Published April 24, 2013
Throughout Kelly Smith’s pregnancy with her daughter Matilda, everything went exactly as planned.
“I had this instinct that because she was conceived so easily and the pregnancy went really well, I kept joking she would be an easy baby – cause everything else was so easy,” Smith, 28, from Canton, N.Y., told FoxNews.com. “But of course, the beginning was rough.”
Just four or five days after being born, Matilda started to become very lethargic, and Smith found she had trouble waking her up to breastfeed. Even when she would get Matilda to latch on, her daughter would fall asleep right away – prompting Smith to worry about her nutrition.
Then just a few days later, Matilda began projectile vomiting after she would nurse.
“The first few times I thought it was just spit up, but it was a lot more than I had ever experienced with my son,” Smith said. “Then it continued to happen, and I became really concerned. I called my pediatrician who was out of town – who said we should take her into the ER as a precaution.”
Thinking their daughter perhaps had GERD or something similarly benign, Smith and her husband Tyler took 8-day-old Matilda to the ER of Canton-Potsdam Hospital in Canton, where doctors then sent her to Upstate Children’s Hospital in Syracuse. There, blood tests revealed the problem was much more serious: Matilda was suffering from acute liver failure, and all signs pointed towards her needing a liver transplant.
After the diagnosis, the doctors sent Matilda and her family to Mount Sinai as quickly as possible. The four-hour drive from Syracuse to New York City was one of the longest drives of Smith’s life.
“It was horrible; I was in complete shock,” Smith said of accepting her daughter’s illness. “My husband and I, we didn’t have a lot to say to each other. We just sat in each other’s presence… I kept saying, ‘But I love her,’ as if that would fix everything. I just wanted it to get better.”
When Matilda arrived at Mount Sinai, she was placed in the pediatric ICU and was connected to life support to help strengthen her liver function. At this point, Matilda had turned a dark shade of yellow, and a CT scan revealed swelling in her brain – a very troubling development.
At the hospital, Smith and her husband met Dr. Ronen Arnon, the director of pediatric liver disease and transplantation at Mount Sinai Medical Center, who would oversee Matilda’s treatment for the next month and a half. Arnon and his team diagnosed Matilda with neonatal hemochromatosis, an extremely severe form of liver disease, which results in excessive iron deposition in the liver. The underlying mechanisms for the condition are still not very well understood, and the disease is so rare that no prevalence rates have been reported.
Without life support to prevent further deterioration, babies with this condition can die in just a few days.
Playing the waiting game
Arnon said Matilda was so sick that she was placed on the liver transplant list the day after she arrived.
“This list has priorities according to the severity of liver disease,” Arnon told FoxNews.com. “Each child gets a specific priority, and we call it status 1 when they have acute liver failure. These critically ill patients are above the other children with chronic liver disease. So first we listed her, and we put her as status 1.”
Then came the waiting game, which Arnon said was particularly tricky, because the physicians needed a donor who was more or less the same size as Matilda. During her stay at the hospital, Matilda’s weight had only remained around seven to eight pounds.
“Sometimes you can take parts of the liver or half of the liver (from the donor), but because she was so small, we decided to wait for a whole liver,” Arnon said. “During this month, we gave her support for blood pressure, a lot of blood products because the liver could not synthesize the proteins in the blood, and we gave her glucose to the veins. She was on the ventilator as well.”
As the doctors and Smith family waited for word on a possible donor, many physicians explained to the family the risks involved with doing a transplant on such a small child.
“They explained that of the 40 percent that make it past initial surgery, that (75 percent) make it to age 5,” Smith said. “So that gave us an idea that it’s not a quick fix; it’s something that takes a lot of effort as a family to continue to support each other and move forward, so she continues to be safe.”
About a month after Matilda had arrived at Mount Sinai, doctors finally found the perfect organ. Up until then, Matilda had been having severe complications: She had contracted a urinary tract infection, she need fresh frozen plasma several times a day, and her blood pressure had dropped to just 20/10.
At what seemed the last possible moment for Matilda, doctors performed her liver transplant surgery – what Arnon said was a very challenging procedure.
“It’s become a very technical issue,” Arnon said. “The liver vessels are very small, and it’s really unusual to transplant such small babies."
After just six hours of what was meant to be a 12-hour surgery, doctors told Smith and her husband that the surgery had gone as smoothly as possible. They told her that Matilda was very lucky to have undergone the operation when she did, because at that point, only 1 percent of her liver was functioning.
Now, it’s been nearly five months since Matilda’s surgery and Smith said she is recovering “beautifully.”
“Even just days after the transplant, her coloring came back, and she looked so much happier,” Smith said. “You could tell she just felt better. She is continuing to thrive; she’s sitting up and hitting all the typical baby milestones.”
Matilda’s doctors still don’t fully understand what caused her liver failure, but they believe that she also may have contracted a virus after she was born, creating a “perfect storm” of illness. Even though the mystery still scares Smith, she is incredibly grateful to the doctors at Mount Sinai for giving her daughter a second chance at life.
“I just know that her life was created to go through this journey; it’s part of who she is,” Smith said.
To follow Kelly Smith's updates on her daughter Matilda and her family, visit her blog: http://www.cloudydaygray.com.