Chord’s story

Alagille syndrome

Patient Voice spoke with Tara and Ben Jaques about their son Chord’s rare diagnosis and how a life-changing medication has finally granted him a sense of peace.

Chatham, ON

Our son Chord was born prematurely. He had an overwhelming load of health concerns that were attributed to his early birth and tiny size. The first years of his life were filled with endless doctors and tests, and it’s only looking back that it becomes clear how all of these things stemmed from Alagille syndrome (ALGS).

ALGS is a rare genetic disorder that can affect the liver, kidney, heart, brain, bones, and more. Chord’s greatest concerns are his liver, heart, and bone formation. ALGS has no cure.

And yet, the aspect of ALGS that devastated us the most was not any of the life-or-death complications that can develop with ALGS. It was the itch. Pruritus. The accumulation of bile in Chord’s liver causes an overwhelming itchiness deep within his body. An itch that never gives him a moment’s peace. An itch he can never scratch.

“…he was never able to truly be himself because he spent every moment in distress."

Chord would wake up in the morning with his bedsheets covered in blood from where he had scratched himself raw in his sleep. He wore footed zip-up pyjamas to school until he was six years old, just to keep him from breaking his skin while scratching. And he’d still be scratching away through the fabric, unable to focus in class. The itching caused him to struggle with sleeping, eating, and quality of life. Our constant focus was on helping him cope with this torture and he was never able to truly be himself because he spent every moment in distress.

It was a heartbreaking way to live. But then the day came when he was able to finally take a medication that controlled the itchiness, and it changed everything. We got to know the amazing child who had been hidden deep inside. The child who loves to sing, loves to dance, loves to read. The kid who is so inspired by Robin Williams’ Genie that he dreams of becoming a voice actor. The social butterfly who is, in the words of his dance teacher, ‘electricity.’

It’s very easy to lose hope with a medically fragile child. Things can change instantly and a new battle is always around the corner. We’re grateful for every moment of peace and joyfulness that our family experiences.”