April 16, 2024
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Small Blessings Smyrna couple fight one day at a time to save their 2 children from fatal disease

Hanging on a corner of Kris and Keely Calaman’s living room wall is a small wooden sign with a simple message: “Count your blessings.”

The young Smyrna couple know how to do that all too well.

Both of their children have Krabbe’s disease, a fatal genetic disorder that affects nerve development in the brain. Most children with the disease don’t live past age 2.

Already, Michael, 2, has lived longer than the eight months he initially was given to live when first diagnosed in April 2001 with the disease.

His sister, Michaela, 1, underwent a stem cell transplant last January at Duke University Medical Center in Durham, N.C., that could save her life. The process, which is 85 percent effective, was completed five weeks after she was born, making her one of the youngest children ever to undergo the procedure.

“She’s really lucky to be alive,” Keely said during a recent interview at the couple’s home, as she cradled her daughter on the couch and fed her with a bottle. “There were a lot of kids down there who didn’t come out of transplant.”

For 10 days, the infant was given drugs through tubes into her body that destroyed her bone marrow in preparation for the transplant. After the transplant, she was given more drugs to suppress her body’s immune system.

The last of the tubes was removed last month, leaving small red scars on the child’s chest – marks Keely calls “battle scars.”

The family spent more than five months in North Carolina living at a Ronald McDonald House, away from family and friends and the familiar surroundings of their small town, while Michaela underwent the treatment.

Earlier this month, there were weekly trips for tests at Maine Medical Center in Portland. Now the trips are down to once a month.

Keely described the past year in one word: “hell.”

The couple was looking forward to holidays at home with their children. Four red stockings were hung over the couch beneath family pictures, the Christmas tree was decorated and there were presents underneath.

“We’re not going anywhere,” Kris said with a chuckle of relief. “We’re staying right here.”

The ordeal for the couple began when Michael was less than a year old. He hadn’t been acting right, so they took him to the doctor. It was then that they learned he had Krabbe’s disease.

To make matters worse, they learned that Keely was pregnant with Michaela and that the baby also had the disease.

Krabbe’s disease (pronounced “krah-beez”) is a defect in the genes preventing proper development in the brain. Specifically, the nerve fibers’ myelin sheath, which carries electrical impulses to other nerves in the body, does not form properly, preventing the signals from properly reaching other nerves.

The disease eventually affects all brain areas. Sections of the brain shut down, and body functions fail.

Chances of having a child with the disease are one in 285,000, but both Keely and Kris unknowingly carried the gene that causes it, which increased the risk for their children.

Today, Michaela is doing well and they see progress every day, Kris said.

“It looks to us like she’s right on track,” he said. “She’s a little delayed, but every day it seems like she’s catching up.”

The first two years after the transplant are critical, but Kris and Keely are optimistic.

“The oldest living kid that’s had a transplant is 6 years old,” the mother said.

Michael, who has progressed too far to benefit from a transplant, seems to have stabilized. Though he is still much like an infant and must be fed and receive medications through a tube in his stomach, his condition hasn’t deteriorated significantly.

“He’s doing great,” Keely said, looking toward her son as he rested on his father’s lap.

“We still don’t understand why he’s doing so well,” she said. “He can see you when you’re up close; he can see his toys; he can see his sister.”

For now, as a stay-at-home mother, she devotes her days to taking care of and playing with her children while Kris is at work at a trash company in Houlton.

The day begins at 5 a.m. with medication for Michael and a bottle for Michaela, and a change of diapers for both. By 9 a.m., both children have been bathed, dressed and fed, enjoyed some TV and played with toys.

The day can include going to the store, riding in the car, being pulled in a sled, or visiting with Keely’s parents, who live next door.

The children also have physical therapy sessions three days a week.

“I wouldn’t say it’s really any different than anyone else who has kids, other than the physical therapy,” said Keely.

“We try to do all the things that other people do,” added Kris.

Despite the siblings’ being apart for much of the past year and Michael’s disabilities, Kris and Keely are amazed at how close the children are.

While next to each other on the couch, Michaela coos and smiles and reaches out to touch her brother’s face.

“He tries to slide toward her and sticks out his tongue at her,” Kris said of Michael.

“It’s like they can communicate,” said Keely, adding that Michael, who has never cooed, makes subtle noises at his sister.

Despite all that has gone on in their nearly three years of marriage, the couple have managed to stay positive and in many ways have become closer, Keely said.

It’s based on a simple outlook: “We don’t wake up every day thinking that Michael’s going to die,” Keely said. “We just take it day by day.”


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