Ailing boy defies the odds, makes medical history

December 05, 1993|By Karin Remesch | Karin Remesch,Contributing Writer

The frail boy with big hazel eyes sits in a wheelchair tethered by tubes that feed him and pump oxygen into his lungs. He gasps for air after every word.

A rare disease has hardened the lungs and ravaged the body of 7-year-old Nicholas Eagle, who loved the outdoors and swimming, bike riding and playing baseball just five months ago.

His legs -- thin and sapped of strength -- no longer can manage simple steps. His face is puffy and pale, the result of heavy doses of steroids.

But on a crisp and sunny late fall day in the family room of his two-story Colonial house in Bel Air, Nicholas smiles and says he's glad to be back home after spending more than four months in bed at the Johns Hopkins Children's Center.

He pushes the controls of a Genesis video game, skillfully maneuvering a rabbit through a maze of obstacles.

"I love playing Genesis," he says while brushing thick brown curls from his forehead. "It's good to be home."

Nicholas' doctors and his family feared he would never leave the hospital. The odds were overwhelmingly against his ever being released -- or even surviving the first night at the hospital in mid-July.

Nicholas has interstitial fibrosis, a rare lung disease. Hopkins experts say there are only a few cases worldwide.

The progressive disease turns healthy lungs into scar tissue. As the scar tissue spreads, the lungs stiffen, making breathing more difficult and usually resulting in death.

But Nicholas is defying the odds. By living, he is contributing to medical history.

"Most kids in his condition would have died," says Dr. Peter Rowe, director of the diagnostic referral clinic at Hopkins' department of pediatrics and one of Nicholas' physicians.

"He might be the first child surviving this form of lung disease," Dr. Rowe says.

In fact, his condition stabilized enough that doctors decided last week to send Nicholas home, along with his hospital bed, monitors, oxygen tanks and around-the-clock nursing care.

When Nicholas arrived at Hopkins in July, the prognosis was grim. Doctors were almost certain he would die.

They had even asked his parents for permission to perform an autopsy in an effort to determine what caused the rare lung disease.

'Am I going to die?'

His mother, Anna Eagle, had to deal with her son's nightmarish question: "Mommy, am I going to die?"

"What a difficult question to ask a parent," Mrs. Eagle says. "All I could tell him [was] that he was a special child in God's eyes and that God had chosen him because he was strong and a fighter."

But Nicholas didn't want to be a special child, Mrs. Eagle recalls.

"He asked angrily, 'Why did He pick me?' " she says.

That was almost five months ago. Since then, Nicholas has survived six weeks on a ventilator, up to six tubes protruding from his chest to keep his lungs from collapsing, biopsies and the removal of 35 percent of his right lung.

Mrs. Eagle calls her son her miracle child. Doctors agree that a miracle and technology at Hopkins have kept Nicholas alive.

Though the damage to Nicholas' lungs has been diagnosed as interstitial fibrosis, how he contracted the disease remains a mystery.

The rare fibrosis usually is caused by a hypersensitive reaction to inhaled particles, such as molds and chemicals, Dr. Rowe says.

"With Nicholas, we just don't know where it came from, and we don't really know how much better he will get," says Dr. Mark A. Helfaer, associate professor and pediatric anesthesiologist at the children's center.

Dr. Helfaer believes that the use of technology generally reserved for the newborn intensive care unit saved Nicholas' life.

Nicholas was transferred to Hopkins after his condition worsened while he was being treated for acute pneumonia at Franklin Square Hospital.

Unable to breathe on his own, Nicholas was switched from a powerful conventional ventilator, which forced air into his lungs, to an oscillator, which gave him smaller amounts of air more frequently.

The oscillator prevents the forceful opening -- and eventual damage -- of the lungs that occurs with conventional ventilation, Dr. Rowe explains.

"We had nothing else to offer him. The oscillator gave him a chance to live," he says.

Though frequently used in the newborn intensive care unit, oscillators have not been approved by the Federal Drug Administration for use on children of Nicholas' age, says Dr. Helfaer, who recommended that Nicholas be hooked up to the device.

Dr. Helfaer says the device has been used fewer than 10 times on older children.

"The oscillator got [Nicholas] over the hump" by supplying more gently than the ventilator could the air necessary to keep him alive, Dr. Helfaer says.

Watching their son being connected to a mechanical breathing device was devastating for Mrs. Eagle and her husband, Chris, a senior field service engineer for Columbia-based Beckman Instruments.

"Nicholas was so heavily sedated . . . he was paralyzed," Mrs. Eagle says. "He was very swollen from receiving so much oxygen."

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