It was Thanksgiving weekend, and our family was gathering at my brothers’ farm outside of Humboldt to celebrate.
As we were all hanging outside enjoying the beautiful weather, I got a phone call from my sister saying they would be unable to make it out, and that they were on there way to RUH to meet her daughter, Megan, and husband, Kian, who had taken their daughter Emma, to the Children’s Hospital in Saskatoon, following a blood work up that showed Emma had high direct bilirubin levels.
Through a nuclear scan, it was confirmed Emma has biliary atresia (BA), a rare liver disorder. Essentially it is when bile flow from the liver to the intestine is blocked, which causes a backup in the liver. This can quickly cause damage and scarring of the liver, which eventually leads to liver failure.
After this was confirmed, she was scheduled for a 5-hour surgery called the Kasai procedure a week later.
“We were told by our pediatrician and surgeon that most children with BA have a liver transplant by age 2 to 5; however, a small percentage will do fine after the Kasai procedure and never need a transplant,” said Megan.
But Emma’s journey continued to have more bumps along the way.
In mid-November Emma’s routine blood work was quite elevated from when it was done at discharge from hospitalization following the surgery a few weeks before. Upon consultation with the pediatric GI team in Edmonton, it was decided that Emma should be hospitalized and treated with IV antibiotics; it was suspected her blood work was elevated due to cholangitis, (an infection of the bile ducts, the tubes that carry bile from the liver to the gallbladder and intestines), despite not having other symptoms. Luckily, they only had to sleep at the hospital for one night and were then granted day passes, which meant driving to hospital at 8am, 4pm, and midnight.
Following the series of antibiotics, her blood work only slightly decreased. Next step; repeat bloodwork and see Emma’s pediatrician the following week.
In December they travelled to Edmonton to see a liver specialist for Emma and have an ultrasound. The specialist expressed that Emma’s bloodwork was not good. Everything had been quite elevated since her surgery, including the direct bilirubin, which had basically been over 100 since (normal range is up to 5). She informed them if the direct bilirubin had been over 100, the surgery was not successful.
They were told that because her bloodwork is all high, this will likely cause her liver to get sicker. This can look like slow growth, portal hypertension (increased pressure within the portal venous system) and bleeding, and cholangitis.
The good news was that the doctor was very happy and appeared surprised with Emma’s growth and milestones. “Emma was doing very well in this category for a BA kiddo! So that was at least good news.”
After her ultrasound, the specialist informed them she spoke with the transplant team about Emma. They were told they would have to go back to Edmonton in February for transplant assessment, where Emma would be an inpatient at the Children’s hospital in Edmonton for one week.
This was very disheartening as they were hoping this would not occur until Emma was older.
“I’m still having a difficult time comprehending the fact that, in her short five months, this disorder has caused enough havoc that my little baby needs a new organ.”
“The thought of not being in Saskatoon, with our support network, during this time is also daunting,” said Megan.
As the month went on, most of Emma’s bloodwork relating to her liver continued to worsen. Her GGT (marker for bile ducts) had actually decreased the last two times they went for bloodwork, which led their pediatrician to think it may have taken this long for the procedure to work; however, the damage to her liver had already been done.
Emma continued to see the pediatrician, as well as bloodwork and weight check, every two weeks at the Children’s hospital in Saskatoon.
“Every visit he brings in residents and medical students to assess her belly and educate on biliary atresia. Emma’s liver and spleen are so enlarged that they can be felt, which is not normal. While each doctor/student pokes and prods her belly, little Em is as chill as can be. One time she was flirting with the male medical student; she was grabbing his hand and trying to roll over to see him.”
Emma continued to gain weight during this time, which was a good sign, however, the thought of what was to come with the transplant assessment was lingering on in their minds.
They would meet with the transplant surgeon and every specialist under the sun. Emma would have multiple tests, scans, swabs, labs; and Megan and husband Kian would attend a transplant education session. By the end of the week, they would know whether Emma needs a transplant and an approximate timeline of when this would need to occur.
After spending a week at Ronald McDonald House while Emma is assessed at the Stollery Children’s Hospital in Edmonton, it’s the end of the transplant assessment, and the team has listed Emmy at Level 0, which means she needs a transplant but needs to grow bigger. Her portal vein, the main vein supplying blood to the liver, is only 5mm and it needs to be 7mm. The hepatologist suspects the transplant to happen around her first birthday, although, it could be sooner as this disorder can change rapidly. She is also a candidate to receive a living donor.
“She ideally needs to gain weight, as this makes the surgery “easier” and less likely to have complications. As a result, an NG feeding tube was placed to ensure she is getting more calories and fat. Her diet will need to consist of 50% MCT oil to hopefully fatten her up!”
During a scheduled clinic visit with the hepatologist (liver specialist) Monday, February 26th in Edmonton, it was determined that Emma needed to be hospitalized at the Stollery.
“We were shocked, unprepared, and heartbroken to be back so soon. We don’t know when she will be discharged…. The hepatologist said ‘Emma is giving us signs’, meaning her body is letting us know she is near the end stage of her liver failure. She will likely need her transplant sometime in April.”
Emma received her live vaccines on Thursday, the 29th, and they need to wait four weeks post live vaccines.
“We may be here until the transplant…. Then we are in Edmonton, at the minimum, for three months post-transplant. I don’t have many other words right now; except we are extremely grateful to three donors being actively worked up right now. The living donor coordinator office has been overwhelmed with the amount of phone calls; they have not been able to keep up and return them all. Thank you to everyone who even considered being a donor. We cannot explain how much this means to us.”
A GoFundMe page (https://gofund.me/43974ba3) has been set up to help them with financial distress as they are displaced from their home, work, and at times, their three-year-old son for the next 5 – 6 months. This will help with accommodation in a private home as Emma’s immune system will be compromised post-transplant, as well as food, gas, etc. All donations are greatly appreciated.
