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

Mathilda Stacey suffers from Von Hippel-Lindau disease, a rare genetic condition that can cause tumors and cysts to grow in different parts of the body.

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

Stacey, who also suffers from Von Hippel-Lindau disease, says she was heartbroken when Mathilda’s test results came back positive.

“Nobody wants to go through this for their family or their children,” she said. “Obviously, if you find out there’s a problem with your kids, you want to deal with it as quickly as possible. You don’t want to wait.”

Stacey says she found out she had the genetic condition just before she gave 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 those kinds of terms,” she told CTV News. “I’m just saying, ‘Our family has this condition that sometimes causes us to get extra bumps and lumps in our bodies.'”

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

Last year, Mathilda’s family found out she had a tumor in her adrenal gland that required surgery.

“I said, ‘Unfortunately we found one that needs to be taken out’ and she just dealt with it like a champ,” she said. “She really was a brave little person.”

The question then became whether to perform a complete or partial adrenalectomy.

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

The only doctor willing to perform the surgery was at the Montreal Children’s Hospital.

“If you catch the tumors while they are still small, you can only remove the tumor and not the entire adrenal gland,” says 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.

“When you touch the tumor, the hormones that cause high blood pressure are released,” he explains. “We go in with the understanding that if we feel things are getting too risky, we will go back to the standard option (completely removing the adrenal gland).”

Although the family says they were prepared for Mathilda to require surgery, navigating the health care network and managing the details of her condition was admittedly difficult — including traveling from Carleton Place, Ont. to Montreal, Que. before the operation.

“(At) one point, when she was being wheeled into the operating room, she was like, ‘stop talking about it, mom,’” Stacey recalled. “And I was just like, ‘Okay, little buddy.’ She was managing her own expectations and her own emotions around that, and I thought that was pretty powerful for a nine-year-old.”

Mathilda Stacey and her two sisters. (MUHC)

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

“(I) definitely had a lot of inner turmoil about having chosen that strategy, especially for her surgery, where you’re always questioning whether you made the right choice,” she said. “It was very difficult to make a medical decision for someone who is not old enough to make their own decision, and the fear that I might have been making the wrong choice for her all along was very tough.”

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

“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.”