Sunday, November 09, 2003
Copyright © Las Vegas Review-Journal

CARING FOR MACKENZIE

Family struggles as baby fights disease attacking her brain

Bryan Hymas comforts his daughter Mackenzie last week. The 23-month-old suffers from opsoclonus-myoclonus syndrome, which causes the body's immune system to attack the brain. Photo by John Gurzinski. Bryan and Shannon Hymas play with Mackenzie, who is being treated for a rare brain disease at Sunrise Children's Hospital. Photo by John Gurzinski.

There used to be times Mackenzie Hymas would laugh so hard playing peek-a-boo it seemed she could turn blue.

It might be on a Saturday morning, when her two big sisters were watching cartoons. Mackenzie, all of 16 or 17 months old, would toddle around her family's living room. Her mom, Shannon, might walk up and tickle her, and Mackenzie would just laugh and laugh.

"Peek-a-boo!" her mom would say, ducking behind the furniture.

That's how it used to be.

Now, as Mackenzie approaches her second birthday, she's stuck in a hospital bed in the pediatric intensive care unit at Sunrise Children's Hospital.

Doctors have fastened tubes to her face, her arms and her leg. The tubes feed her and pump a host of powerful drugs into her bloodstream to help her in a fight against her own body.

Electrodes taped to her chest keep track of how fast her heart beats and her breathing rate. A machine makes sure the nurses are alerted if her blood pressure gets too high or if there's something wrong with the oxygen levels in her blood.

Mackenzie had been potty training before this happened. She was still learning to talk and was figuring out how to dress herself.

Now, because of a mysterious disease that showed up without warning and started attacking her brain, she cannot walk or crawl or talk or sit up or even give out one of those hugs her mom said used to be "the best baby hugs on the planet."

"It's like, mentally she's there," said Mackenzie's dad, Bryan. "She just doesn't have the physical control to do anything."

Bryan and Shannon married five years ago after an on-and-off courtship that had been going on since high school.

Shannon, 28, and Bryan, 29, had two girls, Annalisa, now 3 1/2, and Mackenzie, who will be 2 next month. Shannon already had a daughter, Francesca, now 7, when she and Bryan married.

They bought a house earlier this year near Windmill Parkway and Interstate 215.

In late June, as Shannon was getting the girls ready for school one morning, Mackenzie tried to stand up, and she bumped into the wall.

It actually looked kind of funny.

"What's wrong with you? Are you drunk? Did you get into your daddy's beer," Shannon remembered saying. It reminded her of when kids spin themselves in circles until they're so dizzy they can't stand up right.

But clearly, there was something wrong with Mackenzie, who is often called Boo because she looks just like the little girl with the same name in the Disney movie "Monsters, Inc."

Thinking Mackenzie had an ear infection, Shannon took her to the family doctor. The doctor agreed, Shannon said, and prescribed amoxicillin.

Days later, Mackenzie was worse. She still couldn't walk and was shaking uncontrollably.

"It's like her whole body had the hiccups or something," her mom said.

And there was something wrong with her eyes. They kept darting back and forth, like Mackenzie wasn't in control anymore.

A pediatrician sent her to the emergency room at Sunrise, and that's when the long process of figuring out what was wrong really began.

Doctors ran all kinds of tests on her urine and blood. They ran a cat scan, an MRI and an EEG. They found nothing wrong, Shannon said.

"Everything is completely normal, but she obviously is not," she said.

Mackenzie was helicoptered to UCLA Medical Center in August, where more tests finally led to a diagnosis. Mackenzie had a disease called opsoclonus-myoclonus syndrome, OMS for short.

"Unfortunately, it is a very difficult disease to diagnose," said Mackenzie's Las Vegas neurologist, Dr. Sri Halthore, who has seen only a handful of such cases in nine years of local practice.

For whatever reason, the disease commands antibodies to attack the brain. In effect, the thing that is supposed to protect the person from disease is trying to kill them.

"It's a case of friendly fire of the immune system on the brain," said Dr. Michael Pranzatelli with the National Pediatric Myoclonus Center at the Southern Illinois School of Medicine.

OMS is rarely fatal in children, and sometimes, in mild cases, some seem to recover. They never really do, though. Pranzatelli, a nationally known expert on OMS, said children often develop behavioral disorders such as attention deficit hyperactivity disorder, or ADHD, and can be prone to physically attacking family members.

It also often takes a tremendous toll on families, where financial and other pressures often lead to divorce, Pranzatelli said. Other children in the family can feel neglected, and sometimes lash out, he added.

Its cause is still unknown. About half of those who are afflicted also have a neuroblastoma: a rare, cancerous tumor. It is thought that the antibodies designed to fight off the tumor somehow also end up attacking the brain.

Often, if the disease is caught soon enough, the tumor is removed and the disease goes into remission.

The other half of OMS patients apparently have no neuroblastoma. In those cases, doctors think the disease might be caused by a virus, a known cause of other brain abnormalities.

That may be what happened in Mackenzie's case. Doctors have found no sign of a tumor.

Whatever the cause, the disease is already taking a heavy toll on Mackenzie and her family. Since that day in June, both of her parents have lost their jobs because they had to spend so much time with her. Bryan is still looking for work.

They have lost their home, too, and had to move in with Bryan's parents.

Shannon estimated that medical bills are nearing $2 million. The family is uninsured but is hoping Medicaid or Social Security will help out, she said. They're also applying for emergency welfare, something that Shannon said they never thought they'd have to do.

"It's really hard to ask for help," she said.

She said she has been staying with Mackenzie in the hospital almost constantly as she tries to find her way through the maze of government paperwork she needs to complete so the doctors and the hospital can be paid.

Mackenzie has undergone a flurry of expensive medical treatments recently. They include the injection of powerful drugs to treat the OMS and the side effects from other drugs, a surgery to remove an adrenal gland, and placing her on a ventilator because her brain wasn't telling her body to breathe. That is not a normal condition of OMS, and Shannon said it is unclear what's causing it.

Mackenzie's future also remains unclear.

Pranzatelli said she may one day have to undergo chemotherapy, a treatment directed to certain parts of her immune system to get it to stop attacking her brain.

He said brain damage is a common outcome in OMS patients, even in those who respond favorably to treatment and, in very rare cases, later show no signs of the disease.

In the long term, OMS can lead to serious behavioral problems and a dramatic loss in IQ points.

"Worst case scenario is the IQ drops to between 40 and 80," the doctor said. "These are previously normal children. It's very scary."