RALEIGH, N.C. – Shane Benoit, a carpenter by trade, has no medical training. Yet, standing over his 7-month-old son Ethan’s bed at the University of North Carolina Children’s Hospital, Benoit chatters away as he suctions the tracheotomy tube in the boy’s neck or adjusts the rolls of gauze that hold open Ethan’s stiff, balled hands.

When a doctor stops in, Benoit uses the moment to show how Ethan’s feeding tube, which dumps directly into his intestines, is positioned incorrectly and is causing gas to build up in the boy’s abdomen. The doctor nods and promises to discuss it with the nursing staff.

Since Ethan’s birth, Benoit has become a reluctant expert in a disorder most doctors in the United States have never seen.

Metatropic dysplasia, a severe deformity of the spine, arms and legs, has compromised all of little Ethan’s internal organs, including his brain. The disease is so rare it has thrust the Benoit family into a medical no man’s land. As of 2004, there were 80 cases documented worldwide, 25 in the United States.

There is no protocol for treatment. There are no local support groups for the families, no Web sites to consult.

Shane and Sherree Benoit ask the doctors the myriad questions one might expect. The answers are few.

“There are no yeses, there are no noes,” said Shane Benoit. “There are only “I don’t knows.”‘

Born at Rex Hospital in Raleigh, N.C., Ethan was diagnosed at Duke University with the help of a visiting geneticist. Of the three forms of the disorder, Ethan has the rarest and the most severe. It will almost certainly be fatal.

“They told us to live every day as his last and take lots of pictures,” said Sherree Benoit, 31.

The doctors at UNC Hospitals, where Ethan has been since late May, told the couple they have never had a case of metatropic dysplasia at the research hospital before. Ethan’s team includes a neurologist, orthopedic surgeons, pediatric surgeons, nurses, cardiologists, physical therapists and others.

Dr. Terry Noah, a lung specialist working with Ethan, said the disorder is so unusual that the medical team attending Ethan is charting a new course.

“With rare disorders like this, there is not a lot of experience to go by,” Noah said. “The care is very individualized. It is supportive care. There is no cure.”

The baby with a cherubic face has spent time at every major hospital in the area, his parents educating emergency room doctors, nurses and emergency medical technicians along the way.

Ethan’s rib cage is extremely narrow before curving out like a bell, making it difficult for him to breathe and putting pressure on his internal organs. His spine is malformed. His arms are locked in a curved position, as are his tiny fingers.

His one knee is the size of a baseball. His legs, one long and one short, twist at odd angles and cannot be straightened.

Until May, he seemed at least to know his family members – to respond to the voices of his mother and father.

“His eyes followed us like hawks,” said Shane Benoit, 36. “He giggled. He had million-dollar smiles.”

Then, on the night of Memorial Day, Ethan apparently suffered a stroke in his sleep. The next day, he had three more strokes. The doctors placed a shunt in his brain. But the damage had been done.

His eyes no longer track, though they do respond somewhat to shadow and light. He reacts to some sharp sounds, though he no longer responds to the lilt of his mother’s coos.

The million-dollar smiles have faded.

Even his cries have been silenced – by the tracheotomy tube that allows him to breathe.

The Benoits moved to North Carolina from in August. Shane Benoit was looking for more consistent year-round work in construction. Sherree Benoit wanted to be closer to her mother and siblings in Wake Forest. The couple wanted their children, Tia, 12; Taylor, 9; and Saige, 3, to get to know family. They were expecting Ethan when they moved.

They had no idea Ethan had any problems until they reached the delivery room.

“At first I didn’t grasp how badly all of this was going to affect us,” Shane Benoit said.

But reality quickly sank in. Self-employed, Benoit realized he didn’t have the time or energy to build a new business on his own.

He tried taking jobs with other firms. But he missed too many days for doctor’s appointments and medical emergencies. Medicaid pays for Ethan’s care, but this spring Benoit sold his tools to pay other bills. He and his wife pawned their wedding rings.

The landlord on their mobile home has been understanding. But they’ve missed payments on their pickup truck, as well as the insurance on it. The registration has expired, but Shane continues to drive.

At night, he pulls the pickup around behind the trailer, chains it to a pole and takes the license tag inside, so it isn’t repossessed.

“What else can I do?” he said. “I’ve got to get groceries. I’ve got to get over to Chapel Hill to see my baby boy.”

When Ethan comes home from the hospital, the Benoits’ lives will become even more complicated. Their two-bedroom trailer will have to be reordered and outfitted for round-the-clock nursing care.

The Benoits are looking for good homes for their three pets, a hound dog, a black-and-white cat and a ferret.

The older two children won’t be able to return to school in the fall, because the family cannot afford to risk the germs that might come home.

So, whenever Ethan comes home, Sherree Benoit will be home-schooling two children, taking care of a preschooler and tending a critically ill baby.

She acknowledges how hard this will be.

The Benoits know that many parents, in their shoes, might put Ethan in a nursing facility – or perhaps send him to hospice for end-of-life care. But no one can really say how to face the situation, what should be done, and what should be expected.

All they know is that Ethan has beaten all the odds to survive this long, and they’re guided by the only tool they’ve got in a journey that has no road maps – the parental instinct to nurture.

“There is the slightest chance that his brain could work around all this,” Shane Benoit said, “that he could interact with us again. Is it likely? No.”

“But,” Sherree Benoit said, “we cannot give up hope.”

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