March 26, 2010

Youngest liver transplant patient beat long odds

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Physical therapist Greta Roof and 5-month-old Rijac Armstrong look at their reflections in a mirror during a recent therapy session. (photo by Susan Urmy)

Youngest liver transplant patient beat long odds

Rijac Armstrong was born five weeks prematurely, and at 11 days old he was transferred to Vanderbilt, where he was diagnosed with a rare condition called neonatal hemochromotosis (NH).

The often deadly disease is an accumulation of toxic levels of iron in the liver and other organs. He was not responding appropriately to the medical therapy and due to ongoing liver failure, would require a liver transplant to survive.

William Walsh, M.D., professor of Pediatrics and chief of Nurseries at the Monroe Carell Jr. Children's Hospital at Vanderbilt, said Rijac's case was teamwork at its best.

“This baby was a very unusual case,” recalled Walsh. “He actually presented to the NICU (Neonatal Intensive Care Unit) with the complaint from his referring hospital that his scrotum was swollen. From there the team carefully evaluated the possible causes, which eventually pointed to liver dysfunction and then, finally, with the assistance of our wonderful GI team, concluded that he had NH.

“This was truly a team effort and his successful outcome is due to the persistence of the team in not being satisfied until they understood his disorder completely.”

On Nov. 8, 2009, five days after he was placed on the transplant waiting list, Rijac received a new liver. The date is a special one for his mother, Kim Destephano and father, Robert Armstrong — it was her original due date.

Rijac also made history on that day, as he became the youngest and smallest patient in Tennessee to receive a liver transplant.

“We were not expecting this to happen so soon,” said Destephano. “We were told it could be six to nine months for a pediatric liver, and finding one to fit our son was going to be particularly difficult. And all the while he was getting sicker and sicker. It was a really tough time.

Because the bigger liver of the donor was transplanted into 11-day-old Rijac, his abdomen wall was left partially open to allow his body to adjust to the larger-sized organ. Over the course of three to four weeks, the

Rijac Armstrong returns to Vanderbilt every week for physical therapy since becoming Tennessee’s youngest liver transplant patient last November. (photo by Susan Urmy)

Rijac Armstrong returns to Vanderbilt every week for physical therapy since becoming Tennessee’s youngest liver transplant patient last November. (photo by Susan Urmy)

“He spent a month in the PCCU (Pediatric Critical Care Unit). Each day or so they would slowly stitch up his belly. The liver was so big for his little body.”

Beau Kelly, M.D., assistant professor of Surgery and surgical director of Pediatric Liver Transplantation, performed the operation, assisted by surgical resident Andrew Murphy, M.D.

He recalled the difficulty explaining to Rijac's parents that their son fit into the highest waitlist mortality group.

“We were hopeful, but we certainly had to brace the family for the reality that their son might die without an opportunity for transplant due to the low likelihood of getting a size-matched organ for him and the high percentage of children with NH who succumb to the multisystem organ failure.

“In the pediatric population, especially in neonatal patients, dying on the waitlist is an unfortunate reality.”

And because NH was acquired from his family, living-related partial liver donation was not an advisable alternative.

The only choice was to wait for a pediatric donor.

“He was transplanted on his projected date of birth — what a gift,” said Kelly. “His life will be full of extra challenges, but Rijac's prognosis is good. He was discharged from the hospital six weeks after transplant and is gaining weight and beginning to meet some of his cognitive and developmental milestones.”

Today Rijac is thriving, said his mom. And she owes it all to Vanderbilt.

“We have the very best team at Vanderbilt,” said Destephano. “I couldn't ask for better people to be in our lives and taking care of Rijac. I have no worries that he is getting the best care.

“If Vanderbilt wasn't in our lives, he wouldn't be here today. I am forever grateful and thankful to them. Rijac is a miracle baby and we couldn't be luckier people to have such a great baby,” said Destephano.

Today Rijac is 5 months old and takes nine medications, five times daily. His mom said he will have to take a couple of medications for the rest of his life. He returns to the hospital for weekly physical, occupational and speech therapies and is seen monthly in the pediatric liver transplant clinic.

“In our culture, we hardly ever laud our smallest heroes,” said Kelly. “His triumph through nearly insurmountable odds has been inspirational to our team and the hospital staff.

“It is this level of dedication and expertise [that was provided to this family] that will allow Vanderbilt to continue to excel in providing life-saving care to even the smallest of patients with end-stage liver disease.”