The amazing baby Jake
It’s been a tough year for this
soon-to-be-1-year-old, but you’d never know from his upbeat attitude
A child’s first birthday is always a special time for a young family, but this
coming Tuesday will be a particularly joyous occasion for Steve and Chellie
Dering of Tualatin.
Their son Jake – born Oct. 18 with a rare genetic disorder – has fought through
his first year on earth despite daunting odds.
It has been a year during which Jake has faced down more life-threatening
situations and excruciating pain than most of us will in a lifetime. To the
amazement of his parents and those around him, he has persevered with a smile
on his face.
“Jake is just a very unique guy in what he brings to the world,” says Steve.
“It’s amazing how many people have come to us and said how much of a difference
Jake has made in their lives.”
Born with Oto-Palatal-Digital Syndrome, Jake suffers from an underdeveloped
trachea, or wind pipe, that has led to numerous episodes of respiratory
failure.
Aside from causing the tracheal malaysia that endangers his breathing, OPD also
afflicts his bone structure and his hands and feet. Only about 20 to 30 people
on the planet have the condition, leaving doctors and scientists with very
little information about the disorder and how to treat it.
The Derings first found out something was wrong during Chellie’s pregnancy.
During a routine fifth-month ultrasound, doctors noticed that the proportions
of their child’s body were unusual.
“There are things they measure, like bone length,” remembers Steve. “There were
some red flags that had come up.”
“They said, ‘There’s definitely something not quite right here,’” says Chellie.
More complicated
Initially, doctors believed Jake had a form of dwarfism. But as the fetus
developed, new ultrasounds revealed the problem was more complicated.
“It was pretty disheartening,” says Chellie. “Every time we went in, they found
something new or something different, and we weren’t sure exactly what it was.”
When Jake was born, the collection of symptoms indicated to doctors that he had
OPD. The syndrome can take many forms, and each case manifests itself
differently: Some people have cleft palate, cranial malformations, deafness and
mild retardation, while in others the disease takes an entirely different form.
“There’s a list of characteristics with the syndrome of OPD,” explains Chellie.
“Some kids have all the things, some kids have just a few of the things.”
Jake’s form of the disorder has dramatically affected his limbs. The bones of
his upper arms and upper legs are shorter than normal, and his joints and hips
are dislocated. This prevents his arms from fully extending, and caused his knees
to originally bend in the reverse direction. The skin of his middle finger and
ring finger is fused together, which occurs in his toes as well. Also, he was
born with club feet, a condition where the feet turn inward and downward.
To correct the problem, Jake has undergone several surgeries to his feet and
legs. One of these involved attaching external fixators, which are expandable
metal bars connected to pins that go beneath the skin and penetrate the bone.
During the three weeks Jake wore the apparatus, Steve gave a daily
three-quarter turn to a screw on the fixators, which served to lengthen the
boy’s femur bones prior to knee surgery. Chellie, meanwhile, cleansed the pins
that were fastened to the bones beneath Jake’s skin.
“The pin sites were like an open wound. I had to clean them with hydrogen
peroxide and saline,” said Chellie. “It was so painful for him, but we had to
do that to keep infection out.”
Torture devices
These ordeals taught the Derings a great deal about their son’s resilience. Holding
a photo of Jake that was taken while he had the external fixators, Chellie
explains that the picture embodies her sons’ spirit. Despite the contraptions
on his legs that look like futuristic torture devices, Jake is sitting up and
looking into the camera, grinning widely.
“That is Jake,” says Chellie, proudly. “He would turn over onto his stomach,
and be laying on [the fixators] with the pins driving into his legs. He would
just look up at you.”
After his last knee surgery in August, Jake developed a major staph infection
in his leg that went unnoticed beneath his cast, leading to a five-day stay at
the hospital.
Nonetheless, the series of surgeries have proved successful, and have provided
Jake with a birthday gift that most 1-year-olds take for granted: a pair of
functioning legs that he will one day be able to walk upon.
Although the surgeries have been somewhat nerve-racking, they pale in
comparison to his repeat hospitalizations for respiratory failure. The first
incident, the Derings agree, was the most traumatic.
Four weeks after Jake was born, the family was traveling to Chellie’s sister’s
home in Beaverton for Thanksgiving. Jake was sitting in the back seat, tucked
in a car seat with a blanket and a hat on. He sounded stuffy, and when Chellie
reached around her chair to check on him, she noticed that the tiny face
beneath the hat was completely blue.
“I ripped him out of his car seat and pulled him into the front seat with me,”
said Chellie. “The minute Steve could look over at him, he knew something was
very wrong.”
Tires squealing, Steve drove them to nearby Meridian Park Hospital while
Chellie performed mouth-to-mouth resuscitation. Despite her efforts, he
remained blue and began foaming at the mouth. Once they reached the hospital,
however, a tube was placed down Jake’s throat and he began breathing again.
Luckily, he had not gone into cardiac arrest.
Nonetheless, it took hospital staff 40 minutes to fully stabilize his
condition.
Since then, three similar episodes have landed Jake in the intensive care unit.
He has been hospitalized for respiratory failure another 12 times, but in these
cases his mother and father were able to recognize the problem before it grew
too dangerous. Still, it has often involved hurried drives to the hospital with
the steering wheel in one hand and an oxygen mask over the boy’s face in the
other.
“With Jake, we say he goes zero to 60 real quick,” says Steve. “When he gets a
cold, it affects him very quickly. We’ve gotten much better at recognizing when
we’re over our head or not.”
‘It kind of flops’
The reason for these incidents is that Jake’s wind pipe is flaccid and prone to
collapsing. When he develops an illness, mucus aggravates the problem. Steve
compares the regular human trachea to a straw; you can easily breath in and out
of it due to its rigidity. In Jake’s case, it’s as if “you took a balloon and
cut off the fat part,” Steve explains. “There’s nothing to hold it open; it
kind of flops.”
The good news is that as Jake grows older, his trachea gradually becomes
firmer. Eventually, he will outgrow the problem altogether by the time he is
about 3 years old.
“Every day he grows, that pipe gets stronger and more rigid,” says Chellie.
Steve and Chellie have plenty of other things to be grateful for as well. Their
daughter Sidney, 2, is a healthy, robust toddler who has fully embraced her
little brother.
“She’s in love with him, and he watches her every movement. The thing that
makes him the most happy is watching her,” says Chellie. “He lays on the floor
and she’ll run around and jump, and he laughs and giggles.”
While some children are jealous of their younger siblings – especially ones who
receive a great deal of attention – Sidney can’t seem to get enough of him.
When his raspy cry is heard over the baby monitor, her eyes light up. “Jake!”
she whispers excitedly and points at the device.
“Should I go get him?” asks Chellie.
“Yeah!” she answers in the same eager whisper.
The Derings agree that Sidney’s experience with Jake will shape her into a more
caring, compassionate person. Having a vigorous, athletic older sister may work
in Jake’s favor as well.
“If anyone picks on Jake, for sure she’s going to beat them up,” says Chellie.
Also promising is Jake’s mental aptitude. Children who are neurologically
disabled often are unable to grasp cause-and-effect, visually track objects or
react to basic stimuli, but Jake is meeting all his developmental mileposts.
All signs indicate that his intellect hasn’t been affected by OPD.
‘There are dark days’
When a light cloth is thrown over is head, and the question “Where’s Jake?” is
posed, he rips off the cloth and laughs furiously. In other families, this is
just cute. For the Derings, it’s both a relief and a reminder of the joy they
have found amongst much anxiety and sorrow. This is their guiding philosophy:
to find reasons for optimism in a situation that is outwardly negative.
“There are definitely dark days. There are days when I cry and I’m frustrated,
and I’m not really happy about it,” says Chellie. “But what’s the alternative?
This isn’t going to change. We can be miserable and have a dark cloud over us
and be frustrated, or we can try to be positive and move forward and enjoy what
we get.”
The years ahead aren’t going to be easy for Jake Dering. Surgeries to separate
his fingers and possibly to repair his dislocated hips await. Because he can’t
fully extend his arms and because of the surgeries, learning to crawl, walk and
perform other necessary day-to-day duties will be immeasurably harder for him
than for other children his age.
The future is full of burdens, and no one can deny that. But, as long as he
retains his ability to smile in the face of adversity, Jake’s future will be a
bright, hopeful one as well.
Anyone interested in helping Jake can visit www.jakesake.com. Steve and Chellie
have 4 JAKESAKE bracelets available to raise awareness of OPD and other rare
disorders and to help with Jake’s medical expenses. Suggested donations for
bracelets are $3.