Teen Athlete With Debilitating Deformities Back On Feet After Complex Surgery At

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Teen Athlete With Debilitating Deformities Back On Feet After Complex

Surgery At Cedars-Sinai Medical Center

http://www.medicalnewstoday.com/articles/116904.php

After seven years, Woodward, 16, is getting a new pair of shoes.

Not even a new car could elicit the excitement the Canadian teenager

feels about this seemingly ordinary purchase, made possible after

painstakingly complex surgery at Cedars-Sinai Medical Center's

Charcot-Marie-Tooth/Hereditary Neuropathy Center.

Since childhood, has contended with the debilitating-and

initially mystifying-symptoms of Charcot-Marie-Tooth (CMT) disease, a

neurological disorder that typically affects the nerves and muscles

in the feet, causing varying degrees of pain, deformity and

disability.

" Imagine walking on two fists, " describes Glenn Pfeffer, M.D., a

distinguished foot and ankle surgeon who directs the Cedars-Sinai

Charcot-Marie-Tooth/Hereditary Neuropathy Center, one of just two

such specialized centers in the U.S.

Now, can't wait to slip into golf shoes-instead of the clunky

sneakers that for so long accommodated his malformed feet.

" Golf is my main sport, " explains the 6-foot, 190-pound high school

junior, who once ranked near the top of his age group. " The shoes

should help me improve my swing, and I'll be able to walk the

tournaments now. "

When was 5, Woodward says " mother's intuition " told her

something just wasn't quite right about the way her son walked and

the shape of his foot. She took the youngster to the family doctor

and a podiatrist in Edmonton, Alberta, but neither offered an

explanation.

As time passed, pursued his love of sports-golf, hockey, soccer,

lacrosse and water polo-with increasing difficulty. " It got to a

point where he couldn't keep up, " remembers his mom, who watched with

concern as his feet became more misshapen.

Finally, at age 10, was referred to a specialist who immediately

diagnosed Charcot-Marie-Tooth disease, a hereditary neuropathy that

affects one in 2,500 people. CMT causes progressive deterioration of

nerves and muscles that control balance and gait and can trigger

painful and disfiguring contractions of the feet. In more advanced

cases the hips, spine and upper extremity become involved. Even

breathing can become difficult in some patients. The condition often

appears in childhood, though symptoms are usually subtle, leading to

misdiagnosis.

" It was such a relief to know what it was and what we were dealing

with, " recalls of the CMT diagnosis, a further shock since

neither she nor husband Geoff nor other family members had the

disease. " The next challenge was what to do about it. "

After appointments with five different orthopaedic surgeons in

Canada, the family explored the Web for a physician with the

expertise to tackle the complex case. Through the Hereditary

Neuropathy Foundation in the U.S., the Woodwards learned about

Pfeffer, who has treated hundreds of patients with CMT over the past

20 years.

Pfeffer first encountered the disorder as a young surgeon, when a

vibrant teen patient timidly removed her shoes. " This young girl's

feet were twisted and deformed like the limbs of an old oak tree, "

Pfeffer remembers. " This was the kind of deformity a 90-year-old

might develop-not a healthy adolescent. It made quite an impression. "

CMT's unique complexity-and latent emotional toll-fueled Pfeffer's

keen interest in the disorder and potential therapies. " Adolescence

is a very vulnerable period in life, " he explains

Though 's classmates were supportive, he admits to

feeling " pretty isolated from friends, " primarily because CMT

sidelined him from the sports he loved. His mother concurs: " There

were definitely feelings of frustration. He just couldn't keep up. "

was eager to schedule an appointment with Pfeffer, before her

son's naturally upbeat disposition and self-confidence took a

downturn.

" Dr. Pfeffer answered our questions the way we'd hoped, and we had

total confidence he could do the surgery. "

This highly specialized " custom " surgery is actually a compendium of

intricate procedures-more than 25 in all-designed to " remodel " the

foot by correcting both muscle and bone deformities. Pfeffer likens

the surgery to turning a " gnarled oak tree into a beautiful willow. "

Unlike the fusion surgery of the past, which left the foot frozen

with little movement, surgical advances have enabled " motion sparing "

of joints to allow greater range of motion, flexibility and balance.

In 2007, Pfeffer successfully operated on 's first foot-with

dramatic results. The teen was eager to return in May 2008 for repair

of his second foot. After a six-week recovery in Los Angeles, the

family was scheduled to return to Canada-and normal life. may

need additional tune-ups down the line, says Pfeffer, but the major

work is behind him.

This likely won't be the Woodwards last visit with Dr. Pfeffer. Their

daughter a, 12, also has CMT, though her symptoms have developed

more slowly. plans to be a source of support in the years

ahead: " I can tell her what CMT is all about and help her through

it. "

Pfeffer fears many people with CMT are suffering needlessly, not

realizing the dramatic improvements specialists can offer.

" There are a large number of Americans with CMT who sit at home,

don't go to high school dances, don't play sports, don't get jobs-all

because they don't know what can be done for them, " he says.

Pfeffer hears from former patients who, after surgery, are elated to

put on " normal " shoes for the first time, to run or stand without

pain, to go on dates and to start college .

" When you take away the burden of CMT, these young people finally

begin to fly, " says Pfeffer.

's mother has witnessed his transformation first-hand: " I've

never seen a happier, more outgoing child. "