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Two-year-old Amara Wismann-Espinosa circulates the house at a full sprint, popping the ball popper like it was her job. “You’d never guess she was sick,” says Nick Wismann, her dad. For the last couple of days she’s had diarrhea and a nasty cough. Amara catches a lot of normal childhood bugs, a side effect of the immunosuppressive drugs that keep her body from rejecting her transplanted liver.
Amara had biliary atresia, a progressive fibro-obliterative disorder affecting the liver and biliary system that results in end-stage liver disease and liver failure by age 2. Though relatively rare, it accounts for 50 percent of pediatric liver transplants in the U.S.
At 26 days old, Amara’s doctors at Children’s Hospital Colorado performed the “Kasai,” an operation to reestablish the flow of bile into her bowel. It’s a common operation for children with biliary atresia, and while it doesn’t cure the disease, it can help. Eventually though, most children with biliary atresia will need a transplant. Amara got one at 16 months old.
“Three days later, we noticed something wasn’t right,” says Nick.
Amara had developed a hepatic artery thrombosis, a blood clot in the artery that supplies the liver, a known complication of liver transplantation. Amara had spent months on the waitlist for her first transplant, and while her doctors at Children’s Colorado were doing all they could to keep her stable, they knew she’d need another transplant, very, very soon.
“That was the worst day of my life,” says Nick.
“I’m not a person who prays,” says Angelica Espinosa, Amara’s mom, “but I went to the chapel that night and I prayed for like three hours. I prayed to the Virgin of Guadalupe — I honestly don’t remember why. But I promised her I would start a nonprofit to help other families with biliary atresia, and that I would take Amara to Mexico to see the Virgin if she would let my baby live.”
Within hours, a liver came through. “That second liver was a miracle,” Angelica says.
In some ways, every organ transplant is miraculous. But transplantation is not without problems. No treatment is more invasive, and none as fraught with risk. Living with a transplant requires a cocktail of critical drugs and the constant, lifelong vigilance of a comprehensive transplant team.
In an ideal world, doctors would be able to treat the primary liver disease in a child like Amara and avoid the risks of transplant altogether. That day could soon come.
Researchers like Dr. Cara Mack, one of Amara’s pediatric hepatologists at Children’s Colorado, are exploring the root causes of biliary atresia. A drug that could treat the disease’s basic inflammation is currently in its Phase II trial at Children’s Colorado. “It’s too early to know if it’ll work,” says Dr. Mack, “but because it targets many features of the immune system, it has good potential to slow down inflammation.”
In the meantime, Angelica has made good on her promises. She and Nick took Amara to Mexico to meet the Virgin of Guadalupe within months of her transplant. They started a non-profit called Amara’s Smile to assist families of children with biliary atresia with the non-medical expenses and logistical problems that pile up during long hospital stays. They’ve already helped six families from as far away as Ecuador.
In many ways, Angelica’s promises to the Virgin have become a full-time job. She’s happy with that. The harrowing night she made them touched every aspect of her life. “When you go through something like that,” she says, “you never get over it.”
Read about Dr. Cara Mack's research on biliary atresia triggers and possible treatment.