Brothers with rare disease cherish the good days

• One in every 3%2C000-3%2C500 boys born each year has Duchenne%27s muscular dystrophy
• Disease involves loss of protein that allows the body to repair tears%2C wear in muscles
• Duchenne%27s %22exceedingly rare%22 in girls

BURNS, Tenn. -- Kids ask all kinds of difficult questions.

Is Santa Claus really just your parents?

Do pets go to heaven?

How are babies born?

Sonya and Terry Marlin are waiting for this one: Am I going to die?

The Marlins' two sons — Jonah, 8 and Emory, 6 — live with a rare form of muscular dystrophy that is quickly robbing them of muscle mass. In their teens or 20s, it will stop their hearts.

"Only God knows how you're going to die," Terry Marlin said. "But the end result is if you live long enough, this disease will kill you. That's going to be a hard question. I have a feeling it's going to come a lot sooner than we think it is."

The diagnosis

It is the worst thing that could ever happen: the call Terry Marlin received from his wife three years ago as he was traveling to Arkansas to meet with a customer of his manufactured diamond tools business.

"I was on this same road trip," Terry said recently, calling from his cellphone as he drove toward the Arkansas customer.

When Sonya called, she told him to pull over. Terry remembers the exact gas station where he stopped.

"When she told me, all we knew at that time is that they thought (Jonah) had muscular dystrophy. But she said, 'You need to come home' because they were going to test Emory the next day. 'They think this is a genetic disease.'

"I had made it as far as Memphis and I just turned around and came home. When I pulled over, all I had at that time was a Blackberry, ... and I just started Googling 'muscular dystrophy.' … I'll never forget that call. It was Dec. 17, a week before Christmas."

Jonah, their oldest, had never developed the way other kids do. As a toddler, he was slow to walk. When he did walk, he couldn't run or jump. With his pronounced calf muscles, this puzzled his parents. He looked like he would become an athlete.

"I saw his inability to run, but I didn't see it was a disease. I really just thought he was going to start running one day," Terry said.

But Sonya is a nurse, and though she was a first-time parent, she knew this wasn't right. She and Terry took Jonah for testing.

Doctors feared Jonah may be among the one in every 3,000-3,500 boys born each year with Duchenne's muscular dystrophy. It is a degenerative condition involving a loss of dystrophin, a protein that allows the body to repair tears and wear in the muscles.

"The muscles are not able to repair themselves and keep up with that normal process," said Dr. Larry Markham, the boys' pediatric cardiologist at Vanderbilt University Medical Center.

Markham said that's why, when they're toddlers, "They may walk a little bit later (than most) but typically are strong enough to walk. And so the diagnosis is generally made when they're around their peers at age 5, 6, sometimes sooner than that, and they're not able to keep up with their peers."

The vast majority of boys with this condition live into their 20s, commonly dying of heart failure.

The overwhelming majority of Duchenne's patients are boys. The disease is the result of a genetic abnormality passed along on the X chromosome by the mother, either through her bloodstream or a mutation in the egg. Because boys have just one X chromosome, they are exponentially more likely to inherit the abnormality. Because girls have two, they may be carriers but, Markham explained, it is "exceedingly rare" for them to manifest the disease.

Painful milestone

Duchenne's patients eventually require a motorized wheelchair to get around, which is what happened to Jonah earlier this year after a precipitous decline.

The family had gone to Oklahoma City for Sonya's cousin's wedding. When they left for the trip, Jonah struggled to lift himself — just as his little brother is now — but he could walk. By the end of the long weekend, he was nearly immobilized.

"He kept saying his legs felt like Jell-O," Terry said. "I was carrying him around a lot more and then, all of a sudden, he couldn't stand up anymore. We attributed it to a busy weekend. He did a lot of swimming, a lot of running around, and we thought maybe he was tired. But when we got home, he was just done."

The Marlins bought Jonah a motorized scooter.

"It wasn't like it was literally overnight, but it was very quick," Terry said.

The scooter helped Jonah get from point A to point B, but it was positioned too high and he could not pull up to a desk or table at school. Terry's friends rigged it with a tray so Jonah could have something on which to work.

The couple began the process of finding a more permanent solution — a wheelchair, a van with a ramp, modifications to their home.

Some parents would have seen the wheelchair as a tragic milestone.

"With the chair, it can be 'Oh, my gosh, look at this poor boy. He's in a wheelchair'," Markham said.

But boys with Duchenne's often view the chair as a source of independence.

"They can keep up with their peers," the doctor said.

Jonah's chair is hydraulic and can lift him, like a dentist's chair, to help him reach things that he would normally have to stand to reach. Its turn radius is much sharper than the scooter, so he can pivot in tight spaces. What he can't do in his chair is lean over to pick up things he has dropped. When that happens, others must retrieve items for him.

Very often, the person at his beckoning is his brother.

"My brother can't walk and pick things up," Emory said. Beyond that, he added, "I don't want to talk about it."

Bond is unique

Parents with more than one child understand how the younger ones are enamored with the older ones. That is true with Jonah and Emory, too.

But Jonah and Emory have a little something extra. Right now, Emory is the arms and legs for Jonah.

"They're always together," Terry said. "They're not able to wrestle around like some kids are. But Emory will do anything Jonah asks, and Jonah doesn't mind asking. You can just see the bond between them."

On a warm day near the end of the summer, Jonah and Emory discussed what they loved about each other.

"He's funny, nice, silly, behaved. He jokes around, and that's all I got," said Jonah of his little brother.

Said Emory of Jonah, "My brother is funny and does funny stuff and does jokes and jokes around and likes to build puzzles, and he likes to paint and build bird houses, and he's nice to me."

"And I'm fun," said Jonah.

"And he's fun," Emory said.

Though Emory understands he has the same disease as Jonah, he's too young to grasp entirely what that means.

"He's always questioned why he's falling," Terry said. "He knows he's got the disease, and we tell him he's got the disease, but it's hard for a young kid to put the disease and falling together."

Terrible questions

But Jonah understands.

In the Marlins' home, he watches his little brother for signs he is beginning to struggle the way he himself did.

"He's not like me now, but he will be," Jonah said. "I'm sad for that. I feel like he should be able to walk and I should, too."

"That's an honest answer," his father said.

If Jonah understands the inevitable finality of Duchenne's — and his parents say he may — he hasn't articulated it. He does ask scary questions, though, yet he is increasingly accepting of the answers.

A few months ago he again asked why his legs were so weak, and he talked about when he would be out of the scooter.

"We had to explain it to him again," Terry said. " 'So I'm never going to walk again?' he asked. You could see the wheels turning."

The Marlins know their sons' questions will get tougher as their conditions worsen.

"We have not yet had the talk about, 'If you live long enough, this disease will kill you,' but we've always been open about it," Terry said. "I want them to find out as much about it as possible. It's their bodies, and they need to know what it's going to do, but I want them to be at an age that they can understand."

Faith and opportunity

Ironically, having less time can bring more opportunity.

Why wait to take that trip to Disney World? Why wait to go horseback riding? Why not ride in a helicopter? When you are healthy, you find reasons to wait. The Marlins, who have discussed taking the boys rafting through the Grand Canyon next summer, have found reasons to live.

"This has put life into perspective more than anything else could put life into perspective. You appreciate life so much more when you go through this," Terry said.

On Jan. 30, Jonah will undergo experimental surgery to help him stand again. Doctors will cut just below his calf and stretch the muscle so Jonah can stand without a device to hold his hips in place. Jonah is scared — and rightfully so — that the surgery will hurt.

"I told him the whole goal was to maybe get him standing again," Terry said.

And so he agreed it would be worth the pain.

Of course, life will continue to grow more challenging as the boys age. Care will become more intense, invasive and infused with tough questions and decisions.

But Terry believes only God knows what is ultimately in store for his boys.

And so it is that his wife offers this:

"God is teaching us a lesson we are still trying to learn."