From the moment Jordan and Hillary Weidner began dating seriously, the desire to build a family served as a baseline for their relationship. So when they eventually married in their hometown of Cincinnati about three and a half years ago, there was no question they would one day try to have a child.
“We had a conversation about it, and next thing we knew, we were having a baby,” Hillary, 29, a social media strategist, told FoxNews.com.
Hillary carried her and Jordan’s newborn baby girl, Bea, to term after what she described as an easy pregnancy. On New Year’s Eve 2014, the same night they met 10 years earlier at a local dive bar, the Weidners brought their firstborn home from the hospital.
“I was there for the delivery, and I remember the doctor showing me Bea and telling me it was a girl,” Jordan, 31, who works in commercial real estate, told FoxNews.com. “That was probably the most special day of my life— knowing that forever our family is complete, and the sense that we’re officially building something.”
Hillary and Jordan saw their joy shift to fear shortly after Bea’s two-month pediatric visit, when doctors diagnosed her with a rare condition called biliary atresia. In all cases, the mysterious condition, which has to do with a dysfunction of the liver’s bile ducts, requires an organ transplant. Untreated, biliary atresia is deadly.
Despite the risks, both Hillary and Jordan immediately raised their hands to donate their liver— the only organ that can regenerate itself. Hillary, whose liver turned out to be best match, went through with the procedure.
“Anybody that is a mom would understand, or a dad,” Hillary said. “You do everything you can to give your child a chance at growing and a chance at life. It’s instinct; you just do it.”
‘We were optimistic’
Bea was born jaundiced, or with yellowed skin, a sometimes-harmless characteristic that occurs in about 40 percent of births. At her pediatric checkup, the doctor noticed Bea also had yellow in the whites of her eyes and elevated levels of direct bilirubin, so she recommended Jordan and Hillary take her to Cincinnati Children’s Hospital Medical Center for further examination.
At Children’s, doctors took a blood draw and sent it to the lab to be tested.
“I didn’t think it could get any worse— watching them take blood from a little 2-month-old baby,” Hillary recalled, “but we were optimistic. Our mind didn’t go anywhere else than, ‘We put her on an antibiotic, and it will go away.’”
Doctors told them to come in early the next morning for an ultrasound, but when Weidners arrived home 20 minutes later, the hospital called and asked them to return that night, around 11 p.m.
A subsequent liver biopsy at the hospital confirmed Bea’s diagnosis of biliary atresia, a condition in which the bile ducts inside or outside the liver don’t have normal openings. Scientists don’t know what causes biliary atresia, but one theory is a viral infection triggers the inflammation. The challenge for doctors is there’s no single virus linked to biliary atresia, nor are there any overt symptoms for whatever supposed virus that may be, said Dr. Kathy Campbell, medical director of pediatric liver program at Children’s.
“Most of the time, it does not seem to be something that starts in utero or before the baby is born because the children are usually born healthy, they go home from the hospital in a reasonable time, they’re eating well, they’re gaining weight,” Campbell told FoxNews.com. “But sometimes in the first two months of life, there is inflammation in their bile ducts that leads to scarring and complete blocking of the bile ducts.”
Looking back, Hillary and Jordan said there were other signs Bea had been in poor health, like her seemingly insatiable appetite. Bile ducts carry bile from the liver to the gallbladder to aid in digestion. The liver generates bile, which helps digest fats and absorb the fat-soluble vitamins A, D, E and K. Bile also helps release toxins and waste from the body. Bea’s bile ducts weren’t working, so her body wasn’t fully absorbing those nutrients, causing her to always be hungry.
A subsequent intraoperative cholangiogram— whereby doctors inject contrast material into the gall bladder to analyze the bile ducts— further confirmed Bea’s diagnosis.
‘That uncertainty was always hard’
Biliary atresia occurs in about 1 in 40,000 people worldwide, and the Kasai procedure is standard treatment for the condition unless the patient has liver scarring, in which case doctors skip this step because trends indicate the surgery will be unsuccessful. During the Kasai, surgeons aim to bypass the bile ducts that have become scarred and closed off to re-establish bile flow so the bile can drain from the liver to the intestine. Doctors then usually watch patients two months postoperatively to see whether the procedure worked. Campbell said one-third of patients who receive the Kasai see full success, one-third see moderate success and need a transplant sooner rather than later, and one-third do not see any success.
Bea’s stool continued to turn white in the immediate months following the Kasai, meaning the surgery wasn’t successful for her and biliary atresia remained. Supplementary strategies to promote the procedure to work— like antibiotic and steroid treatment— also proved fruitless, meaning she would need a liver transplant as soon as possible.
“There was no reason that we could identify as to why the Kasai surgery wouldn’t work,” Campbell said. “It’s always devastating to our entire team when we feel like everything has been done correctly, and still we’re faced with a failed Kasai and a 3-month-old infant who now is going to need a liver transplant before they’re a year of age.”
The Weidners admitted that although the news was unhappy, the failed Kasai meant Bea would begin a seemingly faster route to recovery, as all biliary atresia patients need a liver transplant at some point in their lives anyway.
“That uncertainty was always hard for us,” Hillary said. “If [the procedure’s success] lasts for us every year, we’re going to say on New Year’s Eve every year, ‘Is this the year she is going to need a liver transplant?’”
‘What we needed to do to save Bea’
Bea was put on a waitlist for a deceased donor at the end of May, about two and a half months after her Kasai procedure and when she was 5 months old. In children age 11 and younger, a patient’s place on that list is determined by his or her pediatric end-stage liver disease (PELD) score, which is calculated by his or her height and weight, and how normal those values are for their age, as well as the child’s objective size and blood type. The calculation spits back a numerical value from negative 6 to positive 40, with the higher scores being higher priority for the next available transplant. Bea’s score was about 10, Campbell said.
“Like many things associated with liver transplant, it is a bit of a balancing act,” Campbell said. “As her physician I want to do everything absolutely to keep her healthy and to try to make her growth normal and her labs as good as can possibly get them. But at the same time, the worse her labs are [and] the more stunted she is as far as her growth is, the higher her score.”
To ensure Bea’s health as she waited for a deceased donor match, the Weidners fed Bea every three hours around the clock, mixing concoctions of formula to help her get the most calories out of every ounce of food she ate. When that wasn’t enough, they had doctors place a feeding tube through her nose and into her belly so that when she was asleep at night, she could continue to consume a sufficient amount of calories and maintain her body weight.
From the day the Weidners began talking about transplants with doctors at Children’s, they both expressed interest in donating to Bea.
“Some kids wait so long [for a transplant] that they’re in the ICU and they’re green as grass waiting for these organs,” Jordan said, “and these kids are sometimes even too sick to receive them when it’s their turn, and we weren’t going to let that happen.”
Jordan and Hillary went through initial tests to determine whether they were a proper match for Bea, and both were. But because Hillary’s liver was a better fit for Bea, she moved forward with the process.
But the Weidners’ decision to opt for a living donor wasn’t easy. For one thing, the couple had to put living wills together. Jordan recalled talking to Hillary in Bea’s nursery and asking whether she thought they were making the right choice. Living donations are known to be risky, and while Bea was in need of the surgery, Hillary was healthy and did not. For Jordan, Hillary going through the procedure meant he stood to risk both his wife and child.
“I don’t think we were grasping the full scope about what we were going to take on,” Jordan said. “We understood the risks and complications that could occur, and complications that could happen to our family, but at the end of the day, this is what we needed to do to save Bea.”
The testing and counseling required for Hillary to receive donor approval involved six weeks of tests including those in the lab, as well as imaging like X-rays and EKGs, and counseling to determine that Hillary was making the decision of her own volition.
“We used to think about living donors and approach them more aggressively, but the reason we don’t do that is we want people to process the operation. That’s where the idea of rushing people through with a workup is something we try to minimize to maximize patient understanding and safety of the procedure,” Dr. Greg Tiao, director of the liver transplant program at Children’s, who performed Hillary’s surgery, told FoxNews.com.
Tiao began Hillary’s surgery around 8 a.m., July 7, and finished around 1 p.m. From there, doctors transported part of her liver to Bea, who was out of surgery around 7 p.m. Both surgeries went smoothly, and Bea and Hillary recovered normally.
“The overall recovery was emotionally exhausting and physically exhausting,” Hillary said. “[But] it’s like when people have babies, they say they don’t even remember how badly it hurt because you did it for your child.”
‘We can do this’
Bea no longer has biliary atresia, but as a transplant patient, she’s at a higher risk of certain types of cancer like lymphoma in the short term and skin cancer in the long term. She’s also at a higher risk of infection due to her immunosuppressant medication, which she must take to prevent her body from rejecting the donated liver.
Immediately after her surgery, she was on upwards of 10 medications at a time, but today she takes about four. In a year, if all goes well, she’ll only have to take the immunosuppressive twice a day, Campbell said.
The Weidners said that through their trials with Bea’s health, they have drawn strength from their community, which jump-started a campaign called Bea Strong to raise funds for research on biliary atresia through online donations as well as retail. A local children’s store began selling apparel printed with the campaign’s namesake.
Earlier this year, the couple presented Cincinnati Children’s with a check for $37,000— all funds raised from their community as well as donors around the world. The hospital plans to honor Bea and the Weidners at an upcoming dinner to express their thanks for the contribution.
“We have such strong families and groups of friends,” Hillary said. “We have meals dropped off at our house three days a week from people. We have people knocking on our door. We can’t let them in [due to Bea’s necessary limited contact], but they’re there.”
The couple has also endured because they’ve leaned on each other, they said.
“There isn’t a day that we haven’t looked at each other and said, ‘We got this. We can do this,’” Jordan said. “At a baseline, we’ve looked internally at each other for that source of strength.”