Presque Isle infant needs stem cell transplant to beat life-threatening illness

By Kathy McCarty, Presque Isle Star-Herald
Posted Oct. 12, 2013, at 3:03 p.m.

PRESQUE ISLE, Maine — Brylee Ann-Marie Gagnon, born Aug. 8 at The Aroostook Medical Center to Shandi Page and Chad Gagnon of Presque Isle, was considered a healthy child at birth, weighing 8 pounds, 13 ounces. But in the few short weeks afterward, Brylee’s family discovered she suffers from a life-threatening illness.

“She appeared to be the picture of health. Doctors had no concerns with her and after a one-day stay in the hospital, baby and Mom were discharged and sent home,” said Debby Page, Brylee’s maternal grandmother.

Like some newborns, Brylee had a few problems with gas and what seemed to be cramping during her first week at home, said her grandmother.

“She was brought in to see the doctor and her formula was changed. But that did not seem to improve things. Then suddenly when she was 10 days old, she spiked a fever of 104 degrees. She was rushed to TAMC where they decided to LifeFlight her out to Bangor immediately,” said Page.

Page said the family later found out that doctors did not expect Brylee to survive the trip, but against all odds she did.

Brylee spent nearly a week at a neonatal intensive care unit in Bangor. After what Page describes as “dozens of tests,” doctors determined Brylee had a serious infection in her blood and gastrointestinal tract.

“She was then transferred to Portland to meet with a pediatric gastroenterologist. She ended up spending a month in the hospital to be sure the infection was completely gone,” Page said. It was during this time that the underlying problem was discovered.

Brylee was diagnosed with leukocyte adhesion deficiency, a rare disorder that affects the body’s immune system. According to the Centre of Immunodeficiency, only about 300 cases of the genetic disorder have been diagnosed worldwide. The disease occurs when white blood cells are unable to produce enough of a specific protein that helps them travel to the site of an infection.

“We have been told only three or four babies a year are born with it in the U.S.,” said Page. “It basically means her immune system is not functioning, and she cannot fight off any bacteria or fungi.”

Page said doctors indicated most babies born with this disease will die of an overwhelming infection before their first birthday unless they get a stem cell transplant.

“Doctors have given her an approximate 80 percent chance of recovery [if she gets a transplant], which makes us very hopeful,” she said.

Brylee is now 8 weeks old and is home awaiting her transplant. She has monthly doctor’s appointments, and will have to undergo a week of chemotherapy before her transplant, according to Page. After the procedure, Brylee will stay at Dana-Farber/Boston Children’s Cancer and Blood Disorders Center for at least 100 days for continued monitoring.

Once the 100 days are up, Page said Brylee will be allowed to go back to the Bangor hospital and must remain there for two months for additional monitoring.

The little girl will be away from home for six months or more, Page said, and her parents plan to be with her throughout her treatment. Brylee’s father will take an unpaid leave of absence from work to be with his family while Brylee is in Boston.

Page said once her granddaughter is stable, the girl’s father will return to Aroostook County while her mother remains with Brylee.

Shandi Page and Gagnon’s three other children — Cheyenne, 5, Justin, 7, and Jocelyn, 8 — will stay at home while their parents are away, Debby Page said. Grandparents, including Page’s husband, Steve, and Liz and Kirk Lilley, will look after the three children. Page said Brylee also has a 5-year-old brother, Gage, who lives out of state.

So far, no donor has been found.

“Obviously, everyone in the family is ready and willing to donate, but in this case, because this is a genetic disease, family is not the best option for a donor, so we rely on the kindness of a stranger to save the life of our beautiful little girl,” said Page.

Supporters are trying to raise funds to help the family cover travel and other expenses.

Page said those looking to donate can go to www.fundraiserforbrylee.com. There’s also an account to help the family through County Federal Credit Union. Checks can be address to Brylee Gagnon, Attn: Dan Bagley, County Federal Credit Union, 82 Bennett Drive, Caribou, ME 04736.

“We will also be doing a benefit dinner and silent auction in the near future, but plans are still in the works for that,” said Page. “Most importantly though, we’re asking for prayers for Brylee and her family.”

http://bangordailynews.com/2013/10/12/health/presque-isle-infant-needs-stem-cell-transplant-to-beat-life-threatening-illness/ printed on September 18, 2014