Jeffrey D. Allred, Deseret News
TAYLORSVILLE — Before their first child was born, Rachel and Ryan Glad shopped for the happy toys and trinkets that nearly shout a baby's on the way. At the same point in their second pregnancy, they picked out a single outfit, one blanket and a tiny coffin. They signed an autopsy permission form.
Doctors had told them halfway through the pregnancy that their baby had osteogenesis imperfecta (OI). Brittle bones. Shocked, they'd still been learning all they could about it and were ready to welcome him just the way he was. Then, two weeks before he was due, doctors said the baby was too small and too frail to survive the rigors of his own entrance into the world.
"It took our breath away," said Ryan. "My parents looked at cemetery plots for us."
"It was the end of all hope and planning," Rachel said.
As she tells the story, Rachel, 30, snuggles that child, Nathan, who is now 4, stroking his arm gently with her thumb. The doctors got only part of his story right: He really is too small and too frail by normal standards. His little brother, Jason, 3, outweighs Nathan by 11 pounds, and Nathan's size doesn't even make the growth charts in the pediatrician's office.
But he's just right to fit into her arms — and her heart. As they cuddle, they look alike: petite and pale-skinned, with soft, downy hair. And as he tells her again how much he loves her, his smile is actually too big to be contained by his surroundings.
On a crisp November day, a doctor delivered Nathan by an emergency C-section that was also an exercise in extreme care because they all realized the newborn was as delicate as a robin's egg.
He screamed lustily as he emerged.
"It was like he said, 'I'm coming and I'm living,' " his mom said, smiling. "He's been by my side ever since."
OI is a collagen disorder and since bones get their strength from collagen, those who have OI have bones that break easily — sometimes, for no apparent reason at all. Nathan is at the severe end of the OI spectrum. It's broken down into numbered types based on the quality of the collagen and how much of it is produced. Type Is, for instance, make normal collagen, but not enough of it. Type IVs make enough, but poor-quality collagen. Rachel compares the bone from Nathan's severe OI to a brick wall that lacks mortar. It falls apart.
Found in an estimated 1 in 20,000 live births, OI has gone by various names, from Ekman-Lobstein or Vrolik syndrome to "glass-bone disease." It's not new. The name osteogenesis imperfecta was around as early as at least 1895. And it has been found in an ancient Egyptian mummy from 1000 BC. Several articles suggest that the Norse king, Ivar the Boneless, had OI.
There are also differences in the lifespan expectations as well. Children with Type II, for instance, seldom survive their first year. And some children, like Nathan, are hard to classify into an exact type.
Some children with OI improve as they get older.
"He doesn't produce good collagen," Ryan said. "So his improvements won't ever be much. Maybe more muscle to protect his bones."
Not long ago, Nathan picked up a plastic toy helmet and shattered his collar bone. He's broken something — usually an arm or leg — about once a month his entire life.
"We've all had moments when we've been the cause of him breaking," said Ryan, 31. "It's not an easy thing."
When Nathan's sister and best friend Courtney, 6, caused a broken leg, she was traumatized.
"We had to sit down and tell her that it's nothing she did on purpose. It just happens," Ryan said. "But it's a little difficult to get over."
Malformed bone is a given with the disease. Surgeons tried to mitigate femur curvature by putting a rod in his leg, but one of his bones crumbled and they changed their minds. He broke a leg once while just sitting in his rear-facing car seat. Ribs, jaw, every long bone in his body — all broken. He can break without really doing anything. Only his pelvis and head, and the little bones like the ones in his fingers and toes, have not been injured — as far as they know.
"We stopped counting a long time ago," Rachel said. "And I know we've missed some."
That it happens so often does not mean it happens pain-free. "He has high tolerance because he's dealt with it so long," she says. "But it really hurts. After a break, it takes two to three days to get back to being goofy, playful Nathan."
But when he prays, he thanks his Heavenly Father for his body. "He's so grateful for it," Rachel said. And so are his parents.
While OI can be passed down from an affected parent, it can also result from a genetic mutation particular to that individual. That seems to be the case with Nathan, since no one else in his family has it. It's autosomal dominant, so his own children would have a 50 percent chance of inheriting it.
Nathan can't walk, but he moves fast lying down, often grabbing his feet to boost his locomotion. Sometimes he looks like he's swimming on his side. He has a funny, cheerful voice, not quite squeaky but not robust.
Rachel said that's an OI voice, as typical of the adults who have the condition as with the kids.
In preschool at Westbrook Elementary two days a week, he uses a power wheelchair. At home, he sometimes uses a standing wheelchair his dad modified for him. He straddles a little saddle, his feet touching the floor as he bears just slight body weight, which helps build bone density.
When she explains OI to others, Rachel describes snowflakes. Fragile. Unique. And quite lovely.
"I like to appreciate him for what he can do, rather than what he can't," Ryan said. "He has a giant personality. He's a thinker. And the things he comes up with and says."
Recently, Rachel was getting ready to cut his hair.
"Nathan, are you ready to get your hair cut?" his dad asked him.
"Not all of it," the boy said. "I don't want my brains to get cold. So I can think."
Nathan is very articulate at 4, though you have to listen carefully to that little voice. In a way, it fits him. Since so many breaks leave little opportunity for growth, he looks much younger than his age, but he hates being called a baby and he's not shy about saying so. He is a big boy in a genuinely tiny body.
At first, it took both Rachel and Ryan to change Nathan's diaper because they were fearful and tentative. It was six months before she tried to pull a shirt over his head; early on, she made sure everything snapped. But their determination to see that he has a full life with all the experiences that it offers soon made them fearless.
Recently, they watched — smiling and with fingers mentally crossed — as Nathan went down a slip-and-slide at a special camp for people of all ages who have OI and their families. At Camp Attitude, in Foster, Ore., Rachel gently placed him in an inflatable raft before sending him down the hill.
Ryan and Rachel have allowed him in a tethered hot air balloon, on a gentle horseback ride, even on jet skis at the Oregon camp, where every precaution that can be taken is by those familiar with OI. Still, there are no guarantees.
"He's going to break anyway. It might as well be doing something fun," his dad said.
Fragile is a word that's sometimes overused. There's no better word to describe Nathan Glad. Despite that, his parents have chosen to let him live a robust and experience-rich life, a decision that took significant courage.
"I jumped in when he was born," Rachel said. "I had to. There was no way I wasn't going to learn to hold my baby."
She's watchful, though, and careful about to whom she entrusts him.
"It's those who are comfortable — and easy," she said. After all, someone nervous and tentative handling a child who shatters is a recipe for disaster.
Like others with OI, Nathan has a short body, though his head is normal size and he's very smart. Those with OI are also plagued by loose joints and muscle weakness. The medical journals speak of brittle teeth and curved spines, as well as eyes whose whites are sometimes stormy grey or blue. Rachel and Ryan believe his eye whites become a pale blue when a fracture is imminent. He will have to wait to see if some of the traits, like hearing loss, develop. That's typically two or three decades down the road.
He has chronic bone pain and he must exercise every day, stretching out on his belly, then placing his hands so he can arch his back and raise his head, strengthening both arm and neck muscles while stretching his back. It's hard work. And though his head is only in the 1 to 2 percentile on growth charts, it's big for his body.
Several times a year, he goes to Shriners Hospital for an intravenous injection of a treatment for osteoporosis. It eases the chronic pain for a while, though not too long.
Still, the little boy's surname is a great fit. He's pure sunshine, happily demonstrating his robot moves or how he can lick his own elbows (his arms are too short to reach much, but the lack of decent collagen makes them very flexible and that aspect, at least, amuses his mom).
A fracture makes him quiet and more tentative at first. Right now, he's been several weeks without one and is more playful and rambunctious. Yet, compared to most kids, he's relatively subdued in action, though not in spirit.
His high tolerance for pain is a good thing. Last August, he was sitting in a chair and fell forward, breaking both his arms. He was heartbroken because he couldn't hold his treasured little white teddy bear. He couldn't feed himself. Really upset, he told Rachel, "This one's all better," so she'd unwrap one of the arms and let him use it. "He'd rather have the pain than have both arms wrapped up," she said.
Sometimes he cries when he breaks. Sometimes he just seems less Nathany, more subdued and sad. His pain tolerance is exceptional, but the fractures hurt him. His sunny nature goes behind a cloud. Whether it's a storm cloud or not depends on the bone.
Often, depending on the fracture, they just have to let it heal. His bones are too weak to support casts.
It's a balmy May day — a rare dry, sunny one in a recent string of rainy Saturdays — and Nathan's taking his sister and brother for a ride on his wheelchair while they wait for his turn at bat. He plays in the Miracle League on the Angels team, with kids from 3 to young adult who have a variety of physical and mental challenges. Today, they face off against the Braves.
Nathan's mom is the coach, though she admits she's no baseball fanatic. Her son loved it last year, but when she called about the league this year, she was told they were looking, so far without success, for a coach.
"I said I guess I could do it," Rachel said. "I didn't want him to lose the experience."
Courtney, 6, is his best friend in the world. She's careful with him, gentle, but unafraid as she tosses him the ball and teases him. Right now, though, she's standing on one of the giant casters on the back of his chair, next to their little brother Jason, as they make figure 8s on the asphalt at the Gene Fullmer rec center.
Some of the children make the home run circuit in wheelchairs, like Nathan. Some use walkers or walk painstakingly. One little girl holds her mom's hand as she circles.
It's a game where no one sits out, no one gets booed and everyone makes it back home safe.
Email: email@example.com, Twitter:loisco
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