Tualatin boy with rare syndrome still beating the odds
Jake Dering isn't supposed to be walking. He's not supposed to be a quick-witted, strong-willed seventh-grader at Hazelbrook Middle School. In fact, according to the prognosis doctors gave him at birth, he shouldn't be alive at all.
Jake Dering's story is one of defying the odds — again, and again, and again.
It's a story to which you may have read the early chapters in The Times several years ago (an article from when he was nearly 2, and another from when he was 9). But it's a story that is still being written, and this is just the latest recapitulation of it.
Unusual bone growth causes physical problems
Jake, who is now 12, was born with severe congenital scoliosis and several displaced joints. He lives with an extremely rare condition called otopalatodigital syndrome type II. Because of the condition, Jake's bone growth is abnormal.
"For whatever reason, Jake grows bone fast and hard and difficultly, and when you take it out, he replaces it, and it's a problem," explained his mother, Chellie Dering. "I mean, his knee fused because of that. The same thing happened in his spine."
Within hours of being born, Jake said, he underwent his first surgery. He has had 24 more to date and counting — another spinal surgery will be needed soon, said Dr. Ivan Krajbich, Jake's surgeon at Shriners Hospital for Children in Southwest Portland, for which Jake is a patient ambassador.
"We had to deal with what was most important — kind of triage each thing along the way," Chellie said.
Scoliosis, an uneven curvature of the spine, is not an uncommon condition — as many as 4 percent of children between the ages of 10 and 18 experience what is called "adolescent idiopathic scoliosis," according to the Scoliosis Research Society. However, Jake was born with more than an 80-degree curve in his spine, Chellie said.
"As he grew and became weight-bearing, it became worse and worse," Chellie said. "He had such a severe curve in his spine that he was hospitalized with pneumonia at least twice a year. … That curve was so bad, it was crushing his lung, and so his lung was down to, like, 41 percent lung capacity."
Magnetic rod surgery was life-changing
In November 2014, Jake underwent surgery at Shriners Hospital to have rods inserted into his spinal column, straightening it and holding his vertebral column upright. Since the surgery, Chellie said, his lung capacity has more than doubled — his bouts of pneumonia have stopped, and the chronic back pain he experiences has lessened.
The rods in Jake's back are different from traditional "spinal instrumentation," Krajbich said — they are engineered in such a way that they can be manipulated without surgery using powerful magnets. Since his surgery, Jake has returned to Shriners Hospital every six weeks for an adjustment, lengthening his spine by a millimeter or two at a time.
"It's not, like, painful," Jake said of the procedure, which he described as producing "clicking" in his neck. "It's just kind of uncomfortable."
The efficacy of the magnetic rods has lessened lately, though, and Jake soon will need to undergo surgery again, which Krajbich said may correct vertebral fusion that has occurred since his last operation. The rods may need to be replaced, too, he said.
The trial-and-error approach to addressing Jake's condition is nothing new for the Derings.
"Dr. Krajbich definitely tried lots of different things with Jake," Chellie Dering said. "And that's one of the things I really appreciate about Dr. Krajbich is that he's conservative but not to the point of not acting on what we need to do. I mean, he'll try noninvasive techniques first and then say, 'OK, that didn't work. Let's progress on.'"
"There isn't any sort of an easy cookbook recipe to treating them," Krajbich said of his patients. "You know, you have to try to find the best solution for some of these children."
But Krajbich has learned from Jake's case. Since operating on him in 2014 to insert the magnetic rods and seeing the operation's positive outcome, he said, he has performed the same surgery on two other young patients.
Despite obstacles, living life like any other kid
Although Krajbich's specialty is in treating spinal deformity in children, the doctor said every case is different, and there's no tried-and-true method for treating them all.
"Although magnetic spinal rods are a huge step forward, it's by no means a solved problem," he said.
But the Derings are grateful for what Krajbich and Jake's other doctors have been able to accomplish.
"He's had so many surgeries and so many life-changing situations, for sure," said Chellie, who called Krajbich one of her favorite surgeons and praised his openness.
"There's no question that the biggest credit for this goes to Jake's mom here," Krajbich said. "She is a huge advocate and a perfect parent to work with, for sure."
Jake's disabilities are entirely physical, Chellie said, despite doctors' warnings since before he was even born that he could grow up with learning disabilities or other psychological issues. And while he still uses a wheelchair to traverse longer distances — it has an electric motor synced to his Bluetooth-enabled wristwatch — and relies on crutches to move around much of the time, he is capable of walking on his own, something he wasn't able to do until the age of 4.
Jake even plays sports. He's a good golfer and enjoys basketball and dodgeball, which he said, with a mischievous glint in his eye, gives him the opportunity to "wreck some kids."
"When he first asked me (about playing dodgeball) last year, I was like, 'Excuse me, what? You want to do what?'" Chellie remembered. "He's like, 'Yeah! I mean, Mom, if it's terrible after the first class, I just won't do it anymore.' And our philosophy with Jake is always 'do whatever you can until you can't.'"
She explained, "Jake is supposed to unload the dishwasher. He's supposed to sweep. And his back hurts after it, but I'm like, you still have to do these things. So we have always said to him, 'We're not going to treat you any differently.' … But having said that, it is a fresh perspective for me every day when I see him overcoming, and doing, and not complaining, and wanting to do everything everybody else does. So fresh perspective is what I get to see every morning."