Young Fighter : Girl Has Braved Deafness, a Heart Defect and Brain Surgery, and She Battles On

From the beginning, she has beaten the odds.

Born deaf, Jennifer Cottini learned to speak and to read lips.

Born with a defective heart valve, she underwent several operations to enlarge it so enough blood could pass through her heart.

All of this before the age of 9.

It seemed she was finally on her way to a normal childhood--until two months ago when she suddenly and inexplicably had a massive stroke.

“We were in shock. My whole world just crumbled,” said Jennifer’s mother, Deby Jowett. “As a parent, you are just helpless. There is nothing you can do.”

Confounded by a child having a stroke, doctors at Children’s Hospital at Mission in Mission Viejo searched for a reason.

They found it in a rare disease called Moyamoya, which affects blood vessels in the brain and prevents the organ from receiving sufficient blood.

Last month, Jennifer was the first Orange County youngster to undergo a risky operation that transferred an outer neck vessel into her brain to increase the blood flow and prevent another stroke.

Luckily for Jennifer, she was in the hands of Dr. Michael Muhonen, the director of pediatric neurological surgery at Children’s Hospital of Orange County, who had performed the operation nearly a dozen times in other states.

In October, she is scheduled for another operation, this time to the right side of her brain, to further increase the amount of blood flow, and, doctors hope, to ensure that another stroke does not happen.

Though she is slowly recuperating from the stroke, Jennifer has a long way to go and the Mission Viejo family is suffering the hardship of trying to regain a normal life.

She is learning to walk and speak all over again. Her right side is still paralyzed. She was planning to begin fourth grade at Kaiser Elementary School, where she was to begin mainstream classes rather than special education.

Every day, five hours a day, she and her mother are in the Children’s Hospital rehabilitation ward in Orange, exercising the brain and muscles devastated by the stroke.

It is a hard battle and one that demands endless faith and strength.

“You get up in the morning and you put on your armor,” Jowett said. “We don’t take things week by week or day by day. We take them hour by hour.”

June 30 began like any other Monday morning in the family’s home. But by 11 a.m., Jennifer started having seizures and lost consciousness. Doctors worked quickly to stabilize the youngster. For two weeks, Jennifer Cottini lay in a coma on life support.

“We had some very difficult times asking ‘Why her?’ ” said Jowett, a woman with soft, large blue eyes and a quiet voice. “You find yourself almost getting upset with God. She had worked so hard to get to where she was and the stroke took everything away.”

Miraculously, Jennifer came out of the coma.

But John Jowett--the girl’s stepfather--and Deby Jowett had to make another difficult decision. Should the doctors attempt the high-risk operation, where even a small drop in blood pressure could cause another stroke?

They decided they had to press ahead.

“We knew the chances for survival were not good,” Deby Jowett said. “We knew she was in the hands of God.”

Muhonen and his team prepared for the operation.

Not much is known about Moyamoya, which means “something hazy like a puff of cigarette smoke drifting in the air” in Japanese. The word is meant to describe the chaotic look of vessels in the brain of an afflicted person. Normally, the vessels look like tree branches, streamlined to supply plenty of blood.

Muhonen said perhaps Jennifer inherited the disease from her father, Joe Cottini, who died of a stroke at 39, but the origins of the disease are not known. Though Muhonen said it is likely that the stroke has left Jennifer with some permanent damage, he is hopeful that her young brain will be resilient enough to recuperate.

“We didn’t have many options,” Muhonen said. “There are major risks with any brain operation. I knew I could induce a stroke if I damaged any minor vessel.”

Muhonen and anesthesiologist Dr. Eric Walbergh had to make sure Jennifer’s blood pressure was high--rather than the usual low--during the operation. So, they used a new device for the first time. It is attached to the jugular vein and gauges the amount of oxygen being supplied to the brain during surgery.

After four hours on the operating table, the doctors had successfully taken a main vessel supplying blood to the face and neck and transplanted it inside Jennifer’s skull to begin providing the brain with an ample flow of blood.

Within a few weeks, Jennifer began to speak.

She was determined to begin walking again, determined to ride her bicycle, swim and play baseball.

Her strength abounded on a recent weekday when she announced to her physical therapist that she would walk. With a little help, hanging on to a railing, she began to move her legs, pushing her paralyzed right foot forward with her left foot.

She is swimming again, in a small rehabilitation tank, and has informed her mother that she is eager take a trip to Disneyland soon.

Though she is rail thin, her deep blue eyes still shine, winking slyly at strangers as she greets them. She is coquettish, twirling her long, brownish-blond pigtails when practicing her speech therapy. She is once again a semblance of the little girl who once kicked an older, bigger school bully in the shins for picking on a little boy waiting in the lunch line.

“Jennifer has always been strong-willed,” Jowett said. “She has overcome everything.”

Though she can only muster out one syllable words, slowly she is now expressing slang like “Oh man!”

Of course, she has her days. Those days when a deep depression overcomes her and she cannot eat and she cannot look up, only hangs her head silently. Those are the days she sees other children riding their bicycles and tearing it up on the baseball fields. Those are the days her mother wishes God would give them a break and let her daughter be free to run around like most 9-year-olds.

Still, they can only stay on track, keep positive and hope.

“You never think such a severe illness could happen to your child,” Jowett said. “But you find yourself with tunnel vision. [Helping her] is a tireless task. Still, I think things happen for a reason and this has been a learning experience for us to appreciate life.”