusion program. But the next decision was not easy. Bob and Jacqueline Lehman had to choose whether or not they felt Jordan might benefit from the program - not just survive but live meaningfully.

"Dr. Kurtzberg promised he would survive," Bob Lehman says, "not just five or 10 years, but a full life. The quality of life question was solely ours."

A child with a metabolic disorder loses body function as the disease progresses. The child may lose so much body function that, even with successful therapy, some body functions - walking, perhaps, or hearing or muscle function - might be lost forever. The risk was that Jordan would survive Tay-Sachs, only to be disabled for the rest of his life.

The Lehmans decided to proceed with Kurtzberg's program.

"We erred on the side of hope," Bob Lehman says.

Rebuilding with stem cells

Kurtzberg is a pioneer in the treatment of children's diseases with blood from umbilical cords. For nearly 20 years, she has used stem cells found in cord blood to fight leukemia and genetic disorders.

The development of stem cells as an agent of healing is an area of exciting research. Stem cells are considered "master cells" of the body. They have the ability to differentiate - divide and specialize - into a wide variety of tissues.

Research is showing that stem cells can be used to replace defective bone marrow, to rebuild muscle or organ tissue and to treat a variety of life-threatening diseases.

The most powerful stem cells come from a fertilized human egg that has divided a few times. But harvesting these cells for research involves destroying a human embryo, a hotly debated method. President George W. Bush has strictly limited federal funding of research using stem cells from destroyed embryos.

Stem cells can be found in other sources, as well. Humans of all ages have stem cells in their bone marrow, for example, which differentiate into new blood cells of all types.

Another source is blood from umbilical cords that are cut when a baby is born. No embryos are killed when harvesting stem cells from cord blood, Kurtzberg points out.

"Cord blood is noncontroversial," she says. "It can be collected without risk to the donor - the baby - or the baby's mother. It is otherwise discarded after birth."

Blood banks around the country now collect and store blood from umbilical cords.

Kill in order to heal

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