Doctors pioneer limb-lengthening procedure
(MCT) -- BALTIMORE - Chandler Crews sat on a bench outside of Sinai Hospital in Baltimore one recent day, put her feet on the ground and grinned.
Sitting this way was impossible for the 20-year-old from Arkansas just a few years ago because she was born with achondroplasia, a form of dwarfism in which limbs don't grow in proportion with the torso. With bowed legs and a 3-foot-10-inch stature, she said she was unable to do many such "normal" things.
She couldn't drive the family car or go for much of a walk without back and joint pain or even wash her hands in most sinks.
Now she stands just under 5 feet tall, thanks in part to a new procedure that was pioneered in part by Dr. John Herzenberg and Dr. Shawn Standard at Sinai Hospital, who had been searching for years for a better way to lengthen the limbs of those with trauma and genetic disorders such as dwarfism.
Limb lengthening itself is somewhat controversial among some with dwarfism who question the need to change. Some also wonder where surgeons should draw the line between medical necessity and cosmetic increases to height.
But Crews had no qualms, citing the pain she once experienced just trying to walk.
"Just a year ago, I was six inches shorter," Crews said. "I couldn't sit with my feet on the ground. Can you imagine what that was like?"
The new procedure is a radical departure from the past method, which involved forcing screws into bones through skin and muscles and building a sort of scaffolding, which Herzenberg likened to "a medieval torture machine."
The apparatus, called an external fixator, pulled the bone apart after it was surgically broken. New tissue would grow slowly in the gap, eventually hardening into new bone. It was effective but the system was cumbersome, painful and prone to infection. Doctors had to be careful not to turn the screws too much and risk a gap too big to heal.
The Sinai doctors found various new devices, mostly less reliable and unapproved by the U.S. government.
So Herzenberg and Standard worked with an Irvine, Calif.-based company called Ellipse Technologies and another doctor in California to develop a new system.
Doctors surgically insert a titanium rod inside the thigh or shin bone, or both. The rod contains a small magnetic motor and gear box that is used to slowly lengthen the rod. It's powered by a hand-held magnetic field generator placed atop the skin at the site of the magnet four times a day for about a minute.
Like the external fixator, when the bone is pulled apart - at a rate of about a millimeter a day - tissue and eventually new bone fills the gaps. The procedure can stretch each part of a limb about three inches. A bone typically takes at least two months to lengthen and three months to solidify. The rod is removed about a year later.
Herzenberg, who has been working in orthopedics for 35 years, called the innovation a "game changer" because of the simplicity for patients. He and Standard are among a handful of doctors using the Precise rods and have inserted them in about 100 patients, out of about 800 or 900 who have gotten them worldwide. Other hospitals using the system include St. Mary's Medical Center in West Palm Beach, Fla., and Shriners for Children in St. Louis.
The Precise system was first launched in 2012, and the Food and Drug Administration approved a revamped version in February that is stronger so patients can bear weight on their legs earlier. It also can be used on smaller patients.
The Sinai doctors have used the Precise system on patients ages 69 to 7, when limbs are large enough for the rod.
"This is more comfortable and safe and precise," Herzenberg said. "But it's still limb lengthening and doesn't solve every problem."
Surgery still is needed to correct malformed legs, and another procedure is done to insert the rods, which must be threaded inside the bone without causing new problems. Patients can't walk during the lengthening period, and bone doesn't always grow as planned. Patients need close monitoring for months, so they need to stick close to the hospital.
And the system is expensive. The rod alone costs $13,000, or about twice the external fixator's price. All told the tab can reach $100,000, though the doctors said it can be covered by insurance.
Crews endured external fixators during a first round of leg lengthening four years ago, which gave her about 6 inches, in addition to about 2 inches she gained when her bowed legs were straightened. She also used that method to lengthen her arms in between leg treatments.
Offered the Precise method last year, she said she was thrilled to shed the scaffolding, which left scarring and always seemed to be in the way. Even though her bones have hardened, she still returns to Sinai for occasional physical therapy.
Standard said just getting a few extra inches can change patients' lives in ways big and small.
"The thing I hear from boys is that they don't have to use a step stool in the bathroom," he said. "My goal is to help people be more functional."
That's why the Sinai doctors shy away from purely cosmetic lengthening, something they believe is done more regularly in Asia.
But some doctors worry others will not be so discerning.
It is likely to draw attention from new patients - and doctors - because it's much more user-friendly, said Dr. Sanjeev Sabarwal, president of the Limb Lengthening and Reconstruction Society and a pediatric orthopedic surgeon specializing in spinal and limb deformities.
"Caution is needed," said Sabarwal, also a professor at Rutgers-New Jersey Medical School. "This needs to be in the hands of people who know how to bail themselves out of complications, and even before that they need to recognize there is a problem that needs to be worked out. For certain things, you can't go back in time."
Sabarwal doesn't want this to become "like a face lift," done assembly-line-style in clinics. But he called the system an advance and has "cautious optimism" that it will improve the lives of patients with deformities. He said he expects to use it himself on his older patients.
Concerns over limb lengthening extend beyond medical circles to some in the dwarfism community, who believe they should not be viewed negatively because of the way they were born.
The Little People of America takes no position on limb lengthening, but a spokeswoman for the support and advocacy group said its medical advisory board found there was only a cosmetic benefit, not a medical one, and there is little known about the long-term consequences.
"As an organization we've chosen to say it's an individual decision, and we really encourage individuals to do their own research," said Leah Smith, director of public relations for the group, which reports there are about 30,000 people in the United States with a form of dwarfism.
"We're not here to knock anyone's decision," Smith added. "Personally, I've never considered it. I've never questioned or wanted to be something else."
Crews understands the view, but said she sees her procedures as important to her health. Her back and leg pain is gone. She's more independent. She'll be able to go for long walks, improving her long-term well-being.
Like others of her generation, Crews turned to Facebook to tell her story. She launched a group called L.E.A.D., or Limb Extension Awareness for Dwarfism, that "invites all to openly discuss the concept of limb lengthening without disparagement and negativity."
The condition she was born with occurs in 1 in 15,000 to 40,000 newborns, according to the National Institutes of Health.
People can decide for themselves, she said. They also can understand the commitment it takes for the procedure.
There have been sacrifices: She's spent months away from home and endured 18-hour drives from Arkansas to Baltimore with her mother, as well as surgeries, wheelchair confinement and physical therapy.
She still walks a bit slowly. And she still needs to have her last rods removed and her many scars smoothed over.
But the experience convinced her she wants a career in medicine, helping children.
For now, Crews is happy just to "look my age" and for little things like being able to order at the counter of a coffee shop.
"I'm the same person," said Crews. "But taller."
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