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Liver Care Against the Odds, a Transplant Works Wonders
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Against the Odds, a Transplant Works Wonders

The infectious smile of Josh Buller, an active and fun-loving 16 year old, struggled to emerge for most of his childhood. This young boy spent the first 15 years of his life battling a rare disease called Arginiosuccincaciduria, better known as ASA.

What seemed to be a normal pregnancy and delivery quickly vanished three short days after Josh’s mom, Jennifer McElroy, gave birth.

"Josh became very irritable, screaming almost nonstop and vomiting up every feeding," Jennifer said.

On the fourth day, Josh barely woke up and was grunting with each breath he took. Jennifer took her son to see a doctor. Once in the pediatric intensive care unit at a local hospital in Wichita, Josh fell into a coma.

"We spent two days in the PICU and were told there was a slim chance he would live," Jennifer said.

After being put on a ventilator for respiratory support and undergoing multiple tests, doctors found Josh to have dangerously high levels of ammonia in his blood.

"His ammonia levels topped out over 2,000, and the high end of normal is typically around 32," Jennifer said.

A Diagnosis

Doctors diagnosed Josh with ASA, which is an inherited disorder that causes ammonia to accumulate in the blood, affecting only one in 70,000 newborns.

"Josh didn’t have machinery in his liver to break down ammonia, which was causing brain swelling and pressure and had the potential to cause permanent brain damage," said James Daniel, MD, Director, Liver Care Center and Chief, Section of Hepatology and Transplantation at Children’s Mercy.

Josh and his family learned to cope with this disease for the first 15 years of his life, but it was no easy feat. The ammonia build up caused him to be volatile towards family members, classmates, and in public settings. These multiple episodes of high ammonia levels required Josh to be emergently hospitalized and started on an IV to bring his ammonia levels into a normal range.

"Josh was in the hospital for a total of 29 days during his first episode. The next 13 years that followed resulted in a few yearly episodes that required hospital stays to stabilize his ammonia levels," Jennifer said.

These episodes led to central nervous system damage, developmental delays, severe behavioral problems and Josh had to be put on a strict diet of little to no protein.

"We felt like we were running out of hope. He kept having longer, more extensive episodes and we knew there had to be a change," Jennifer said.

New Possibilities

Josh’s mom decided to dig deeper into the possibility of Josh having a liver transplant. A formal evaluation was then done by the Children’s Mercy transplant team. A decision was difficult, due to the rarity of the Josh's condition.

"There has only been one published report of a successful liver transplant for ASA," Dr. Daniel said.

Josh’s mom struggled with what to do, but knew a change had to be made to save Josh. Despite the uncertain outcomes of ASA liver transplants, the decision was made for Josh to be put on the transplant list.

Receiving a Gift

On March 1, 2013, Josh received a generous gift of life from an organ donor and has done extremely well after his transplant. He was discharged 11 days after surgery and has required no further hospitalizations. The transplant reduced his medications from 13 to 6, with the hope of removing two more soon. He has also gained 13 pounds and grown nearly two inches during the last year.

"Josh’s teachers have said that they can now accomplish more with Josh in 45 minutes than they could in a whole semester before Josh received his liver transplant," Jennifer said. "He also has a new bond with his siblings, we have a renewed sense of hope and it is great to see him progressing, not regressing."

The transplant was a true success and is shown through Josh’s remarkable improvements physically, mentally and emotionally.

 

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