Hunter's Hope: Families in the News

COLCHESTER -- Some mornings, Pam Kristoff wakes up and wonders whether her 5-year-old son will take his last labored breath that day. She has little time to let the worry linger.

She must shoot multiple syringes of anti-seizure medications and nutrients into the feeding tube in his belly, clear his raspy breathing with a suction tube and constantly watch the machine that monitors his oxygen level. He cannot speak, walk, eat or hold a toy.

When she gets a break from Ryan's care, it is to go to the pediatric intensive care unit at Connecticut Children's Medical Center, where her 2-year-old daughter, Alyssa, is suffering from the same cruel congenital disease.

Krabbe disease will kill Ryan, who already has surpassed his life expectancy. The prognosis is better for Alyssa, although she is now dependent on a respirator to help her breathe.

When Colchester building official Tim York saw the children in April during a routine pellet stove inspection at their home, he knew he had to help them.

"I started thinking about it," said York, a low-key, bespectacled grandfather of four, three of them about Ryan's age. "And I felt compelled to do something."

Within a couple of weeks, York had rounded up 40 contractors to build - free of charge - a three-room addition, an extreme makeover of sorts, to make the Kristoffs' lives a little easier while they wait for one child to die and fight for the other's survival.

The addition, worth $80,000 and designed by local builder Bruce Hayn, will have a handicapped-accessible bathroom with a 7-foot shower big enough for a wheelchair, a bedroom for Ryan and a room for a hot tub, donated by the Make A Wish Foundation, which grants gifts to seriously ill children. Not only will the tub help with muscle stiffness, it will loosen mucus in Ryan's and Alyssa's chests and help them breathe. The addition will have a pulley system mounted on the ceiling to help lift Ryan's limp, 40-pound body from room to room.

"I'm so overwhelmed," Pam Kristoff, 35, said, fighting back tears. "It's hard to believe people exist who would help you for nothing."

Her husband, Bill Kristoff, 37, said he doesn't want people to feel sorry for them. "We have a nice house. I work," he said. "There are a lot of people out there who have nothing. We are just kind of in awe of what they are doing for us."

For the past few weeks, various contractors came to help during their free time, on weekends, and on their lunch hours. "Everyone I talk to asks, `What can I do to help?'" said Don Sheak, an electrician working on the addition, which has already been framed.

"All it takes is five seconds of watching this family," Hayn said. "Whatever problems you think you have, they are minor

When Pam Kristoff, an elementary school teacher, and Bill Kristoff, a civil engineer, had their first baby, Katie, in March of 1999, they dreamed of having four children who would grow up in the quaint yellow colonial in a small subdivision surrounded by woods and farmland.

Katie, now 7, developed normally. Ryan was born on March 7, 2001, an easy birth, and he seemed healthy and normal like his sister. Then, between 4 months and 6 months of age, Ryan became extremely irritable, crying all day and night sometimes. His neck was weak, he never rolled over or tried to sit up, and he held his arms straight out, as if they were stiff. By October 2001, he was diagnosed with Krabbe disease. Doctors told the Kristoffs that he probably would not make it to his first birthday.

Krabbe disease, they would learn, is a rare inherited degenerative disorder of the central and peripheral nervous systems that breaks down the nerves' protective myelin coating, causing severe impairment to mental and motor skills. Only 200 to 300 families in the U.S. have children with the disease, which is generally fatal before age 2.

But the Kristoffs didn't accept the death sentence given to their son, and instead began to do their own research. They called Hunter's Hope Foundation, a charity started by former National Football League quarterback Jim Kelly, whose son, Hunter, eventually died of the disease. They learned that Duke University Hospital in North Carolina was leading the research on Krabbe disease.

Ryan was a candidate for umbilical cord blood transplantation, an experimental therapy, even though he had already been robbed of his eyesight, his ability to swallow and much of his ability to move.

"You had a little bit of hope that maybe we wouldn't bury my son when he's a year," Pam Kristoff said.

Since he had the treatment when he was 9 months old, Ryan's lifespan has increased, but he has no chance of surviving in the long term, said Dr. Joanne Kurtzberg, director of the Duke Pediatric Bone Marrow and Stem Cell Transplant Program.

Any one of his seizures could stop his breathing, and the Kristoffs have decided not to resuscitate him should he go into full arrest.

A staff of nurses helps the Kristoffs 80 hours a week, as do Pam and Bill's parents and their close-knit neighborhood.

Ryan has ways of communicating, such as sticking out his tongue when he doesn't like something. He receives physical therapy, lying on a mat or exercise ball, and likes to be read to, hear music or have his pudgy hands and feet dipped in finger paint.

After his nap, he is able to manage a weak smile, and he can move his head enough to cuddle into his parents.

"It's wonderful. It's a time when our baby boy comes back," his mother said.

Despite Ryan's grim prognosis, the Kristoffs badly wanted another child, and they were told the chances that a new baby would have the disease were only 25 percent. While carrying Alyssa, Pam Kristoff learned that the baby had the genetic defect that causes Krabbe disease. They say they never considered aborting the child.

"These kids were given to us. God gave them to us for a reason, and I was going to take care of them," Pam Kristoff said.

Since Alyssa was diagnosed before birth, her chances for receiving help from umbilical cord blood were far better.

Alyssa received her first transfusion before she was a month old. At age 2, Alyssa has already reached milestones her brother never did, such as sitting up, grabbing toys and saying words. Cognitively, she is a normal, social child, who loves balloons, stuffed animals and playing peek-a-boo, but she has been in and out of the hospital for the past several months for an unrelated intestinal problem that required surgery.

She is one of 17 children who have received the cord blood transplant, and one third of them are developing normally, Kurtzberg said. The oldest is 10. Another third have difficulty walking, and the last third don't walk well at all, she said.

Where Alyssa will fall in that range is unknown. The Kristoffs hope to bring her home from the hospital next month so they can live together as a family again.

York and the contractors know there is no guarantee the children will enjoy the addition long term, but, he said, "This is a family that deserves to have some help."

Donations to support Ryan's and Alyssa's care can be made to the Kristoff family, in care of the Colchester Rotary Club, Liberty Bank, 219 S. Main St., Colchester, CT 06415.

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