Rare surgery in Montreal allows 9-year-old girl to live normal life

A rare surgery at the Montreal Children's Hospital is allowing a nine-year-old girl to keep her adrenal glands and live a normal life.

Mathilda Stacey lives with Von Hippel-Lindau disease, a rare genetic disorder that can cause tumours and cysts to grow in various parts of the body.

"She's just such a cool kid," beams her mother, Stephanie Stacey. "I try my best to explain things in an age-appropriate way... This is a genetic condition, but it doesn't define her."

Stacey, who also lives with Von Hippel-Lindau disease, says she was heartbroken when Mathilda's test results came back positive.

"Nobody wants to go through that for their family or their kids," she said. "Obviously, when you find out that there's an issue with your kids, you want to deal with it as soon as possible, like, you don't want to wait."

Stacey says she found out that she had the genetic condition just before giving birth to Mathilda -- and had to undergo surgery on her adrenal glands around the same time.

"When we talk about her illness, we don't really use terms like that," she tells CTV News. "I just say, 'our family has this condition that sometimes makes us grow these extra bumps and lumps inside of our bodies.'"

Stephanie and Mathilda Stacey at the Montreal Children's Hospital. (MUHC)

Last year, Mathilda's family found out that she had a tumour on her adrenal gland that would require surgery.

"I said, 'Unfortunately, we found one that needs to be taken out' and she kind of just took it in stride like a champ," she said. "She was really, really a brave little person."

The question then became whether to do a full or partial adrenalectomy.

Doctors in Ontario, where the family lives, said a partial removal was not an option.

The only physician willing to perform the surgery was at the Montreal Children's Hospital.

"If you catch the tumours while they're small, you can remove just the tumour and not the entire adrenal gland," said Dr. John-Paul Capolicchio, the pediatric surgeon who performed the operation.

It is a procedure that is very rarely performed in Canada and requires a specialized team of experts.

"In touching the tumour, it releases the hormones that cause high blood pressure," he explains. "We go in with an understanding that if we feel things are getting too risky, that we'll go back to the standard option [of completely removing the adrenal gland.]"

Though the family says they were prepared for Mathilda to need surgery, navigating the health care network and managing the details of her condition were admittedly hard -- including travelling from Carleton Place, Ont. to Montreal, Que. for the operation.

"[At] one point, when she was being wheeled into the surgery, she was like, 'stop talking about it, mom,'" Stacey recalled. "And I was just like, 'Alright, little buddy.' She was managing her own expectations and her own emotions around it, and I thought that was pretty powerful for a nine-year-old kid."

Mathilda Stacey and her two sisters. (MUHC)

Stacey, who has two other daughters who also live with Von Hippel-Lindau disease, says she often worried about whether she was making the right choice for Mathilda.

"[I] definitely had a lot of inner turmoil about having chosen that strategy, particularly for her surgery, where you're always second guessing whether you made the right choice," she said. "It was really hard to manage a medical decision for someone who's not old enough to make their own, and the fear that maybe I might have made the wrong choice for her the whole time was very heavy."

In the end, Mathilda underwent the life-changing surgery in December 2023 and, after two days in hospital, was released from care.

"She was such a good kid," Stacey said, smiling. "When we got home, she was excited to sleep in her bed and see her sisters and hang out on the couch with the family."