Ian Thomas's Christmas gift to infant daughter Luca: Part of his liver

Luca Thomas, with parents Katie Frieder and Ian Thomas, was born with a genetic disorder.
Luca Thomas, with parents Katie Frieder and Ian Thomas, was born with a genetic disorder.
Washington Post Staff Writer
Tuesday, January 4, 2011; 7:02 PM

Little Luca Marie Thomas, just 4 months old, needed a new liver. Doctors had known since she was 2 days old that a rare genetic defect had left hers unable to process nitrogen. She'd been placed on the national transplant list immediately - high on it, given her great need - but a matching organ just wasn't becoming available.

That left Luca's closest relatives: first-time parents Katie Frieder and Ian Thomas. It was too soon after Katie had given birth for her to donate. That left Ian.

Of course, you only have one liver. But the liver has excess capacity and it regenerates. Doctors could take out Luca's liver and replace it with part of Ian's.

But the liver is a complex organ and, from a transplant surgeon's point of view, a maddening one. Spongy and shot through with tiny blood vessels - extremely vascular, in medical parlance - it does not present the relatively tidy package of a kidney.

"This is not something you do lightly," said Marshall Summar, chief of the division of genetics and metabolism at Children's Hospital. "We prefer to use a donor organ [from a cadaver] simply because there's less risk to the family member, and also the rejection rates are really not that different."

There is a risk of complications from any surgery. It would be greater for Luca but not nonexistent for Ian. The ultimate complication is death.

Said Katie, "We went through the conversation: What if we lose her and something happens to you, what are we going to do?"

Ian was adamant, telling his wife, "If we don't move forward with me being her donor and something happens to her, how are we going to live with ourselves?"

Ian and Katie are both carriers of a urea cycle disorder called carbamoyl phosphate synthetase type I deficiency (CPS-I), something neither knew. As a result, Luca was prone to a dangerous buildup of ammonia in her bloodstream and around her brain, something that was hard to predict.

"There wasn't a clear indication when she was having an issue," Katie said. "It was very subtle things. She would be fussy. Or she would take a little longer of a nap. Those were my clues: She slept half an hour longer. Is she just sleepy today, or do I need to take her to the hospital?"

Said Summar: "Luca was starting to show us. Even with everything we were doing, her ammonia was drifting up. We knew it was time."

Children's partners with Georgetown Hospital for liver transplants, and on Dec. 20, Ian entered one operating room at Georgetown, and Luca entered another. Georgetown's Thomas Fishbein oversaw the procedures that transferred the left lateral lobe of the father's liver into the daughter.

Ian came home from the hospital after a few days. Luca is still there. Although her new liver has performed admirably, she was beset by other complications. But she is making progress and should go home later this month.

Ian, 30, works for a national insurance company. Katie, 28, was about to enter her final year at the University of Maryland's law school in Baltimore, where the couple lives. "I was very much a control freak before I had her," Katie said. "That's kind of gone out the window, because every day is something new."

Something new and now something hopeful.

A helping hand

My hope? That you will donate to the uncompensated care fund at Children's Hospital. That's the money that pays the medical bills of underinsured children. Our campaign ends Friday, and we've raised $255,560.57 toward our goal of $400,000. Send a check or money order (payable to Children's Hospital) to Washington Post Campaign, P.O. Box 17390, Baltimore, Md. 21297-1390. To donate online with a credit card, go to www.washingtonpost.com/childrenshospital or call 301-565-8501.

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