Amazing Kids, Amazing Stories: Tyler's Story

Pediatric Genetics and Metabolism

Amazing Kids, Amazing Stories: Tyler's Story

Tyler HallTyler Hall of Baraboo, Wisconsin will one day learn how he overcame odds that were heavily stacked against his survival.
 
Born in 2006 in Sauk City and transferred to Madison four days later, Tyler was ultimately diagnosed with life-threatening heart failure resulting from a very rare condition known as MCAD deficiency, a genetic disorder that substantially inhibits the body's ability to use fat as a fuel.
 
"MCAD deficiency is diagnosed in about one in 15,000 children," says Greg Rice, MD, a pediatric genetics specialist at the Waisman Center. "All Wisconsin newborns are screened for MCAD and 46 other disorders by the State Laboratory of Hygiene. Most babies diagnosed with MCAD deficiency present at 2 to 3 months of age and do not go into crisis before treatment begins.
 
"What put Tyler in such great jeopardy, however," Rice says, "was his presentation of MCAD deficiency just three days after birth. As a result, the accumulation of unmetabolized fat was causing his heart to fail. About a dozen cases have been reported throughout the world similar to Tyler's and most of those infants did not survive."
 
Essential to getting Tyler on the road to recovery at UW was a heart-lung bypass machine known as ECMO (extracorporeal membrane oxygenation). 

"Use of the ECMO circuit allowed Tyler's heart and lungs a chance to heal during a very critical period," says Tom Brazelton, MD, a children's hospital critical care pediatrician and medical director of the Pediatric ECMO Service.
 
"ECMO is essentially a partial heart and lung bypass machine that allows us to support the vital functions of the heart and lungs in dying infants and children - it is truly a last resort - and only available at children's hospitals that offer the highest level of care."
 
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A week later, Tyler was able to come off the ECMO machine and begin receiving a special formula orally. A month later, he was healthy enough to go home and his long-term outlook is excellent.
 
Brazelton, who has seen his share of remarkable cases in the Pediatric Intensive Care Unit, will have a hard time forgetting Tyler.
 
"Tyler is alive and, even with his diagnosis of MCAD deficiency, should have a normal existence," Brazelton says.