'You wish it was you: 2-year-old battles serious brain condition

Posted: Sunday, July 19, 2009

When Marcia Jacobs' 2-year-old daughter Emily began having bouts of intermittent, uncontrollable vomiting in the middle of the night six months ago, she couldn't help but think of her other child, Anjuli, who died from brain stem cancer eight years ago at the age of 4.

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Emily Jacobs giggles after peeking over the fence to see the Kenai River from Soldotna Creek Park, one of her favorite places, during a visit on Friday. The 2-year-old is fighting a condition called chiari malformation of the brain.

Like her sister, Anjuli, too, experienced late-night vomiting, which turned out to be a symptom of increased intracranial pressure, meaning the pressure inside a person's head is increasing to dangerous levels.

In Anjuli's case, the pressure was caused by a tumor. For Emily, it's not cancer, rather her cerebellum is sliding through the hole in the base of her skull that attaches to her spinal column, which is causing the pressure.

Called chiari malformation of the brain, Emily's cerebellum is descending into her spinal column.

Before being diagnosed more than a month ago, Emily made several trips to the hospital, however, doctors still couldn't explain what was causing the pressure inside her head.

In late May, Marcia and her daughter flew to Children's Hospital in Seattle for an evaluation.

At first, Emily was sent to a gastroenterologist, but to no avail. All of the gastro tests came back negative.

Emily needed a brain MRI.

Knowing the potential discovery the test could yield, Marcia said she did everything she could to avoid the test.

"I just had a bad feeling," she said.

Emily's doctors told Marcia the test would most likely rule out the brain as the cause, but after the test took twice as long to complete than it was supposed to, she knew something was wrong.

Marcia was taken into a "special" room, just like she was to hear Anjuli's diagnosis.

"The first thing he said is, 'It's not a tumor," Marcia said.

But the gastroenterologist said they did find something and that Marcia and Emily would be staying in Seattle for some time.

Emily's case was then transferred to the neurological team, headed by the same surgeon who operated on her older sister.

"Just when I see him, I start to shake all over," Marcia said.

Marcia and Emily stayed in Seattle for three weeks while several tests were conducted.

Chiari malformation of the brain affects each person differently. Some patients don't even know they have the disorder, while others, like Emily, are in a much more critical situation.

"Her condition is quite serious," Marcia said.

Emily's brain is putting pressure on her brain stem, which restricts the flow of cerebral fluid in her brain. Currently, Emily has no flow to the back of her brain.

Emily's case is considered life threatening.

Because she has no cerebral fluid to the rear of her brain, it doesn't take much for the pressure to increase, Marcia said. This can cause vomiting, seizures, abnormal eye movement and in extreme cases, death.

Pressure on the brain stem also causes swallowing problems, which in turn leads to eating issues. Marcia said there are only about 16 foods Emily can eat, making it difficult to keep her weight up and include all the necessary nutrients in her diet.

Because the cerebellum, which is being squished, controls balance, Emily also suffers from balance issues.

"She has problems with balance," Marcia said. "She's constantly falling."

After being diagnosed, doctors told Marcia Emily's only option was surgery.

Over and over again, Marcia was told, "She needs surgery. It's not optional. She has to have it."

Again, Anjuli came to mind for Marcia.

"Been there, done that," she thought. "I don't want to go through it again."

Normally the type of brain surgery Emily needs is dangerous, but it's especially dicey for someone so young.

"The surgery is very, very, very risky. The younger a person is, the more risky it is," Marcia said.

The surgery is most dangerous to children under the age of 3.

Because of this, Emily has since been placed on "watchful waiting" until she reaches the age of 3 and surgery becomes a safer option.

There has been some talk of moving Emily's surgery up to December, though, Marcia doesn't have all of the details at this point.

"The surgery is a big deal," she said. "There's a lot of risks."

Surgery could leave Emily paralyzed or worse.

On the other side, should everything go 100 percent as planned, Emily might not need another surgery her entire life.

"I'm clinging to that," Marcia said.

However, it's unlikely, especially with childhood symptomatic chiari patients, as many need multiple surgeries throughout their entire lives, a fact Marcia is well aware of.

When it is time for her surgery, Emily will be in the hospital for a week and in Seattle for one month.

Her convalescent period is three months.

Surgeons will remove the back part of Emily's skull, without replacing it, giving her brain room to expand, instead of sliding down into her spinal column.

Emily also has a hole in the thoracic region of her spine, near her ribs.

The hole, which is a common development in chiari patients, should heal on its own after surgery.

"That hole is leaking cerebral spinal fluid into her spinal column," Marcia said. Over a long period of time, this can be harmful to the spine.

Emily is no stranger to the hospital, as she's been having medical issues since birth.

"She's sick more often than not most of the time," Marcia said.

For her upcoming surgery, though, no one is certain what the results will be.

"There's no way to know how Emily is going to do and what's going to happen," Marcia said.

But she isn't letting the future ruin the present for her and her daughter.

"We're making the most of it," Marcia said about returning home to the Kenai Peninsula. "I'm trying to make good, happy memories for her."

Since their return, Emily has been doing well, Marcia said.

"She has been having some very good weeks since then. She's sleeping better than she ever has. She has just been doing really a whole lot better."

Though Marcia is enjoying the present with her daughter, the fear of the future isn't too far from her thoughts.

"I have no idea when it's going to end. What's the next day going to bring? What's the next night going to bring? When it's your child, you wish it was you."

Marcia said now she's just focusing on day-to-day activities, building good memories for the rough road ahead, and catching up on her sleep.

"The trip to Seattle was exhausting. You just don't sleep very much when you're in that kind of a situation," she said.

Despite all the trials she's been through with her two children, Marcia said there's no greater pleasure than being a mother.

"My daughters are my greatest joy. If she can get through it, I can get through anything," she said.

"I'm just thinking she's going to do fine until December."

Having two daughters both with life threatening brain stem related medical issues, it's hard for Marcia not to think of what the odds are of this happening to the same mother.

"There's this sickening feeling that lightning has struck twice for me."

However, that side is kept hidden from Emily.

"I do not allow myself to fall apart in front of her," Marcia said. "She needs a strong mommy. I am going to give her that."

Without any family living in the state, Marcia has had enormous support from her friends to help get her through this difficult time.

"My friends are just being wonderful as they always are," she said. "You can't get through stuff like that without your friends.

"If there was an Olympics for friendship, my friends would take all the golds."

A non-profit account has been set up at Wells Fargo Bank under Marcia's name to a assist her with Emily's medical bills. The account number is 6044680434. Donations are tax deductible.

Mike Nesper can be reached at mike.nesper@peninsulaclarion.com.

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