When Lucas St. Onge blew out the five candles on his birthday cake, he made just one wish. He didn’t ask for ninja turtles, a T-ball set or an Xbox — the only thing he wished was to be just like any other kid.
“It was a gift I couldn’t give,” says his mom, Heather.
On that same day, last May, he got his wish.
“He got a liver, stomach, pancreas, intestine and spleen — the five organs he needed to help him become healthy and happy,” Heather says.
Heather was 18 weeks pregnant when she and her husband, Anthony, learned they were about to face a myriad of medical challenges with their unborn child. To what extent remained unclear, until the day Lucas was born.
An emergency surgery to repair multiple intestinal blockages left him with short bowel syndrome, or simply “short gut,” a rare condition caused by the loss of a functioning small intestine. It meant Lucas would never be able to eat or digest food.
“I was told to take him home and to let him live out his days,” Heather says. “But I wasn’t willing to accept that.”
Lucas was transferred to Boston Children’s Hospital for a second opinion.
SCID and a bone marrow transplant
Five days after arriving at Boston Children’s, the Immunology Program team diagnosed Lucas with severe combined immunodeficiency (SCID), a rare genetic condition in which a child’s immune system is so compromised that a common cold can kill them.
“I had never even heard of SCID before,” Heather says. “It was terrifying.”
Almost every child with SCID is treated with a stem cell transplant, also known as a bone marrow transplant. It is the only available treatment option that offers a permanent cure. Stem cells are administered through an IV, similar to a blood transfusion. They then become healthy white blood cells that essentially build a whole new, functional immune system for the child.
Lucas’s team at the Dana-Farber/Boston Children’s Cancer and Blood Disorders Center began preparing for the transplant by identifying a donor. In Lucas’s case, the best match turned out to be his 5-year-old brother, Noah.
“It was pretty scary,” says Noah, who is now 11. “But I wanted to do it for my brother.”
On Aug. 30, 2011, Noah underwent the procedure to harvest his bone marrow. Lucas, just three months old at the time, received his stem cell transplant later that day.
Bittersweet homecoming for boy with short bowel syndrome
One month following his stem cell transplant, Lucas arrived home for the first time since he was born. Life had changed dramatically.
Over the next two years, Lucas relied on continuous intravenous feedings to maintain normal nutrition. In addition, he required regular infusions to keep his immune system healthy. But even so, Lucas was still a very sick boy and had multiple recurring infections. The stem cell transplant had helped with his SCID, but he was still dealing with complications from short bowel syndrome, parenteral nutrition (PN) and motility issues — resulting in the need for a multiple organ transplant.
“Because of his repeated infection, PN and poor-bowel function, his liver was getting sicker,” says Lucas’s doctor Dr. Rima Fawaz, medical director of the Boston Children’s Intestine and Multivisceral Transplant Program. “When the bowel doesn’t work, you can do PN for life, but you can’t live without your liver.”
Lucas had been evaluated for transplant before, but he wasn’t healthy enough, and the risks were high. He was three now — bigger and stronger, yet still living on borrowed time. He was ready to be listed for transplant.
“Intestinal and multivisceral transplant is a big deal,” Fawaz says. “You don’t throw this on someone who is doing well. You do it when it is the only viable option.”
Risk of rejection vs. risk of infection
Any person who receives an organ transplant has to take immunosuppressant medications to prevent the immune system from attacking the new organs and causing rejection. From a surgical perspective, a five-organ transplant is routine for Boston Children’s pediatric transplant surgeons, but Lucas’s situation was unique because of the risk of rejection and infection.
“He was born with an immune deficiency syndrome, he had a bone marrow transplant — so he had the circulating cells from another donor — and now on top of that we’re going to introduce a third set of cells with transplant,” says Lucas’s transplant surgeon, Dr. Khashayar Vakili. Vakili performed the nine-hour surgery along with Dr. Heung Bae Kim, director of the Pediatric Transplant Center.
Finding the right balance is critical, and what Vakili calls “a dangerous endeavor.”
“If we immunosuppress him too much, he’s going to be at a high risk for infection, which can be life threatening, but if we don’t immunosuppress enough, we risk rejection,” he says. “So, we work closely with the immunologists and stem cell transplant specialists to sort this out before transplant.”
Five organs on his fifth birthday
For 20 months and six days, Lucas’s parents waited anxiously. And, as fate would have it, the call came on his fifth birthday. Lucas was going to receive the five organs he wished for.
“My wish came true?” he asked his mom. “Who do I need to kiss first?”
It’s been a little more than a year since Lucas’s transplant, and he buzzes about like the Energizer bunny.
“He is doing wonderfully,” Heather says. “He plays T-ball, he is learning piano, he is climbing, and growing and gaining weight. Watching Lucas eat is like watching a baby laugh for the first time. It is simply amazing.”
Lucas gives a tour of his bedroom. It’s full of all the trappings of a typical six-year-old boy — a collection of Ninja Turtles, signed baseballs and hockey pucks, and posters of Captain America and the Boston Bruins. But there, amidst the chaos and clutter, is a discreet reminder Lucas is far from typical. He points to a stuffed Boston Bruins bear, carefully placed on the foot of his bed, and says, “His name is ‘Dr. Vakili.’”
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