But through an online support
group, Mikayla's parents heard of two Australian
neurosurgeons who operated on and apparently cured several children
with HH using an innovative brain surgery. In February 2002, the
Australian doctors taught this procedure to two surgeons at Barrow
Neurological Institute in Phoenix, Ariz.
At that point, Sarah knew she could not let
her daughter suffer any longer. The surgery is now scheduled for
July 29 in Phoenix.
A Danger
Within
Mikayla's tumor, the size of a
small strawberry, sits close to the hypothalamus, a tiny structure
in the brain. The hypothalamus balances and regulates blood
pressure, body temperature, fluid and electrolytes. It also
controls appetite, vision, memory, general arousal, rage,
aggression and embarrassment.
Hypothalamic hamartoma is caused by gene
mutation that starts at conception.
HH tumors cause victims to have laughing
seizures, behavior problems, early puberty and epilepsy. When the
child reaches 4 to 10 years in age, the seizures become longer and
more severe. At this stage the child may begin to mentally
regress.
"She's pretty much like a normal kid -- right
now," said Sarah. "Her future can change."
Mikayla is a pleasant 4-year-old, who enjoys
riding her bike, playing tag and playing with her dolls and her new
puppy.
"My mom likes to be a mom -- she already has
two babies," Mikayla said, referring to herself and her 3-year-old
sister, Amiya.
Sarah has sacrificed much for her daughter
since she was born.
"I noticed something was funny from birth,"
Sarah said. "No one listened to me."
When she noticed Mikayla was making strange
grunting noises, she took her to Roseburg pediatrician Aric
Groshong. Because Mikayla did not exhibit the signs in front of him
and the family was not overtly concerned at the time, the doctor
advised her it was probably colic.
"When the lay person thinks about seizures,
they think about the whole body shaking," Groshong said in a
telephone interview. "It can be more subtle than that."
Groshong said Mikayla's seizures were unique
because she was alert when she was experiencing them.
But by the time Mikayla was about 20 months
old, her seizures appeared as bouts of laughter. Sarah recorded an
episode on video and took it to Groshong, who immediately referred
the patient to Roseburg neuroscience specialist Joel Daven.
"The videotape was dramatic I (the seizures)
were so frequent," Daven said.
Two weeks after doctors viewed the tape, in
June of 2000, an MRI revealed the tumor. The following morning the
family drove to Doernbecher, and Sarah thought an operation was
imminent.
"We had the whole weekend to ponder: Is she
going to die, is she going to have chemotherapy?" she said.
But brain surgery was not in the cards.
"They said they wouldn't operate on her
because it would do more harm than good," she said.
Innovative
Surgery
Mikayla's parents thought they had run out of options after
physicians at Doernbecher told them people with HH are expected to
live through the seizures. But then they discovered a Web-based
community of people who were either afflicted with the disorder or
were related to those who were.
A Web site, called HHUGS (Hypothalamic
Hamartoma, Uncontrollable Gelastic Epilepsy), was started in 1997
by Phoenix, Oregon, resident Carl Christy. It has 150 members from
all over the globe.
While Christy is not a doctor -- he works for
a home theater production company in Ashland -- Sarah said Christy
knows more about HH than many physicians in the state.
Christy said only one to two out of every
million children have the condition and that there have been only
200 to 300 diagnosed cases in the United States.
"It's a real tough thing to diagnose," he
said. "We know they are out there."
He said he knows of five children with HH in
Oregon, one of whom has already died.
"The support group is the best for all of
us," Trevor said.
In February 2002, the hearts of all of the
group's members were in Australia, where a 4-year-old Arizona boy
with HH was about to go through a landmark operation. His operation
was a success.
During a standard brain surgery the tumor
would be attacked through the bottom of the brain -- the incision
would be made in the nose or ears, which is the shortest route. It
has not been successful with HH, because the surgeon could not see
or remove the entire tumor.
Australian neurosurgeons Simon Harvey and
Jeffrey Rosenfeld developed a new technique, which involves going
in from the top of the head between the left and right halves of
the brain.
"Most neurosurgeons did not believe that that
could be done," said Phoenix, Arizona, neurosurgeon Harold Rekate,
in a telephone interview. Rekate will operate on Mikayla.
Harvey and Rosenfeld had by that point
completed about 30 such procedures, all with apparent success.
By the time of the Arizona boy's operation,
Harvey and Rosenfeld were looking to teach their method to American
surgeons. They found two willing participants at Barrow
Neurological Institute in Phoenix, Arizona. Dr. Rekate, the
chief of pediatric neuroscience at BNI, and Robert Spetzler, the
director of the institute -- who at first publicly said the
approach was unproven -- were now willing to try.
In February 2003, the Australian doctors came
to Phoenix and performed six surgeries, all of them successful.
"[Rosenfeld] showed me that it could be done
with safety," Rekate said.
To date, 54 new-approach surgeries have been
performed: 40 by the Australian doctors and 14 by Rekate and
Spetzler.
Out of Rekate's 14 patients, 11 are
seizure-free and only one has not seen dramatic improvements.
Not all physicians support this still-new
procedure.
Daven, the Roseburg neurosurgeon, believes it
is too risky.
"It's still experimental," he said. "The
parents of the patients need to know this. We don't know enough
about the long-term outcome of the patient."
There have been some complications. Rekate
said patients stand to lose their short-term memory, lose the
ability to control hunger and become obese. These functions are
controlled by the hypothalamus. A hormone that controls urine
production can also be damaged, making the patient thirsty all of
the time and urinate constantly. This can be easily controlled by
hormone supplements, but the other two side effects are not.
Critical
Choices
Sarah and Trevor, both 25, had an important decision to make.
Mikayla has from three to 15 seizures every
day -- 95 percent of the time she has one within minutes of falling
asleep. While they do not always cause pain, they leave her
physically spent, and there is the damage they can do in the
future.
Mikayla has tried three different
medications, one of which she had to take five times every day.
"They do damage too," Trevor said. "You use
medications to stop the seizures I but she becomes a
vegetable."
This for a child who her mother describes as
outgoing and who "never wants to miss out on a thing."
Neither of her parents have gotten much rest
for the past four years.
"If the kid's not sleeping right, you're not
sleeping right," said Trevor , a hazardous materials
specialist.
And through it, they have tried to keep up
hope.
"I feel lucky it's not cancer and she doesn't
have to go through radiation," Sarah said, adding she is looking
forward to the time when she can enroll Mikayla in dance and
gymnastics classes like any other child.
Mikayla will turn 5 years old on July 28 --
the day before her surgery. Pending a successful surgery, she will
enter kindergarten next year.
"I want to play with my toys every day (after
getting out of the hospital)," she said.
Her family held a party for her at her
grandmother's house last weekend and are leaving for Phoenix
Thursday. The surgery is expected to cost $150,000, and the
family's insurance company is covering 80 percent of it.
Sarah and Trevor have not told Mikayla any of
the specifics of the operation, but she knows that her head will be
cut open and is nervous.
"She asked me if they were going to cut (the
tumor) out with a knife," Sarah said.
But Mikayla also knows the benefits.
"I won't feel a thing," she said. "No more
seizures."
