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."
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