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'Miracle Baby' Survives 'Spiral of Death'

Posted on: Friday, 16 June 2006, 12:00 CDT

By Brittany Schoepp, The Wisconsin State Journal

Jun. 16--With a special heart bypass machine and the results of a newborn screening test, UW Children's Hospital doctors saved the life of a Baraboo infant suffering from a rare genetic disorder's even rarer complication.

Greg Rice, pediatric geneticist at the UW Waisman Center, said doctors eventually diagnosed Tyler Hall with medium chain Acyl-CoA dehydrogenase deficiency, a genetic disorder that causes problems breaking down some fats. The rare disorder occurs in about one in 15,000 children, said Rice.

Symptoms of MCAD deficiency normally appear in children ages 3 months to 2 years -- a period which Rice said usually gives doctors enough time to test for and treat the disorder. Most patients suffer from low blood sugar levels, which can be easily prevented.

The baby Tyler, however, had a disease that was taking an unusual path, one that would lead to what one expert called "a death spiral" and that had his parents making funeral arrangements.

Tyler developed a "very rare" complication from MCAD called lipid cardiomyopathy, due to a build-up of fat in his heart.

"The problems that Tyler had related to his heart have only occurred in about 10 to 12 children total -- ever," said Rice.

Children who experienced heart complications developed them within the first few days of life, not later on in childhood.

In those first few days of Tyler's life, his condition went from "perfectly healthy" at his April 11 birth at Sauk Prairie Memorial Hospital, to "life- threatening" a few days later.

After returning home, Tyler stopped eating, became lethargic and had a temperature down to 93.8 degrees. Heather Hall and her husband, Jason, took Tyler to the emergency room at Sauk Prairie and then to St. Mary's Hospital in Madison.

His heart stopped, and he was revived with CPR, but no one knew the source of the heart condition, so he was transferred to the UW Children's Hospital.

"It was just horrible," Hall said. "We just didn't know what to expect."

Tom Brazelton, UW Children's Hospital critical care pediatrician, said Tyler required frequent intervention via CPR because fat had built up on his heart and, because he did not have enough sugar, the acidity levels in his body had increased.

"We would provide better cardiac output, so then the acid would go down and he'd get better for a little while," Brazelton said. "But each time this cycle comes around, it's more vicious. This was the death spiral he was on."

Brazelton said doctors turned to "a true last resort," placing Tyler on an extracorporeal membrane oxygenation (ECMO) machine for six days.

"Without it, he wouldn't have lived," Brazelton said. The machine is a heart and lung bypass that puts blood through a thin, gas-permeable envelope to allow carbon dioxide and oxygen exchange in the blood.

"We assumed function of about 80 percent of his heart and we bypassed the need for his lungs, which were also very sick," he said. "The key was once we put him on ECMO, normalized pH and provided normal cardiac output, his heart was basically able to heal itself."

However, Brazelton said ECMO is "full of risk," with the possibility of clotting which could cause stroke, so Tyler's blood was kept very thin.

After Tyler was placed on ECMO, doctors received the results of the newborn screening tests, diagnosing Tyler with MCAD and finally illuminating the source of his heart problems.

"When we were able to provide him with the right nutrition, we were able to reduce the effects of the fat deposits in his heart and liver," Brazelton said.

Although both doctors said the ECMO machine and MCAD test helped save Tyler's life, both his family and doctors said he was lucky to have been born in Wisconsin -- ECMO machines are only at the highest level of children's hospitals, and some states do not include the MCAD test in newborn screening.

Nicole Hoffmann, director of the Capital Wisconsin division of March of Dimes, said state governments determine how many tests are included in the screenings.

According to Hoffmann, Wisconsin performs 46 tests, including 28 of the 29 tests the March of Dimes recommends. However, she said some states require as few as seven tests.

After his recovery, Hall said Tyler returned home May 10 -- the day before his father's birthday and the week of Mother's Day. Now, the family has a "very happy" baby.

"You can't even tell he was sick," she said. "He has a lot of energy, and he keeps us going."

Hall said her family has decided having another child would be too risky, with a one in four chance another child would have MCAD deficiency. But with a healthy baby now at home, she said it is nice just to have Tyler.

"He's just a miracle baby," she said. "Lots of people have been praying for him . . . and after all this, now you kind of believe in prayers."

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Copyright (c) 2006, The Wisconsin State Journal

Distributed by Knight Ridder/Tribune Business News.

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Source: The Wisconsin State Journal

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