Eden’s Biliary Atresia Diagnosis Story
On September 6th, 2023, Eden was a day away from turning 2 months old. We took her in for a routine weight check out of concern that she was not gaining weight, despite frequent feeding, but our pediatrician was more concerned with the fact that she was still jaundiced. We had brought the concern up in the past to different professionals and friends but were told it was “breastfeeding jaundice” and would go away on its own.
We immediately did labs the same day, and the following morning we were told that we were being referred to St. Louis Children’s Hospital because this was more than a case of lingering jaundice.
We met with the GI specialists that Friday, and they explained that they needed to do labs and an ultrasound. Later that evening, we spoke with the doctor who told us that the tests were inconclusive, and we would have to move forward with a liver biopsy.
They told us Eden could be diagnosed with a condition called biliary atresia, which is quite rare (1 in every 10-15,000 babies). It is a congenital defect in which the bile ducts in the liver didn’t form properly in the womb, and bile ends up backing up into the liver instead of flowing to the gallbladder. No one knows why it happens, but without surgery or a liver transplant, it is terminal within 1-2 years after birth. And even with surgery, the likelihood of still needing a liver transplant is very high. In fact, biliary atresia is the leading cause for pediatric liver transplant.
On September 15th, Eden had an intraoperative cholangiogram that indeed confirmed the diagnosis of biliary atresia. The gallbladder was small and nonfunctional, the bile ducts were not formed correctly, and there was some damage to her liver already. They went ahead and did the Kasai surgery while she was in the OR. She was 2 months and 8 days old, and in total, we spent 19 days in the hospital from diagnosis to Kasai recovery. It was then that we realized our lives and expectations were never going to be the same.
Our Journey to Transplant
For the first month home, we were optimistic. Eden’s skin and eyes went back to almost normal. All of her labs began to drop, and we held our breath.
But we kept getting admitted back to the hospital at least once a month, due to suspected infections. As November rolled into December, we noticed her skin and eyes becoming more yellow. Her labs hadn’t been trending down since October, and in our hearts, we knew it wasn’t looking good. We would know for sure if the doctors felt the Kasai had failed by our appointment on December 6th, but we could already tell it had. We prepared ourselves for the recommendation of transplant.
At our appointment, the labs began to trickle in, even as we spoke to her doctor. It was bad. Her bilirubin had increased nearly 3x what it was in November! Our suspicions were confirmed – the Kasai surgery was failing, and we were going to be referred to the transplant team. We had prepared for this and were not surprised.
What we didn’t expect was to be admitted the same day to treat for possible infection again, and for numbers to barely budge even with treatment. In fact, some lab results were going up even higher. Within days, her belly was swelling up with fluid so badly that she couldn’t sit up or roll over. It seemed that instead of a severe infection, her liver was just beginning to give up and her tiny body couldn’t handle it.
While we waited in the hospital for medications to help get the ascites under control, we proceeded with our evaluation for liver transplant and on December 19th, 2023, we received word that she was officially on the UNOS list with a natural PELD score of 29. Shortly after, she received exception points that brought her up to a 35, and within a week, she again was bumped up to a 37.
An Answered Prayer
Christmas passed with no word on a liver, despite Eden growing sicker. She became unable to eat by mouth, often throwing up what little she did eat. Her blood sugar also became unstable and would drop very low within even an hour of just having been fed. We were becoming very anxious that a liver might not come soon enough, so on New Year’s Eve, we put out a plea to social media with a flyer listing all the details for a living donor search.
Within a few days, we had over 500 shares and many people calling in to see if they could be eligible to donate. One of which was a cousin of Eden’s dad who lived in NJ, Nadia. She insisted that she wanted to donate to Eden if she was able to, despite moving so far away and having three children of her own.
After about a week passed, Nadia was approved to be evaluated and hurried by plane to get to St. Louis. The evaluation process and approval to donate only took one week! And it came none too soon, as we felt that Eden was declining quickly. In one more week’s time, Eden and Nadia were in their respective operating rooms, and Eden got her second chance at life on January 18th, 2024!
A Heart Full Of Gratitude
Eden and Nadia are now both doing very well and headed home on February 1st. Eden is like a completely different baby – not only in appearance, but in how happy and playful she is. You can certainly tell that she feels much better and is able to enjoy life now that she is no longer sick!
We are so thankful to Nadia and her family for their sacrifices. We might have still been on the transplant waiting list if it wasn’t for her! We will be forever grateful and can’t wait to share all of Eden’s “firsts” that she will have, thanks to Nadia’s generous gift of life.
We are also so thankful for all who have supported us on this journey, and thankful for the continued prayers and kindness to our family. We could have never imagined this, but we continue to rest in the peace of Christ that surpasses all understanding, and we never stop looking for joy in all circumstances. We now feel that we have received a miracle, and it’s through the eyes of gratitude that we optimistically look towards the future.
The Children’s Organ Transplant Association (COTA) helps children and young adults who need a life-saving transplant by providing fundraising assistance and family support. COTA is the nation’s only fundraising organization solely dedicated to raising life-saving dollars in honor of transplant-needy children and young adults. 100% of each contribution made to COTA in honor of our patients helps meet transplant-related expenses. COTA’s services are free to our families, and gifts to COTA are tax deductible to the fullest extent of the law.