Health Canada approval ensures bright future for Chatham boy with Alagille syndrome

Chord Jaques is an enthusiastic, happy 11-year-old boy, who enjoys having friends over to play games.

To see his big smile and cheerful attitude, it’s hard to believe the Chatham boy spent much of the early years in agony trying to scratch a never-ending itch that was caused by being born with a cholestatic liver that is the hallmark of Alagille syndrome. The condition affects about one in 30,000 infants.

Chord’s mother Tara Jaques said people with a cholestatic liver have either too few bile ducts or the ducts are too small. As a result, she said, “There’s not enough room for the bile to get through so the body reabsorbs it and it causes them to get itchy from inside the body out.”

As Chord frantically tried to scratch an itch he could never reach, he’d draw blood and leave scars. Jaques and her husband Ben would go into “survival mode” just to get through the day, she said.

“Watching your child suffering and being under attack from their own body and not being able to help, it’s a feeling that you can’t articulate — It’s awful. It’s torture,” she added.

“It’s like going to sleep after a camping trip and you’re covered in mosquito bites. It’s when you try to calm down and go to sleep that it’s the worst,” Ben Jaques said.

He said Chord would scratch and scratch until he finally got so tired he passed out.

But Chord and his family are enjoying life and getting a lot more sleep these days thanks to the medication Livmarli, manufactured by Mirum Pharmaceuticals, which received Health Canada approval last month.

“He’s incredible,” Tara Jaques said of how Chord is doing today. “Without the itch, he’s absolutely driving.”

When asked how life is today, Chord said, “It’s pretty easy now.”

He now does things a typical 11-year-old enjoys such a riding his bike.

“I like having company and playing games,” said Chord, who is a big fan of Disney Jeopardy on YouTube.

The ALGS community is celebrating the drug’s approval.

“We are thrilled that patients in Canada will now have access to Livmarli as we have seen the powerful impact it can have on people living with life-altering pruritus,” said Roberta Smith, president of the Alagille Syndrome Alliance in a release.

“I know from my own experience how painful it is to watch your child suffer and how disruptive this disease can be for the whole family.”

Chord was part of a trial for the medication back in May 2016 when he was in kindergarten until November 2018 when he was taken out of the trial after he became ill, Jaques said.

He was able to return to the Livmarli trial in March 2021. Now that the drug has Health Canada approval, she said they don’t have to worry about their son being taken out of the trial.

“His doctor can prescribe it now,” she said.

Jacques said it took two years to figure out Chord had ALGS.

From the time Chord was an infant, “he was never content. He was never settled,” she said.

Once he got older his parents realized he was trying to scratch himself. They changed laundry soaps, tried environmental products, looked at allergies and tried creams and medicines.

They asked for blood tests when Chord was one and a year later they finally received information about ALGS.

“We found an Alagille specialist at Sick Kids (hospital) in Toronto who agreed to take him on,” Tara Jaques said. “It’s been amazing.”

Finding those who understand the situation has also been helpful to the family and a lot of support has come from Chord’s school.

Tara Jaques said Chord wore one-piece pajamas up until he was six to help protect his skin from him scratching it.

“His kindergarten teacher did a lot of pajama days at school, so he wasn’t the only one in pajamas.”

The couple met with all his teachers to explain his condition.

Tara Jaques said one of his kindergarten teachers found him in the coat closet one day on the floor scratching his legs, because the itch had taken over.

“She took her shoes off and sat next to him and she scratched her legs.”

But not everyone has been so understanding. Tara Jaques has had people comment when they saw Chord with scars on his face and body from scratching.

Noting it is not easy to describe the situation in a few sentences, she said, “I started carrying out little postcards that talked about Alagille syndrome and I started handling them out to people.”