In February 2018, Amy and Nick Winkler welcomed their healthy twin boys, Logan and Connor into the world. Amy recalls “feeling a huge sense of relief when both boys were born healthy and without any complications. Nick and I were just really excited to be parents and to move forward with this next chapter in our lives.”
Like many newborns, Logan and Connor experienced mild jaundice after birth. As the weeks passed, Amy and Nick noticed Connor’s jaundice appeared to resolve, but Logan’s skin and eyes still appeared yellow. At nine weeks when Logan’s jaundice still hadn’t gone away, Amy and Nick grew concerned.
Amy remembers, “The whites of Logan’s eyes had a yellow color to them; they weren’t bright white like his brother’s, and like you would expect to see in a healthy infant. With each week that passed, and Logan still looking jaundice, I knew something wasn’t right.”
Amy’s instincts were correct. Bloodwork ordered from a pediatrician’s office revealed that a key indicator called bilirubin was elevated – a classic sign that Logan’s liver was not properly functioning.
Given Logan’s age and lab results, his pediatrician said a diagnosis of biliary atresia was likely. Biliary atresia is a rare pediatric liver disease in which the bile ducts do not develop properly, eventually leading to liver failure. Approximately, one in 18,000 babies are diagnosed each year with this condition, which has no known cause or cure. Amy and Nick were told to take Logan to a local hospital right away, as he would likely need surgery immediately, but even then, it may not be soon enough to help their son.
A week later, after a series of additional tests at a local hospital including a HIDA scan – an imaging procedure used to diagnose problems of the liver, gallbladder and bile ducts – Logan had his first surgery at 10 weeks old which confirmed his diagnosis of biliary atresia.
The operation Logan had as an infant is called the Kasai procedure, which attempts to establish a new path for bile to drain from the liver in place of the missing bile duct. A successful Kasai procedure causes the child’s jaundice to improve, buying important time for the child to grow and develop normally. The liver is not completely fixed though, and most patients with biliary atresia do end up needing a liver transplant at some point in their life.
At the time of Logan’s Kasai surgery, the surgeon explained to Nick and Amy that given the timing of his surgery, and potential progression of the disease, the surgery was only successful about one third of the time; with the remaining two-thirds being temporarily successful or not successful at all. While the odds were not in Logan’s favor, it was the only option to prevent liver failure, and potentially delay the need for a liver transplant.
Unfortunately for Logan, two months after his operation, Logan’s condition was not improving, and doctors concluded the Kasai was not successful.
For those like Logan in whom the Kasai operation is not successful, essentially all of them will need a liver transplant before 2 years of age to survive. One of the most important challenges for children with liver failure is nutrition. It is common for them to need–as Logan did–formula through a tube that goes through the nose down into the stomach. And while getting them this extra nutrition is critical, it can cause side effects of its own such as bloating and diarrhea. The buildup of bile in the body also causes other symptoms such as progressive jaundice and severe itching. These progressive symptoms diminish the quality of life of these young children, and place enormous burdens on the family and caregivers.
They were immediately helpful, Nick and I knew Logan was in good hands.
At this point, Amy and Nick knew they needed a team that specialized in pediatric liver transplants and all the intricacies of helping an infant like Logan. And that’s when they were referred to Michigan Medicine.
“In July of 2018, we started working with Michigan Medicine’s transplant team. And they were immediately helpful,” says Amy. “Nick and I knew Logan was in good hands.”
As the summer progressed, Logan’s liver continued to deteriorate. By fall, it was clear Logan would likely need a transplant within six months, and so the team proceeded with the transplant evaluation process. Logan was officially placed on the national waiting list in January 2019.
Liver transplantation is the only curative therapy for patients with end-stage liver disease, but it is a complex procedure with significant risks and potential complications. The decision to proceed to transplant should only be made when it is clear there are no other options. Furthermore, the number of patients waiting for transplant is far greater than the number of organs available for transplant. Many patients must wait for months to receive a donor organ, during which time they become more ill. Living donation allows children to be transplanted as soon as the donor and recipient are ready for surgery when patients are less ill and more likely to recover successfully.
Of the many questions Amy and Nick had for their Michigan Medicine team, the most pertinent concerned how Logan would receive a new liver. They discovered that, unlike other organs, the liver completely regenerates and that healthy individuals can sometimes donate a portion of their own liver to someone in need, known as a living donor liver transplant.
“Naturally, my husband and I would do anything to help our son, but we discovered we weren’t the right match for Logan. My sister-in-law was a potential match, but was still not the right fit,” says Amy.
“When you’re put on the transplant list, you’re given a score of how sick you are. The higher the score, the more likely you are to receive a donor organ from the waiting list,” explains Amy. “I think this was the toughest part, waiting. Knowing Logan would essentially have to get worse before he could get better.”
As Logan was listed with a fairly high PELD (Pediatric End-stage Liver Disease) score, which is used to estimate disease severity and determines placement on the transplant waiting list, Amy and Nick were told to be ready at any time for the call that would inform them a liver had become available. But, four months later with no phone call or living donor matches, they were beginning to lose hope.
Then something happened that neither Amy, her husband, or even most medical professionals could ever expect. Thanks to a partnership with a hospital in New York, a living donor became available for Logan that was a perfect match.
Although University of Michigan Health has performed pediatric living donor liver transplants since 1996, these complex procedures remain uncommon. For that reason, surgeons and transplant centers rely on collaboration to share techniques and best practices in living liver donation. Beginning in 2017, the University of Michigan Transplant Center partnered with in another large transplant center in New York City with extensive experience with living donor liver transplantation. This partnership has led to the substantial growth of living donor liver transplantation at UM, but also offered a unique opportunity for Logan.
A nurse at Weill Cornell Medical Center in New York had come forward to donate a portion of his liver to a child in New York. Just before the scheduled living donation, the child was transplanted with an organ from a deceased donor. The nurse remained committed to donation, and the team at Weill Cornell were aware of Logan, for whom the nurse would be an excellent match. After the donor agreed to donate to Logan, Dr. Sonnenday approached Amy to share this incredibly unique and generous offer.
“When we received the call about Logan’s donor I remember just crying. Crying out of relief because they had found a match. This was the first piece of positive news we had received regarding Logan’s health, and while I knew he still had a long road ahead, I had this renewed sense of hope and feeling he was going to be okay” recalls Amy.
While normally the living donor and transplant recipient operations happen in the same hospital, in this unique case the donor understandably preferred to have his operation at his home institution, close to his family and home where he would spend his recovery. Similarly, Logan and his family wanted to have his operation at UM, with the team to whom they had entrusted Logan’s care. Dr. Sonnenday and colleagues created a plan to allow this unique procedure – “remote living donation” – to occur. On May 21, 2019, a team at Cornell performed the donor operation, removing a portion of the donor’s liver which was placed on ice in chilled preservation fluid, taken to a waiting plane, and flown immediately to Michigan. At Mott Children’s and Women’s Hospital, Dr. Sonnenday and the transplant team had begun to remove his diseased liver while the donor liver arrived. This unique procedure was the first reported example of remote donation in living donor liver transplantation in the United States.
Logan’s surgery took approximately seven hours; less than the 8 – 12+ hours Amy and Nick had been told it typically takes and had no complications. Following surgery, Amy and Nick were told that Logan would likely be on a ventilator for two to three days. But, like his surgery, in true Logan fashion he was taken off the ventilator less than 24 hours later, and was sitting, standing and playing with his toys just days after his surgery.
Now Logan is a ball of energy, with no off button.
Today, now nearly four years old, Logan is thriving.
“Now Logan is a ball of energy, with no off button,” jokes Amy. “He loves to play outside, ride his bike, climb on anything and everything he can, and play sports. He’s funny and sarcastic, but also loving and sweet, and one of the toughest people I know.”
Amy and Nick are forever grateful to Logan’s donor and for the care they received from their Michigan Medicine team.
“For everything the team did, the months of working around the clock for approvals from insurance companies and making sure everything was in place for the surgeries – we couldn’t have asked for a better team and outcome,” says Amy. I thank God every day for our team. They are an extension of our family and I know that Logan is here because of them.”
Now, Amy hopes to become a living liver donor herself. She has completed testing and been approved for donation. She is now waiting for a child in need for whom she is a match.
Says Amy, “If I can pay it forward, and give another family the same gift we were given I want to do that. For me, thank you will never be enough for what our donor and our transplant team have done for Logan and our family, but I hope if I’m able to pay it forward one day and be a donor myself, they’ll know it was inspired by them and the incredible gift they’ve given us.”
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