3-year-old Caribou boy battles rare disease at Boston hospital

CARIBOU, Maine — When Zyen Provost was just over 10 months old, he suddenly came down with a fever.

His great-grandmother, Karyn Pinette of Caribou, figured he had just picked up a cold. She and the boy’s father, Kristopher Ginn, who is Pinette’s grandson, took him to the hospital.

Tests revealed the baby was suffering from respiratory syncytial virus, a common ailment that is a major cause of lower respiratory tract infections during infancy and childhood.

“They admitted him so they could treat him,” Pinette recalled late Friday evening. “But then he also had an ear infection, an enlarged liver and spleen and pneumonia. It was bizarre. He just came down with all of these illnesses.”

The boy was released but he just couldn’t seem to beat the pneumonia.
Another trip to a local hospital was short lived, as doctors discovered that tests indicated an underactive immune system. He was transferred to Eastern Maine Medical Center in Bangor.

Fright set in, followed by another transfer, this time to Maine Medical Center in Portland. The family grew frantic when doctors began testing for leukemia. That was ruled out, but the baby underwent treatments to combat his immunodeficiency as physicians worked to find a proper diagnosis.

During an appointment in Boston this past January, the family learned three letters that have changed their lives — XLP, short for X-linked lymphoproliferative syndrome.

Also known as Duncan’s syndrome, it is a genetic defect found in boys that causes the immune system to respond abnormally to some viral infections, resulting either in an underactive or overactive immune system. The disease is seen so infrequently that only approximately 400 boys have been diagnosed worldwide, according to the XLP Research Trust.

The cause of the condition, a mutation in one of the genes on the X chromosome, was discovered in 1998. Because of this, medical experts believe there are many undiagnosed or improperly diagnosed cases, according to an article published last month by the scientific journal Immunity. Treatment includes anti-viral medications, immunoglobulin therapy and steroids to ease symptoms presented by individual patients.

If untreated, approximately 70 percent of patients with XLP die by the age of 10.

Currently, the only cure is a stem cell transplant, a procedure that Zyen Provost began preparing for on May 23, just one day after his third birthday.

It was on his birthday that Zyen was admitted to the Boston Children’s Hospital to begin chemotherapy. On May 31, he underwent a nearly seven-hour stem cell transplant.

Zyen has involved parents in Ginn and Jessica Provost, his mother, and Pinette said that they share custody of the child. But Pinette said that she and her husband, Ryan Pinette, handle the majority of his medical care and transportation and have been traveling back and forth to Boston. Ginn and Provost also have been with their child as he recovers.

Pinette headed back to Boston on Saturday morning after overseeing a fundraising raffle that Ryan Pinette organized to help defray some of Zyen’s medical costs. Zyen has insurance coverage through MaineCare, but expenses for travel, food, lodging and lost wages have hit the family hard.

Pinette said she likely will continue to deal with lost wages for the next year or two.

Zyen was released from the hospital on June 27 and discharged to the nearby Ronald McDonald House, which has 13 studio apartments designated specifically for children recovering from bone marrow and stem cell transplantation surgery.

Zyen can only go outside with a mask, but a heat wave and health concerns have kept the 3-year-old mainly inside or in Ryan Pinette’s air-conditioned vehicle, exploring the city.

“Throughout this, Zyen has just had a great attitude,” said Karyn Pinette. “He always has a smile on his face. He’s always happy. When his hair started to fall out, he was laughing. He was taking little clumps of his hair and pulling it out and tossing it around the room.”

He will remain in Boston at least until Sept. 8.

“We were told that 100 days is the milestone,” she said. “If that time period passes with no major issues or rejection of the transplant, it is a good sign.”

Even when the boy returns to Caribou, visitors will be restricted and he won’t be allowed outside for much more than weekly doctor visits.

Ryan Pinette quickly organized a raffle thanks to donations from several Caribou businesses, including S.W. Collins Co, Leo’s Citgo, and Sleeper’s.
A homemade lap quilt donated by Darlene Martin of New Canada also was raffled.

The family has also set up an account through The County Federal Credit Union for donations.

Pinette said she and her husband have been bolstered by the support they’ve received from the community and from the Boston hospital.

In The County, The Cubby Thrift Store, which has locations in Presque Isle and Caribou, created “The Cubby Fund” to benefit families of children with life-threatening illnesses.

Pinette said Zyen was nominated as its July “hero” and the business donated $100 to offer support. The Carl Robert Dream Foundation, a nonprofit dedicated to helping local families with terminally ill children, also has helped the family financially.

The family was able to cut back on expenses for food at the hospital thanks to local businesses and families who have prepared or donated meals for patients and their families. On June 30, several rookie players from the New England Patriots also paid Zyen a visit.

“People have been great,” Pinette said Friday. “It has been difficult, but Zyen has stayed positive. He’s just amazing.”

For those wishing to give to Zyen Provost’s ongoing medical care and support, donations can be dropped off at The County Federal Credit Union locations in Caribou, Presque Isle, Fort Fairfield and Houlton with the donor requesting that the money be put in Provost’s account. Donations also can be mailed to the Pinette family at 1265 Van Buren Road, Caribou 04736.