OAKLAND — Five-year-old Mikey Markwardt attracts more than a few glances as he maneuvers with his walker through a waiting room at Children's Hospital Oakland.
Both of his hips are dislocated. One leg is shorter than the other. He wears a brace after undergoing surgery for a severe spinal deformity. Yet none of this slows him down.
Sporting a new mohawk haircut, he moves speedily and chatters constantly.
"Can I go play over there?" he asks.
"As long as you get suctioned first," says his stepfather, Richard Gould, who uses a machine to clear Mikey's tracheotomy. Mikey heads off to play, pretending he is a server at a fast-food restaurant.
Five to 10 years ago, medical experts would havehad little to offer Mikey, said Dr. James Policy, a pediatric orthopedic surgeon.
But today, new devices and techniques make it possible for surgeons to do spine-straightening operations on small children. As a result, more help is available for those with scoliosis and other spinal deformities.
"It's great that Mikey and other kids like him now do have some treatment options that were not available until fairly recently," Policy said.
Mikey has a rare genetic disorder known as Larsen syndrome, which occurs in about one in 100,000 people.
First described in 1950 by orthopedic surgeon Joseph Larsen, the condition can lead to dislocations of major joints, foot deformities, abnormal curvature of the spine and other
It also can soften cartilage in the windpipe, making it difficult to swallow and breathe. That explains Mikey's tracheotomy, an opening cut in the front of his neck.
Policy has seen two other children with Larsen syndrome during his career. Neither had as severe a spinal deformity as Mikey.
When Mikey was a baby, medical experts at a hospital near the family's Santa Rosa home told them he would never walk.
"We proved them wrong," said his mother, Rachel Markwardt.
After Mikey turned 2, the family brought him to Children's Hospital Oakland for care because doctors there provided more hope.
Even before his November surgery, Mikey used a walker to get around, but his worsening spinal problems threatened to jeopardize that ability and he often complained about back pain.
"It is pretty impressive that he's even able to walk at all," Policy said.
In addition to other medical problems, Mikey has kyphoscoliosis, a combination of kyphosis, which causes his vertebrae to curve forward and form a hump in his back, and the more common scoliosis, which causes his spine to bulge to the side.
"As the deformity was getting worse and worse, it was pinching off his spinal cord," Policy said. "So if it had been allowed to continue, he most likely would have ended up being paralyzed. The second problem is that it was taking away the space in his chest cavity for his heart and lungs to function. That can have some pretty serious long-term consequences as well."
Policy decided to operate. The goal: attach metal rods to Mikey's spine to keep it straighter and give his heart and lungs a chance to mature. The rods contain lengthening devices. About every six months, Mikey will return for surgery to extend the rods to keep pace with his growth.
This procedure is common for people with spinal deformities, but only in recent years have physicians been able to use it on smaller bodies. Mikey stands 36 inches tall and weighs 32 pounds.
"There just weren't implants or devices that were small enough to use in younger children, and the techniques hadn't really been developed," Policy said.
Before installing the rods, doctors put Mikey in traction for several weeks to straighten his spine as much as possible. Pins were inserted into Mikey's skull and connected to what looked like a halo — a metal ring around his head. Sixteen pounds of weights could be attached to the halo to force Mikey into a straighter stance.
The hospital uses the traction technique on three or four children a year.
"It just provides a constant stretch on the spine to help straighten it out gradually and safely over time so that we don't have to do an acute correction during the surgery," Policy said. "That's easier on the spinal cord."
Mikey had traction devices on his wheelchair, walker and bed. This enabled him to move around the hospital and play video games with other children or kick a ball in the hallway with his mother.
On Oct. 30, Mikey had been in the hospital 44 days. He planned to dress up as a pirate the next day for Halloween and had tied a pirate flag to his walker.
"Aye, aye, captain," he said in his best pirate's voice. "Aaarrrrh."
"He's got the whole hospital wrapped around his finger," his mother said.
Mikey's classmates from the Santa Rosa Charter School for the Arts made a book with drawings for him titled "What I like about Mikey."
"I like your courage," his teacher wrote. "I'm sending you rainbows and happy thoughts."
His mother spent most nights at the hospital, sleeping in a bed in his room.
"I feel bad leaving him. I tell all my friends that I left my heart in Oakland and that he's hanging in there," she said, glancing at his wire-and-rope traction device. "That's my joke."
On Nov. 4, Policy inserted the metal rods and fused vertebrae at the top and bottom of his spine to anchor them.
Mikey has since returned home and recently came back to the hospital for a checkup. "This X-ray looks good," Policy told him.
The hope is that Mikey's body will tolerate the rods and extensions until he is between 12 and 14, Policy said. When the rods are removed, surgeons will probably fuse the spine so that it becomes one solid bone to prevent further deformity.
"If we could get Mikey to 10 years old before we had to do a more definitive surgery, I would be very, very happy," Policy said.
Mikey's condition is so unusual that his family has known only one other child with Larsen syndrome. They met the girl once, then the family moved away. There have been no others.
Despite his tremendous challenges, Mikey seems to have endless energy and an enthusiastic, spirited personality. "Feisty" is how many describe him.
He will return to school soon. His mother is pleased that, since the surgery, he can stand straighter and has not complained about pain. Other surgeries may be on the horizon, including one to lengthen his leg, but for now, the family is glad to be home and watching his progress.
"I just want him to be able to do anything he wants to do," his mother said. "That's why we fight so hard."
Reach Sandy Kleffman at 925-943-8249 or firstname.lastname@example.org.
-- Rachel Markwardt,
whose son, Mikey, has
a severe spinal deformity known as Larsen syndrome