When Jayme Fralick’s daughter, Liliana, was born in October 2013, the first-time mom joined a lactation group at Yale New Haven Hospital’s Saint Raphael campus.
Liliana seemed like a happy, healthy baby. She had been born with jaundice, a yellow discoloration of the skin and eyes caused by high levels of bilirubin, a yellow-colored pigment, in the blood. But doctors assured her parents that the condition was common and should go away in a few weeks. She was gaining weight and eating well, and she passed her first few checkups with flying colors.
After a few weeks, though, Fralick began to notice differences between Liliana and the rest of the babies in her lactation group. “Lili was still on the yellow side, and she was very hungry all the time,” she says. “I thought maybe I wasn’t producing enough milk, but she was a chunky baby. She was definitely getting enough food.”
Liliana’s pediatrician said she was fine, and that she would “pink up” when she transitioned off of breast milk. (Jaundice can sometimes be caused by breastfeeding.) Fralick was not convinced. “In my mom gut,” she says, “I just knew something wasn’t right.”
When Liliana was 8 weeks old, one of the rotating physicians in Fralick’s lactation group noticed that Liliana’s bowel movements were drier and paler than they should have been. “I didn’t know it at the time, but the doctor was concerned,” Fralick says. “She emailed our pediatrician and told her to take a closer look.”
The pediatrician encouraged Fralick to take Liliana to Yale New Haven Hospital for a blood test and an ultrasound. The doctors there thought Liliana seemed healthy, too—until her test results came back. Then, Fralick says, things moved very quickly.
A scary diagnosis
Fralick and her fiancé, Matthew Moore, were introduced to Udeme Ekong, MD, the medical director of the Pediatric Liver Transplant Program at Yale Medicine. Dr. Ekong explained to the couple that something was wrong with Liliana’s liver and that they did not have much time to figure out the problem.
“Liver problems like Lili’s are usually identified a few weeks after birth, and it was getting late to be making a diagnosis in her case,” Dr. Ekong says. “The longer we waited, the more serious her condition would be.”
Dr. Ekong performed a biopsy on Liliana’s liver. It confirmed that she had biliary atresia (BA), a condition in which bile ducts in and around the liver do not drain properly, causing toxins to build up in the body. Doctors do not know what causes BA, or why some babies develop it, but they know that it causes liver failure—and, eventually, death—if left untreated.
Liliana needed a Kasai procedure, which is an operation to remove her damaged bile ducts and redirect the flow of bile from her remaining functional ducts into her intestines. But even in a best-case scenario, Dr. Ekong told the child’s parents, the results would be temporary. At some point, Liliana would need a liver transplant. In fact, 50 percent of babies with biliary atresia need a new liver before their second birthday.
The Kasai procedure worked for a few months. But over the next year, Liliana developed several serious infections. This was a sign that her immune system was functioning poorly. And tests showed that she had developed a tumor on her liver, which could become cancerous at any time.
Liliana was put on the national waiting list for a new liver. But there was no way to know how long she would have to wait. Because preference is given to patients with very advanced disease, Liliana might have to get much sicker before a liver became available from a deceased donor.
In April 2015, things took a turn for the worse. “I lifted Lili out of the crib one day, and I noticed that her ‘innie’ belly button had become an ‘outie,’” Fralick says. That was caused by fluid building up in her abdomen—a sign that her liver was starting to fail. She needed a transplant as soon as possible.
A few months earlier, Dr. Ekong had talked with Fralick and Moore about the possibility of finding a living donor for Liliana. Not all institutions provide that option, but Yale Medicine has one of the top living-donor liver transplant programs in the country. Although it sounds scary, the procedure is extremely safe for donors and recipients, Dr. Ekong told them. The one-year survival rate (a common medical statistic) for adult and pediatric liver transplants at Yale Medicine is 100 percent. A living donor transplant would also give Liliana the best chance at getting better.
Fralick is a vascular heart nurse and Moore is a physician’s assistant at Yale New Haven Hospital, but Dr. Ekong talked them through the process the way she would any parents with a sick child. “She made us feel like it was OK to take off our medical hats and just be Lili’s mom and dad,” Fralick said. “From the minute we saw her smiling face, it was easy to trust that the doctors were going to take care of everything.”
It was an emotional decision, but Fralick never had any doubts. “I knew that if I was a match, I was going to be Lili’s donor,” she says. “Whether she needed that transplant when she was 2 or when she was 22, I felt like it had to be me.”
Two surgeries, one goal
On July 14, 2015, Fralick and Liliana were admitted for surgery at Yale New Haven Hospital. After mom and daughter were given general anesthesia, surgeons made an incision in Fralick’s abdomen and removed a small part of her liver. They made a similar incision for Liliana, removed her damaged liver and replaced it with the segment from her mom’s. Then they reattached Lili’s blood vessels and bile ducts. Over the next few weeks what she had received would grow into a fully functioning organ.
When Fralick woke up, her doctors told her that the transplant had gone well. She was in a lot of pain over the next few days but was inspired when she heard that Liliana—who was recovering in the hospital’s pediatric unit—was already out of bed and walking around.
On the third day after surgery, Fralick was able to visit Liliana’s room. “The transplant team was very supportive and they encouraged me to spend time with her,” she says. “I think that was really important to both of our recoveries.”
Fralick was discharged after five days, Liliana after seven. Getting back to real life wasn’t easy. “I couldn’t lift her up because we were both still healing,” Fralick says. “We changed our habits. She’d sleep in our bed, we’d eat on the floor, we did anything we could to make things easier.”
The family also had to get used to giving Liliana several daily doses of medicine. Without immune-suppressing drugs, Liliana’s body could reject its new organ.
Fralick and Moore also have to be careful about Liliana’s compromised immune system, a side effect of the drugs. This means keeping her away from other kids who are sick, making sure she does not put dirty objects in her mouth and reminding friends and family to regularly use “germ juice,” their name for hand sanitizer.
“Socially, it’s been hard,” Fralick says. “We can’t go out to restaurants like a normal family or send her on play dates with kids who are sniffling and sneezing. When we run errands, we really try to just go to one place every day, because we don’t want to overload her immune system.”
They are dealing with one more complication, too. While tests show that Liliana’s intelligence is above average for her age, her language skills are delayed and her vocabulary is limited—a possible sign of cognitive damage from her months living with liver disease.
In many ways, Liliana is a healthy, happy toddler. A year after her surgery, she attended a summer camp and traveled to Michigan to visit with Fralick’s family. She sees a speech therapist, and her family hopes to start her in preschool in the spring. This fall the Make-A-Wish Foundation granted Lili’s wish to go to Disneyland and the San Diego Zoo.
Lili still sees Dr. Ekong every few months. And Fralick knows the staff at Yale Medicine is always there if she needs them.
“Any time I have a question, I can call the coordinator, and Dr. Ekong calls me right back,” Fralick says. “And any time Lili has a fever or anything else wrong, our pediatrician will loop her in so she knows what’s going on.”
Dr. Ekong is happy to have that role. “They’re a really lovely family,” she says. “I like all of my patients, but Lili is such a sweet kid. It’s been very special to work with them and to see her get stronger and healthier.”
Fralick is not sure what Liliana remembers about her surgery or how much she understands about everything they have been through together. But there is one thing she knows. “I talk to her a lot about how I have an ‘ouchie’ and she has an ‘ouchie,’” Fralick says, referring to their matching stomach scars. “She’ll kiss my ouchie and I’ll kiss hers, and we have an understanding of this special thing that we share.”