Dodging a bullet: Lukas’ diagnosis uncovers his family’s predisposition to cancer

Boy with polyposis smiles with family
Lukas and his family

From a young age, Lukas Quinn’s life has centered around sports — playing them, following them and rooting for his favorite teams, the New York Giants and Syracuse University athletics. “He’s a real sports nut,” his mother Juli says with a laugh.

For the first seven years of his life, Lukas was a bundle of energy, active and playing sports without any health issues. So when Juli received a phone call from Lukas’s summer camp, explaining that he was locked in a bathroom with severe gastrointestinal issues, she was worried. “Lukas would not come out of the bathroom until I got there,” says Juli. “It was scary to see him in such distress.”

Little did the Quinns know that Lukas would soon be diagnosed with a medical condition that made him predisposed to develop cancer. And after a series of genetic tests, they would learn that his father and sister had the same condition.

Dozens of polyps

After the episode at summer camp, Juli and her husband Pat took Lukas to a hospital near their home in upstate New York, where a colonoscopy revealed the presence of dozens of polyps in Lukas’s colon. Many of the polyps — abnormal, usually benign, growths of tissue that line the inside of the colon — were removed successfully, however a follow-up exam a year later revealed that even more polyps had formed. The doctor didn’t know what to make of it.

“Without a clear diagnosis, the local hospital suggested Lukas continue his care with a doctor more familiar with such a severe onset of polyps,” says Juli. “We learned about [pediatric gastroenterologist] Dr. Victor Fox at Boston Children’s Hospital and found out that he was highly regarded in this specialty.”

Juvenile polyposis syndrome: A genetic condition

In 2012, the Quinns made the six-hour drive to Boston. After evaluating Lukas, Fox diagnosed him with juvenile polyposis syndrome (JPS), a condition that causes an accumulation of polyps in the gastrointestinal (GI) tract, which in adulthood could potentially become cancerous.

Fox recommended repeated colonoscopies to remove the polyps and stay on top of the condition. “We felt really good talking to Dr. Fox,” says Juli. “He explained to us what JPS meant to Lukas, and what we could expect in the future.”

Since JPS is a hereditary condition, he advised the entire family to get tested, which proved to be timely advice. It turned out Lukas’s younger sister Taryn had the condition, although she did not yet have any symptoms. Even more concerning, however, was that Lukas’s father Pat had it as well.

Pat had polyps since he was a teenager, but after the genetic study and further testing, it was discovered that Pat’s condition had progressed to the point where he would need to have his colon removed. His polyps had become too dangerous, placing him at risk for developing metastatic colon cancer.

Family with polyposis hiking in mountains
“We were blown away; we had no idea Pat had this ticking time bomb inside of him,” Juli says. Read Pat’s story on Dana-Farber Cancer Institute’s blog, Insight.

In February 2015, Pat’s colon was successfully removed at Dana-Farber Cancer Institute. “We credit Dr. Fox with getting our family tested and helping us dodge a bullet,” says Juli.

But the Quinns were not out of the woods just yet.

Excruciating pain

About a year-and-a-half later, in October 2016, Juli received another scary phone call. Lukas was in severe stomach pain at school, worse than any pain he had ever felt before. After contacting Fox, the Quinns made the urgent six-hour drive to Boston to admit Lukas to the Boston Children’s emergency room. Lukas went in for a colonoscopy, which revealed his colon was experiencing multiple intussusceptions — it was so full of polyps it was telescoping on itself in several places. After conferring with Lukas and his family, Fox recommended that the best course of action was to have Lukas’s colon removed.

The news was tough to hear, however Juli remembers that during this emotional time the family was made to feel at ease by Lukas’s team of doctors, which included not only Fox, but also Dr. Belinda Dickie, the colorectal surgeon who performed Lukas’s surgery. “They were just amazing,” says Juli. “They sat us down, explained their roles so well, and how everything was going to go. They kept us informed. You could tell they functioned so well as a team.”

The doctors explained how once Lukas’s colon was removed, they would use the end of his small intestine to create a “J-pouch” that would replace the function of his rectum. Like his father a year and a half before, Lukas would need to use an ileostomy bag for the next few months until a follow-up procedure could be performed to remove the ileostomy bag and allow Lukas to pass stool normally again from his anus.

Surgery and recovery

The surgery performed by Dickie was a success. “Lukas was cared for around the clock by a loving team of nurses who made his recovery that much easier. They were fantastic,” says Juli. “We were fully supported by every nurse there.” She particularly appreciated the small steps taken to make Lukas feel at home and get him focused once again on his passion — sports. “The nurses knew Lukas loved the New York Giants, so they wore their Patriots jerseys,” says Juli. “A social worker had written in his file that he loved sports, so that when anyone new came in who hadn’t met him, they knew that detail. That made such a big difference for him.”

Back to being a boy

Lukas persevered through the surgery, six months with an ileostomy bag and the follow-up J-pouch procedure with flying colors. Today, Lukas is a 13-year-old seventh grader who is once again active and playing sports. He enjoys playing basketball and lacrosse, and serving as the water boy for the varsity high-school football team.

Boy playing lacrosse after polyposis surgery

The Quinns still make the six-hour drive to Boston Children’s every few months to see Fox and Dickie, which, according to Juli, is well worth the trip.

“We’ve had a great patient and family experience at Boston Children’s,” she says. “We don’t want to go to just anybody. We want someone like Dr. Fox and Dr. Dickie who see a condition like Lukas’s all the time. They are not just experts at the hospital, but they also have a great bedside manner.”

Learn more about Boston Children’s Pediatric Polyposis Program.