Photos and text by Paula Ouder
Two weeks before his birth, doctors delivered James William Dixon III's
family shocking news after performing an ultrasound.
"They said, 'Well, basically, your baby doesn't have a face. We don't
see jawbones. We don't see ears. We don't see eyes. We see large clefts
all over him. If he's even born and takes his first breath, you're going
to be lucky,'" recalls his mother, Shannon.
as he came to be known, was born two months premature. Shannon, remembers
feeling relieved when she saw him for the first time. Doctors had shown
her and her husband "horrifying" pictures of stillborn children
with open skulls and sinus cavities.
"We were imagining no eyes and big holes in his face," Shannon
Though his condition wasn't as dire as physicians predicted, 18-month-old
J.D. does not look like other children and never will. He has the fourth
most severe case of Goldenhar's syndrome in the United States, judged
by the malformations of his head and face.
right eye appears to bulge from his head. In reality, his eye is in the
right place, it's his forehead that is pushed back. Where his left eye
should have been is a small, empty circle flanked by numerous eyelashes.
J.D.'s ears never fully formed and have no openings. On either side of
his head is a small, fleshy bud that more closely resembles an adult earlobe
than a baby's ear.
He is legally deaf and legally blind. He breathes through a tracheostomy
tube in his neck and eats through a gastric tube in his stomach.
Despite his problems, J.D. has the love of a devoted family, a medical
team that goes out of its way to care for him and a personality that shines
through his disfigurement.
He plays with toys, loves to be held, responds to people and has a fiery
Goldenhar's syndrome is rare birth defect. Some researchers estimate it
occurs in one out of every 3,500 to 5,000 births. Diagnosing Goldenhar's
is not easy, because the condition has many characteristics that are similar
to other syndromes. It is marked by a combination of eye, ear, jaw, skull
and vertebral abnormalities, tissue growths and facial clefts. Some children
also have heart and spinal problems. Other complications are possible.
The right side of the face is most commonly affected.
Dr. Adel Al-Asfour reports that Goldenhar's patients are "of normal
intelligence or mildly retarded" and the disease has no "heredity
patterns, sex or racial predilection." Some researchers have found
weak genetic links in the occurrence of Goldenhar's syndrome. U.S. military
personnel who served in the Gulf War have had a higher-than-average number
of children born with the disorder. Another theory suggests that an intrauterine
hemorrhage early in pregnancy is the cause of Goldenhar's.
joy to fear
J.D. was born to Lake City, Fla., residents James and Shannon Dixon, now
aged 31 and 23, respectively. He is his mother's first child and his father's
third. Shannon says she felt a tremendous amount of guilt when an ultrasound
revealed J.D.'s problems. James' other children were healthy, so she blamed
herself in a way for what was happening.
"That was at 29 weeks we had that ultrasound done," Shannon
explained. "We were just devastated. I wanted to give James a perfect
little boy, then to find out he may not even live to make it home. We
cried the whole way home. We had to stop the truck several times just
to sit there and think. 'Did we want to end this pregnancy? What are our
lives going to be like? Would we have to change everything at home?'"
After talking with their parents, they decided to give J.D. a chance.
"We all just said, 'We're having him. If he turns out, fine. If he
doesn't, at least we tried.' "
The moment of having him came all too soon, as Shannon went into preterm
labor. When he arrived, she was afraid to see her newborn for the first
parent wanted anyone to take photos of their young son as they gradually
absorbed the magnitude of his problems. That restriction gradually fell
away as they joined J.D. in a fight for his life that continues today.
Shannon says she has learned to speak out to get what her baby needs and
to find proper care for him. She's not afraid to discuss his condition
and takes J.D. out in public at least two times a week for doctor's visits
She said many doctors advised her and James to sign a "do not resuscitate"
order and walk away from J.D. to let him die in the hospital, saying he
wasn't worth saving.
"It was horrifying the thoughts we were having of how bad it was
going to be (when he was born). And it wasn't that bad. It was a ton of
weight off both our shoulders. It was, 'Ok, we're going to get through
this.' It wasn't a 'what if' any more."
J.D. has a host of unusual medical problems and requires constant monitoring.
One corner of the Dixons' living room resembles a small hospital with
machines, tubes and wires snaking around J.D.'s crib. Without around-the-clock
care, he would quickly suffocate from complications of tracheostenosis,
a narrowing of his airway.
though she has to get up some nights as often as every 30 minutes to suction
fluid from his trachea, Shannon calls J.D. her "good baby."
Since he was her firstborn, she says didn't perceive him as any harder
or easier to care for than a healthy child because she had never had an
infant to compare him to.
"He's my boy. He's rotten. He's been very exciting for us. He's brought
meaning to our lives."
She said the struggle for J.D.'s survival has brought her family closer
"You don't take advantage of having children and being the perfect
family with a dog in the back yard. You take advantage of him living today
and looking forward to tomorrow," Shannon says.
"It's been a real joy."
When J.D. was 3 months old, the couple decided to try to have another
child. Their efforts produced a perfectly healthy baby girl they named
Shannon said their motivation was two-fold. They knew they wanted more
children and couldn't bear the thought of an empty crib should J.D. die.
They also had been told that J.D. would develop better if he had a sibling
to learn from. So far, it's working. Sierra has taught him to suck his
thumb, and the Dixons hope he will learn to sit-up when she does.
"He needed the help and he will learn more from her than he will
from us," says Shannon.
the future holds
While their attitude is amazingly positive, dealing with J.D.'s illness
has been difficult for his family physically, psychologically and financially.
They are reminded of the fragility of their son's life daily and have
faced many hospitalizations he was not expected to survive. They consider
his life to be a blessing and make the most of their own lives. They are
hopeful for his future and insist that he be treated like a baby first
and a patient second.
"We'd rather have him the way he is than not at all," Shannon
She craves simple things for her son. "I can't wait for him to eat
his first cookie," she says with a giggle.
and James want him to learn to walk and talk and accomplish whatever he
is capable of.
"I just want him to be able to function normally, no matter what
he looks like," Shannon explains. "He could stay the way he
looks right now, I don't mind. I just want him to be able to sit, stand
(and) walk -- do the things we take for granted. That would make me happy."
With surgery and therapy, he stands a good chance of making it.
Shannon wishes she would have found expert care for J.D. as soon as he
was born. Goldenhar's syndrome is rare, and the severity of J.D.'s case
puzzled local doctors in Gainesville, Fla. Surgical treatment could have
started immediately instead of months later. She encourages parents to
do research to find the best doctors, nurses, therapists and the best
facilities. Above all, she advises families with disabled children to
"No matter how bad people say it gets, look for the bright side.
It gets better. We were told many times, 'Just let him die; he's not worth
saving.' He is worth saving. He's a child, no matter what he looks like.
Just listen to your heart."