August 6, 2000 Raleigh News and Observer Sunday Paper – Front Page Headline
Orthopedic odyssey ends in hope
By SARAH AVERY, Staff Writer
APEX, NC—From the moment he was born, Jesse Rause came fully equipped to make his way in the world.
A strapping 6-pound, 11-ounce baby, Jesse arrived that Saturday morning at Rex Hospital, the third of three boys born to Tom and Dawn Rause, and everything was perfect. Dawn cuddled her newborn, Tom cooed and nurses hovered.
But later, while logging Jesse’s length, nurses discovered that his left leg measured 2 inches shorter than his right. Mom, Dad, everyone had missed it in those first elated moments because Jesse, like any baby, scrunched his knees up to his chest. All anyone noticed were the 10 little toes on his two little feet.
The leg-length discrepancy, the Rauses came to learn, is the product of a rare congenital condition called proximal femoral focal deficiency. No one knows how or why PFFD occurs. All the Rauses knew, and all that anyone could tell them, was that Jesse’s legs would only grow more uneven, possibly by as many as 12 inches.
When the Rauses asked how doctors planned to help Jesse, the response horrified them: Cut off Jesse’s left foot and have him fit for a prosthetic leg.
“How could we do that?” Dawn asks, incredulous. So the Rauses began a medical odyssey that would, eventually, lead them to the forefront of orthopedic technology, to a solution that is considered, in such a case as Jesse’s, radical, difficult and almost miraculous. “We felt this pressure,” Tom says. “We had a window of one year that the doctors were telling us was the best time to perform the amputation—do it while he is young and can learn to walk on a prosthesis. We were running against the clock.” And against hope.
The Rauses could only cling to an ever-fraying rope of faith—in God, in medical technology—that somehow Jesse’s leg could be saved. As Jesse’s parents, they were compelled to pursue every lead. Every option. They knew that Jesse, after all, had everything else he needed to win his way in this world.
Dawn Rause has a wholesomely pretty face that has worn the stress of four children so well that, should she attend her 20-year high school reunion this year in Kent, Ohio, she’d be a shoo-in for the title of Least Changed. Tom, an engineering manager for a network services company, is the optimist of the two. A calming influence. The two met in the Triangle after Dawn had moved to North Carolina to escape Midwestern winters. She was working as a temporary secretary, and he was her boss. They married in 1988. Their first son, Taylor, was born three years later; Shawn came in 1993. The two wanted a big family, but after Shawn was born, Dawn suffered some miscarriages. Finally, she became pregnant with Jesse, and the couple’s dream was back on track. After Jesse’s birth, Dawn grew depressed and guilt-ridden.
“I went over and over the pregnancy thinking certainly I had done something to cause this.”
Two days after Jesse was born, the Rauses took him to a local pediatric orthopedist—a bone doctor for babies—where they hoped to learn more about Jesse’s condition.
There, they were told that PFFD is, basically, a fluke. Studies indicate it occurs in one out of 50,000 births. Doctors don’t know what causes it, although injury during pregnancy, bacterial toxins, viral infection, chemicals and hormones have been suggested as culprits. Forty years ago, thalidomide, prescribed to prevent miscarriages, caused an outbreak of PFFD and other limb anomalies in babies, but it has long been banned. Dawn had no exposure to the drug.
As for Jesse’s leg, the doctor told them, the projected 12-inch disparity went beyond standard procedure for limb lengthening. Orthopedic surgeons are loath to put children through bone stretching ordeals if the limb discrepancy is more than 20 percent. In those cases, amputation is usually the answer.
“Amputation is final,” Tom says. “The way technology changes, and medicine changes, they can do new things every year. We weren’t going to cut off his foot if, 10 years down the line, there was some standard treatment that could help him.”
Looking for optionsThe Rauses went for a second opinion, and a third, and a fourth. They headed to Duke University, where they spoke to Dr. Robert D. Fitch, who has performed, since 1987, leg lengthening procedures on children who suffer congenital anomalies such as Jesse’s, or who have been injured. But Fitch, who currently performs about 75 limb lengthening operations a year, believed Jesse’s discrepancy was too much to make up. He recommended amputation.
The family went to the University of North Carolina at Chapel Hill, where Dr. Edmund R. Campion had also been offering the limb lengthening procedure. He, too, said amputate.
They scheduled doctor’s visits in Virginia, in South Carolina and in Delaware. They all said amputate.
The doctor in Delaware delivered an especially hard blow. He had trained Campion, and other doctors mentioned him as the voice of authority. If he couldn’t save Jesse’s leg, no one could.
“We pretty much cried all the way home from Delaware—an eight-hour drive,” Tom says. “It was the loss of hope and the loss of choices—the continual chipping away of hope. This doctor was built up as being the guy who, of everyone, was the expert. And he said amputate.” For the first time, the Rauses began to consider it. Jesse was 10 months old, and the clock was running out before the optimum time for amputation passed. But one last chance remained.
One more tryBefore the Rauses had gone to Delaware, they had found a Web site called Mothers United for Moral Support, or MUMS. The Web site linked parents of children with various ailments to other parents—a virtual support group. When Dawn typed in PFFD, only five moms came up with similar situations.
That was enough.
One of those moms responded to Dawn’s e-mail message with emphatic advice:
Talk to a Dr. Dror Paley in Baltimore.
“She said that, before any decision was made, whatever we do, we have to see Dr. Paley,” Dawn says.
Two days before Jesse turned 1, the Rauses sat in Paley’s waiting room at the Kernan Hospital in Baltimore, an orthopedic teaching unit of the University of Maryland. Paley, a tall, dark-eyed man with far-set eyes, wasted little time getting down to business.
“He smiled,” Dawn recalls, “and said, ‘I think I can help Jesse.’ “ “We don’t remember much of what he said after that,” Tom says, “because we both burst out crying. I do remember one thing: He said that on a scale of difficulty from one to 10, Jesse was a 5.”
Paley’s optimism about Jesse’s leg stemmed from his training in limb lengthening techniques. Although limb lengthening is nothing new, it has undergone major changes since the mid-1980s. Paley was at the forefront of the revolution, at least in the West. In the mid-1980s, he was the first North American orthopedist to study under a pioneering Russian surgeon, Gavril Abramovich Ilizarov, who had developed a superior limb lengthening device and technique behind the Iron Curtain. The device, called an Ilizarov (pronounced illi-ZAR-ov), is custom-made for each patient—a difference from other so-called external fixator devices commonly used in the West.
v Ilizarov’s contraption consists of a series of rings, which encircle the leg or arm to work like a scaffold, holding a set of pins in place. The pins look like ordinary bolts available at Lowe’s, but they are screwed directly into the bone.
Where the pins protrude from the leg into the scaffolding rings, they are fixed with a nut, which the patient turns a quarter of a millimeter, four times a day, to create a 1 millimeter gap between bones each day. And although the Ilizarov device gets all the attention—it looks like a medieval torture device—it’s actually the bone lengthening technique the Soviet surgeon developed that is truly revolutionary. “With our older techniques, we used to cut the bone with a saw blade, which generates heat and causes a small amount of bone death,” says Dr. Fitch, at Duke.
That thermal injury inhibited bone regeneration. And there were other mistakes, as well, Fitch says. Before Ilizarov, doctors would pull the severed bones far apart, and then attach an external fixator device that would crank the bones even farther apart—something like 3 mm a day. That would create a significant gap, which doctors filled with bone grafting to complete the lengthening.
“Ilizarov studied this,” Fitch says, “ and said, ‘Don’t cut with a saw, cut with a chisel type device. And don’t distract the bones apart, keep them close together, and let the body’s natural healing process occur.” When the bones remain close, they start spinning new material to bridge the divide. If the gap is continually ruptured, in the small, quarter-millimeter amounts that Ilizarov recommends, the body continually produces new bone material, and there’s no need for grafting.
“He was brilliant,” Fitch says.
But because Ilizarov practiced in the Soviet Union, nobody in the West knew of his accomplishments. Finally, in the mid-‘80s, Italian physicians were invited to Russia. They were the first to leak the news. Paley was eager to hear more. Fluent in Russian, as well as six other languages, Paley found a way to study with Ilizarov himself. In 1986, after returning from Russia, Paley started offering the revolutionary limb lengthening technique. To date, he and another surgeon at Kernan have done 4,000 operations, and Paley was recently featured on 20/20 and Oprah.
Difficult road aheadThe Rauses left Paley’s office in a daze. They had been lifted, on the very thinnest of life lines, back from despair, and yet they didn’t want to get too excited.
“He told us this is not a procedure to be taken lightly,” Dawn says. “Our family would be impacted by it. Our extended family. Our friends. Our neighbors. He made it clear this is a huge commitment.” Jesse, Paley said, would need at least three limb lengthening operations to close the eventual 12-inch discrepancy. What’s more, Jesse required some preliminary work to align his hip correctly, and his knee would take constant monitoring.
Not only would the Ilizarov need to be installed, it also would need to be removed. There would be body casts. There would be painful physical therapy. There would be infections at the points where the pins penetrate the skin. Each lengthening procedure meant, at least, nine months of dedicated, constant care.
And while Paley was confident that, in the end, he could make Jesse’s legs even, his approach was significantly more aggressive than other doctors’. “You have to factor in how many lengthening procedures you are going to put the child through,” says Fitch, who began offering the Ilizarov method at Duke in 1987. “Complications can occur with these heroic lengthenings, where you get into injuries to joint cartilage and nerves.” Muscles, too, have to grow with the bone, and they can suffer damage and injury. For the Rauses, though, the complications and the dangers were worth it. “We drove home from Baltimore, and for the first time, we didn’t have to worry anymore, or wonder what else was out there,” Dawn says. Jesse had his first operation, the pelvic correction, when he was 18-months old; it required him to wear the Ilizarov device for 10 weeks. One morning, as the Rauses were waking up, they heard one of their sons walk into their room. They figured it was Taylor or Shawn.
“It was Jesse,” Tom says. “He was the only one of our four kids who crawled out of the crib. And he did it with 10 pounds of metal on his leg. That about sums Jesse up.”
“You can tell that God gave Jesse everything he needed to deal with this,” Dawn says.
Round OneJesse had his first limb lengthening operation Jan. 26, four months before his 5th birthday. Several inches above Jesse’s knee, Paley chiseled apart the bones of the femur as if cracking through ice. Then he drilled holes through skin, muscle and bone for the seven pins, six above the knee, one below. Finally, he attached two full rings and two half rings to complete the apparatus. About a week later, Jesse’s parents started turning the screws, millimeter by millimeter.
“It was horrible at first,” Dawn says. “He was in a lot of pain and was on a narcotic, and it seemed to alter his personality. He was moody, irritable and very, very demanding.”
The pain came not so much from the device that bore into him—and that did hurt—but from the exercises he endured, three hours a day, five days a week, to stretch and maneuver his thigh muscles, knee joint and hip. Jesse spent his days gaining minute movements, tiny flexes, faint bends under enormous pain. He cried. Dawn cried.
Six weeks passed. Dawn and baby daughter Victoria, born in April of 1998, stayed with Jesse in Baltimore, living out of a room in a Ronald McDonald House. When Dawn wasn’t by Jesse’s bedside, she was battling insurance companies over claims (their policies paid for the bulk of the $75,000 procedure, but not without fights) and stitching snaps and Velcro closures into Jesse’s clothes.
Tom held down the family home in Apex, getting Taylor and Shawn to school, with help from Dawn’s parents, who came in from Ohio. Finally, Jesse was sent home on Feb. 28, but within a week of arriving, he began losing mobility in his knee; he needed three hours of physical therapy each day and was only getting one because of insurance complications. When Dawn, Jesse and Victoria went to Baltimore for a checkup, Paley was alarmed. He told her that unless Jesse stayed in Baltimore and kept up with the intensive physical therapy, the operation would be a bust. “I had packed for a one-day trip,” Dawn says, “and wound up staying two months.” “This has taken a toll,” Tom says, “but it has also built character for everybody involved. You’re forced to deal with it—us as parents, the other kids as siblings. They can look at other people with disabilities now and relate better, have more compassion.”
Moving onJesse doesn’t like to talk about the pain of those early days.
“It hurt a lot at first,” he says, “but it doesn’t hurt now.” The pin turning stopped in June, but even now, his hour-long physical therapy sessions at HealthSouth Pediatric Therapy in Raleigh are tough. Therapist Denise Bergeron uses card games and other activities to divert his attention while she presses on his leg, stretching his knee and thigh muscles. He winces, sometimes he whimpers, but he never cries or lashes out. “Ow, ow, ow,” he says softly, burying his head into a blue gym mat on the floor of the small therapy room. “Does it really hurt, or is it just stretchy?” Bergeron asks.
“It really, really, really, really hurts,” Jesse says calmly, all the while playing cards.
Bergeron is openly fond of her patient, but she keeps a stern hand. She pushes. Jesse winces.
Now 5, Jesse started kindergarten last week, wearing the Ilizarov. In six weeks, he’s scheduled to have the device removed. That will mean a body cast for four to six weeks to keep him from breaking his newly generated bones. This first procedure gave Jesse’s leg 2 ˝ inches, enough so that the left shoe of his pair of white leather Converse All-Stars now has just four inches of $90 dollar orthotic lift.
It was a lot of growth, but it was about an inch or two less than the Rauses had hoped for. Jesse’s bones don’t regenerate very quickly, and so his parents are coming to terms with the idea that Jesse may need more than three operations.
“If we can only get 2.5 inches each time, and the discrepancy is 12 inches as an adult, you can do the math,” Tom says.
Jesse is not fazed. Not the least bit shy or self-conscious, he has a ready answer to the kids and grownups alike who ask him about wearing a hardware store’s worth of metal on his leg.
“This makes my leg longer,” he says in his matter-of-fact manner. But the Ilizarov isn’t something Jesse’s much interested in. What he really wants to show visitors is his scooter. He got it for Christmas—a skateboard with handle bars—and when he hops on that thing and zips down the drive of his house in the Walden Creek subdivision, he often stops traffic.
It’s a sight to see. He plants his short leg on the board, and paddles his other leg to gain speed—lots of it—and then he hops the long leg on the board, too, and zooms. Forget the Ilizarov.
“I can go faster,” he says.
His dad knows all too well how fast Jesse can go. “I’m fully confident that Jesse is going to be able to do anything he wants to do,” Tom says, “and that he’s not going to be held back by anything. And I’m sure that would be the case if we hadn’t had the surgery, or if the surgery were unsuccessful. I don’t think he’s going to be held back, period.” Jesse, after all, has always been fully equipped to win. Staff writer Sarah Avery can be reached at 829-4882 or firstname.lastname@example.org