Canadian parents advocate for organ donation as baby daughter with rare liver disease awaits transplant

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When Megan and Kian Crawley welcomed their daughter, Emma, late last year, everything was normal. Their newborn had no apparent health issues, they were discharged from their local hospital in Saskatoon and no one had any major concerns. But roughly six weeks into their lives with a new baby, things took a turn.

During a check-up around that time, Megan brought up with her family doctor that her daughter's skin looked jaundiced and her eyes had become cloudy. According to Megan, the physician wasn't exactly sure if it was something to worry about, but a second doctor who visited suggested they conduct a full blood workup. The morning after the test, results indicated Emma had high levels of liver enzymes and bilirubin in her body, and she was admitted to the hospital.

At Saskatoon's Jim Pattison Children's Hospital, health-care practitioners started taking several tests that began ruling out a variety of potential issues Emma might've been experiencing. Moreover, the Crawleys slowly started learning more about what their daughter was battling — and what their family still faces today.

A tearful diagnosis

Bilirubin, a yellowish substance found in bile, is a normal part of the body that everyone produces and eventually expels from their system. There are two types, including an indirect (unconjugated) version that travels through the blood to the liver, where it's then changed into the direct (conjugated) type. For most people, that latter version of the substance exits the body through urine or stool.

But Emma's bloodwork showed she had high levels of direct bilirubin, and health-care practitioners told the family she likely had a rare liver disease called biliary atresia. It's a condition — affecting roughly one in every 10,000 to 20,000 infants — where the bile ducts become damaged and prevent bile from leaving the liver.

"They had mentioned, 'She's going to need a surgery,' and we're like 'OK, so after the surgery, she'll be good, right?'" Megan recalled. "And they're like, 'No, it's a lifelong disorder.' Then, they drop the bomb on us that she's going to need a transplant."

Initially, Kian believed that wouldn't be necessary until later in life once she's a young adult. However, they were told Emma would need to have the surgery before age two.

"At that point we all broke down in tears and just were in disbelief," Megan told Yahoo Canada.

Nuclear imaging confirmed Emma's diagnosis: She had no gallbladder and bile was getting backed up in her liver. Six days after being admitted to the hospital, Emma underwent a Kasai procedure, where surgeons take a piece of the infant's own small intestine and connect it to the liver to help with drainage. Afterwards, things were looking up. Emma's bloodwork started coming back normal and she began to have green stool, an indication her body was getting rid of bile.

"Then all of a sudden, [her stool] started turning back being like this really pale, yellow-y grey, which isn't good," Megan explained. "I had this gut feeling then right there in the hospital, not even a week after the surgery. I was like, 'This didn't work.'"

The family's health-care team had said it might take a while for things to adjust. But two weeks post-surgery, Emma's bloodwork came back higher than it was before she was admitted to the hospital. Health-care practitioners then believed Emma might've had an infection in her bile ducts called cholangitis. She was given IV antibiotics for a week, but the levels in her bloodwork kept rising.

Hoping for a transplant

At this point, Emma needed to follow-up with a gastroenterologist, so the family was referred to one in Edmonton, more than a five-hour drive from Saskatoon. They went in early December, where they essentially got confirmation the Kasai procedure didn't work and that they'd have to return in February for a transplant assessment. Megan then said they met with "every sort of specialist possible," including child psychologists, nephrologists and cardiologists.

Assessing Emma for a potential transplant, the health-care team conducted CT and MRI scans to get a detailed look at her liver, particularly the portal vein, which is the main vein to the liver. Since Emma would likely be getting an adult donor's liver, the team wanted her portal vein to be as large as possible, ideally around the seven-millimetre mark. At the time, hers was a couple of millimetres too small meaning she needed to grow.

But then, Emma started having a lot of fluid build-up in her body that had to be managed, forcing the family to stay in Edmonton for a few extra days until they were able to return home. A couple of weeks later, they were back in Edmonton for a regular check-up, expecting to be in Alberta for only a couple of nights. However, the family's hepatologist explained she was concerned about more fluid that had built up in Emma's belly, and they were admitted that day for almost two weeks.

"At that point, the hepatologist told us Emma is in end-stage liver failure," Megan shared.

Eventually, they were able to return to Saskatoon. But a few days upon returning home, they were admitted to their local hospital once again due to fluid build up, known as ascites. The teams in both Saskatoon and Edmonton discussed the situation, and it was determined that Emma needed to be in Edmonton until she had a transplant.

'Heartbreaking news'

The Crawleys then became hopeful they'd be able to find a living donor candidate, where someone could give a piece of their liver to Emma which she could grow with. Some family members and friends had underwent the rigorous process to see if they were matches and could be a donor for Emma.

However, the family received results from another CT scan last week that showed Emma's portal vein is too small, ultimately making her no longer eligible for a transplant from a living donor.

"This news became even more difficult to bear as the same day we found out there was finally a living donor match for Emma," Megan said. "We now need to wait for a deceased pediatric donor."

Megan said her daughter is currently at the top of Canada's national deceased donor list. But even if a match is found, circumstances remain complicated. While Emma's portal vein can't be used, there's another vein that offshoots from that direct line that could have potential. Health-care practitioners won't know if it's large enough to accommodate a transplant until she's opened up for surgery, and even then it might be too small.

"If that's the case, they would not be able to simply do a liver transplant," Megan noted. "Because of this, they have to plan for a multi-visceral transplant, which means her stomach, intestine, pancreas and, of course, liver would all be replaced. This comes with more risks, including infection, rejection, longer recovery period and decreased long-term survival rates."

Advocating for organ donation

Since uprooting their lives to be in Edmonton full-time, the Crawleys said it's challenging to organize responsibilities back home and take a step back from their situation.

"It's tough to be present," Kian said. "Someone's always with Emma 24/7, so it's just difficult to disconnect. ... It's not like we're able to go out and do something together as a family so easily."

Still, support from hundreds of people has left them feeling overwhelmed and grateful. In February after a check-up when they found out they'd be in Edmonton indefinitely, Megan's aunt created a fundraiser in hopes of crowdfunding money to go towards the family's expenses such as food and accommodations. As of Thursday, more than 250 donations have raised over $32,000 for the family.

"It's so overwhelming," Megan said. "Emotionally, every time I would see any donation, I would just get tears in my eyes about people that are willing to do that for us. It just blows my mind how people have really come together for us during this time.

"In those dark moments, there is still a lot to be grateful for."

Now, Megan is urging people to have conversations around organ donation and hoping to raise awareness around being a living donor. While she said it's easy to sign up to become an organ donor, she added she didn't know there's an option to be a living donor for organs like the liver and kidneys.

In Canada, less than 25 per cent of people living in the country are registered donors, according to the federal government. That's even though 90 per cent of people say they support organ donation, per Canadian Blood Services.

"Organ donation is important to discuss," Megan said. "I know the thought of having a child in a position to donate their organs is unbearable, but it has the potential to save the life of another child."

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