Surgery to Restore Muscle Power in FSH Dystrophy

The difficulty started for Claire Walker when she was in kindergarten, when her bodily education teacher noticed that she couldn’t do sit-ups like another kids and that her back seemed “lopsided.” Claire’s parents took her from their home in Louisiana to see Yadollah Harati, an MDA-affiliated neuromuscular illness specialist at Baylor university of medicine in Houston.

There, she learned she had facioscapulohumeral (FSH) muscular dystrophy, a muscle-wasting illness that typically affects the muscles of the face and upper body but can also affect the back and legs.

Claire had spine-straightening surgical treatment at age 9, which solved her back problems, but later, she began getting problems utilizing her arms. each of her shoulder blades (the medical term is scapulae) “would flip out like a wing” when she attempted to accomplish for things, and that component of her body was a continuous sore spot. by the time she was in college, “just picking up a glass of drinking water got to get just a little bit difficult. I would drink a whole lot more to the side, not right out in entrance of me. It got old.”

Barbara Williams has similar childhood memories. In college in Oregon, she couldn’t increase her hand in course with no supporting it with the other arm. “The nuns would scream at me,” she says.

In her 20s, Williams worked as a grocery store cashier but was obtaining weaker. getting to stand up all morning wasn’t the hard part, she recalls. “It was the reaching out to grab things to scan them. i had been achy around my shoulder blades all of the time.” Williams, who is left-handed, says her right shoulder always gave her a whole lot more difficulty than her left.

Williams had acknowledged for some time that she and her brother had FSH dystrophy and had noticed doctors in the Portland area. She’d attempted rub treatment and other pain-relieving techniques that didn’t seem to get helping much. in the mid-1980s, she consulted a surgeon, who advised in opposition to an operation, telling her she’d shed so much mobility from this kind of surgical treatment that she’d never have the ability to brush her teeth or take treatment of her hygiene needs. She accepted this verdict.

A ‘marvelous operation’ — for some
Irwin Siegel, an orthopedic surgeon who co-directs the MDA clinic at Rush-Presbyterian-St. Luke’s medical center in Chicago, is really a pioneer in the area of thoracoscapular fusion surgery. This operation attaches the shoulder blade (scapula) to the back of the rib cage (thorax) and can be helpful for that problem affecting Walker, Williams and others with FSH dystrophy.

Occasionally, the operation could possibly be helpful in other neuromuscular disorders, that include limb-girdle or Becker muscular dystrophies, but in the superb vast majority of these cases, upper body weakness is a whole lot more generalized than it is in FSH dystrophy, which renders the procedure useless.

Unfortunately, Siegel says, thoracoscapular fusion (“scapular” fusion for short) is “rarely performed, even today, because fairly few patients or doctors are mindful of its existence.”

Siegel calls the surgical treatment a “marvelous operation,” but cautions that it has to get carried out by the right surgeon around the right patient.

The right surgeon, he notes, is experienced with shoulders and with muscular dystrophy, and the right patient is one with weak scapula-stabilizing muscles but a powerful deltoid — the muscle that connects the upper arm bone (humerus) to the shoulder blade and collar bone.

In FSH dystrophy, Siegel explains, the muscles that normally hold the shoulder blade in place are usually so weakened by young adulthood that the arm-lifting deltoid loses its mechanised advantage. once the scapula doesn’t provide a stable program for that deltoid to pull in opposition to — a situation called scapular winging, because the scapula sticks out like a wing once the someone tries to lift his arm — even a fairly powerful deltoid can’t increase the arm very high or for very long.

One of the most troublesome deficits takes place once the someone tries to proceed his arm up high in entrance of the body (called flexion), the type of motion needed in reaching for any glass inside of a high cupboard or a e book on a high shelf. Equally problematic is reaching out to the facet (a motion called abduction), that include when pulling grocery store items from the conveyor belt. These movements are particularly difficult if they must be sustained or repeated.

St. Louis surgeon J. David Thompson utilizes a anxiety strap wiring technique with one or two bone blocks to attach the scapula to the ribs. Chicago surgeon Irwin Siegel and colleagues use a metal plate in place of the bone block(s).
With help from Harati, their doctor in Houston, Walker’s parents found orthopedic surgeon J. David Thompson, who practices at Cardinal Glennon Children’s Hospital in St. Louis.

Thompson, who says Siegel was “instrumental in my obtaining started [in scapular surgery] and generous with his time,” has now performed a whole lot more than 40 scapular fusions, typically in individuals with FSHD. He agrees with his mentor that in the right arms and with the right patient the operation can be very helpful in improving function.

Fortunately, Thompson says, there’s a fairly easy and accurate way to tell who will benefit from the surgery. acknowledged as the manual compression test, it involves getting an examiner hold the scapula flat in opposition to the back, simulating temporarily what the surgical treatment would do permanently, whilst the patient tries to lift her or his arm up and out.

If he can suddenly lift the arm higher and for longer with the examiner’s hand pressed to his back, the patient’s deltoid is likely to get powerful enough to obtain considerable benefit from scapular fusion surgery.

The manual compression test can be dramatic. “That’s when they all make a decision they want the operation,” Siegel says, noting that patients are frequently mazed at how powerful their upper arm muscles are once the shoulder blade is held steady.

Not for that casual surgeon
“It’s helped out tremendously,” Walker says of the surgical treatment she had on one shoulder when she was 20 and the other at 21. (Her surgeon, Thompson, says most of his patients have been in their late teens or 20s, with a few in their 30s.)

Thompson is enthusiastic, too, but, he cautions, scapular fusion is really a “major operation.” significant postoperative pain and also a prolonged time period of restricted arm motion are certain, whilst respiratory and other problems aren’t uncommon.

Thompson advises individuals to possess one shoulder operated on at a time, so they’ll have at least one arm free for activities of daily residing throughout the healing process.

Siegel recommends obtaining somebody who has encounter with thoracoscapular fusion.

“It’s not something for that casual surgeon,” he cautions. “It’s not really a typical operation even for somebody who specializes in shoulder surgery.”

Procedures vary

The actual methods utilized to attach the scapula to the ribs and the kinds of postoperative restrictions that follow vary somewhat among doctors and even for patients who have the same doctor. “There isn’t loads of cookbook about neuromuscular disorders,” Thompson says.

In the past, methods of pinning down the scapula ranged from the very flexible, that include synthetic mesh materials or fascia (fibrous tissue), to the very firm, utilizing metal screws.

Neither technique is as popular today. The flexible attachments, whilst effective at first, stretched out over time and failed to hold the shoulder blade down. Screws, Siegel says, have a good opportunity of protruding through the bone and damaging or irritating gentle tissue in the chest area.

Today, surgeons are likely to make use of flexible wires combined having a bone graft (small pieces of bone, used from the patient or from one more source) to do the attachments. Holes are drilled through the scapula and cable is wound around three to five ribs.

Siegel reinforces the attachment having a metal plate over the scapula, through which holes are also drilled, matching those in the bone underneath. (Think of the plastic strip that frequently reinforces the margin of loose-leaf notebook dividers to keep the rings from tearing the paper with wear.)

Thompson prefers to make use of blocks of bone to substitute for that metal plate and says experiments have convinced him that the special way of threading and tying the wires called a “tension strap wiring technique” gives a durable but flexible connection. (See illustration above.)

Wires could possibly break some time after surgery, but that normally doesn’t matter once the bone graft has sealed the attachment. In fact, when the wires turn out to be uncomfortable, they can be removed later.

Surgeons understand that getting a bone graft from the patient — most frequently from the hip — exposes him or her to a separate procedure, with a whole lot more pain and also a second incision and scar. But, at least for some, it’s still a better way to go than utilizing donated bone, that is available from bone banks. Thompson calls the patient’s personal bone the “gold standard” for grafts.

Postoperative routines vary, too.

Siegel’s patients stay inside of a gentle arm immobilizer for about three several weeks (see below) and then use a sling for any whilst longer. At about 6 weeks, his patients start exercising with the arm supported by one more someone and then, at 10 to 12 weeks, they exercise a whole lot more vigorously to keep the deltoid muscle strong.

Thompson’s regimen involves arm immobilization for about eight several weeks after surgery, followed by bodily therapy.

His patients are instructed to stay aside from vigorous activities for 6 months. He also advises them to stay out of college for 6 several weeks or an office job for three to 6 weeks.

Getting over it

Both of Walker’s surgeries went pretty smoothly, she says, particularly the second one, but there were some hard periods.

After surgery, the arm around the affected facet has to get kept close to the body for 6 to eight weeks.
She was in the hospital for 10 days with the first procedure and a few days much less with the second. after each surgery, she had excessive fluid buildup and irritation of the membrane around her lung, with pain requiring extra medication.

The prolonged post-op immobility wasn’t easy, either.

“You must keep your arm for your facet from the elbow up for eight weeks,” she says. “There’s a bone graft in between the ribs and the shoulder blade with cable keeping it together that can only take so much pressure, so you’re restricted in what you can lift up to the point where the bone graft has solidified.”

Thompson instructed Walker not to lift heavy things for nine months. She do bodily treatment for two months after each surgery, and she still does some muscle-toning exercises on her own.

Now 22 and working in the sales department of the corporation that markets outdoor equipment shelters, Walker has had no regrets concerning the surgery. She says her posture and appearance are much better, and her purpose has greatly improved.

“My back is flat around my shoulders, that is nice,” Walker says, “and I believe I gained probably sixty or 70 degrees of flexion.”

Williams, now 42 and residing just outside Portland in Clackamas, Ore., is happy concerning the surgical treatment she had many years after the first physician turned her down. She wishes she hadn’t waited so long.

“I believe I lost an awful whole lot of muscle,” she says of the years between the first and second surgical consultations. “I believe I would have been more powerful and recovered faster when i had been young, and that i would have gotten a whole lot more motion out of [the operation] if I had been more youthful and hadn’t lost so much muscle to start with.”

Williams sooner or later left the grocery store store, went into office work and then stopped working entirely to stay home with her two children. She had scapular fusion surgical treatment on her right shoulder shortly prior to turning 40 in 2001. Her physician was Kevin Smith of the college of Washington medical center in Seattle.

She utilized donated bone rather than undergo the procedure needed to make use of her personal bone, and she wore only a sling for about 6 several weeks after surgery. Her physician had her executing mild exercises the second morning after her operation, utilizing a pulley system hooked to the door.

Williams says she’s lost some ability to accomplish to the side, that include when passing things at the table or reaching across the entrance seat of the car, but she’s able to accomplish up higher and lift better. She can now grab a gallon of milk from the refrigerator and put it around the counter “like anybody else,” whilst prior to the surgery, the container would just “flop down by my side.”

Best of all, she says, “I don’t have that horrible burning pain between the shoulder blades and along the spine anymore.”

Smith was also the surgeon for Jesse McKee, an 18-year-old who works in his grandfather’s hardware and construction business in rural Whitehorn, Calif. McKee had each of his scapulae fused when he was 16, in separate methods about 6 months apart. He made a rapid recovery each time, a fact that he and his mother, Maryellen, attribute partly to his youth and general fitness.

McKee also utilized donated bone rather than his own. He utilized a sling for about 6 several weeks and then progressively began lifting things, progressing from capuccino cups to 5-pound weights and sooner or later to the 40-pound bags of soil he’s now able to throw into the back of the truck. He didn’t do any special exercises or therapy.

He says he’s definitely more powerful and looks better (he utilized to get teased at the swimming pool about getting “slouched over”), but he’s not quite as flexible as he was prior to the surgery. Recently, he’s also developed some problems with the thumb and also a finger on his left hand, which he suspects could possibly be from the stretched or pinched nerve, a problem he plans to ask his surgeon about soon.

A significant chunk of life

The expected drawbacks of scapular fusion surgical treatment include postoperative pain, with morphine usually needed for several days; several weeks to months of relative immobility around the operated side, with possible reduction of muscle power, at least in the brief run; and coverage to general anesthesia inside of a fairly lengthy surgery. (FSHD isn’t particularly likely to cause problems related to anesthesia, but the surgical team ought to be mindful of the patient’s muscular dystrophy. See “Coping with Anesthesia,” Quest, June 2000.)

There’s a fairly large scar from the incision over the scapula, even though appearance could possibly be enhanced through better posture and also a flatter back; and there can be some reduction of versatility because of the pinning of the scapula to the rib cage.

Scapular fusion operations are costly (although insurance organizations don’t seem to possess a problem in covering them for FSHD patients), plus they usually require at least several several weeks of your time aside from work with possible reduction of income.

Thompson says the surgical treatment “takes a significant chunk out of the person’s life. by the time you do both sides, you’ve used out a year.” Therefore, he says, individuals “need to get committed to executing it and realize what they’re obtaining into to obtain the most beneficial result.”

Risks which have been unexpected but deserve thing to consider include enough blood vessels reduction to require transfusions; fracture of the ribs or scapula throughout surgery; failure or breakage of the attachment of the scapula to the ribs; puncture of one of the membranes surrounding the lungs, with oxygen or blood vessels entering the chest cavity and compressing a lung (a problem that can usually be very easily treated); irritation of one of these membranes with excessive fluid manufacturing and pain (pleurisy or pleural effusion); infections; and damage, usually temporary, to the nerves that handle the arm.

Because the scapular fixation minimizes rib motion just a little bit, there can be a small decrease in the degree to which the lung can expand around the operated side. Usually, in individuals with FSHD, this slight reduction of lung capacity isn’t important, studies show.
Siegel says the operation requires three or 4 hours in the operating room and can be arduous for that surgeon in addition to the patient. He doesn’t carry out this kind of long methods himself anymore, even though he’s taught many more youthful specialists, such as some overseas.

A good result

Siegel points out that techniques and methods to rehab have enhanced whilst he’s been in practice and will carry on to do so.

Since her surgery, Barbara Williams can accomplish higher, lift better, and shift a gallon of milk from the fridge to the counter “like anybody else.”

“Over the years we’ve utilized much less and much less restrictive immobilization,” he says. “We got rid of the plaster cast and went strictly to the gentle arm immobilizer, and we progressively have begun to make use of that for much less and much less time. The techniques have improved, and the materials have improved,” he notes, and so has the understanding of how surgical treatment and muscular dystrophy interact.

“When you take any person with muscle illness and hold them immobile, they atrophy instead profoundly,” he says, and also this atrophy (shrinkage) of muscles can be hard to reverse later. “We could possibly have been unduly cautious,” he says of the overly restrictive post-op regimens of the past. “But that’s the studying curve.”

Thompson says the solution to success, from the surgeon’s point of view, is to “take a good history, do a good exam, identify patients who are good candidates for surgery, and be meticulous concerning the technique and the aftercare.”

But, he cautions, “this is not an operation where the physician does something and you’re fine. The physician does something and you [the patient] are going to make it work. The patient is required to undergo a time period of rehabilitation to make it work well.

“The determined patient is really the key to a good result.”

Both sides now?

Although a few individuals have undertaken scapular fusion surgical treatment on both shoulders throughout the same operation, orthopedic specialist J. David Thompson says he’s only carried out it once, a whole lot more than 10 years ago, and advises in opposition to it.

The patient, a nurse in her 30s with FSHD, had arranged for relatives to take treatment of her kids and thought she could keep both arms immobile for that required amount of time. But, Thompson says, that proved impractical, and one side, because of use by the patient, failed to fuse.

A second operation was necessary, and that, unfortunately, resulted in some nerve damage that took seven months to resolve.

“I’ll never do it again,” he says of the two-sided procedure. “She was very careful, but the patient cannot protect [both shoulders] enough. I believe it had been my mistake to do both at the same time.”

Leslie Van Alstyne couldn’t agree more. Now 25 and also a medical equipment evaluator in the Philadelphia area, Van Alstyne has just about recovered from extensive surgical treatment for her FSHD-related problems over xmas break throughout her sophomore year in college.

The surgeon, a specialist at a prestigious Boston medical center who was recommended by one of Van Alstyne’s doctors in Pennsylvania, suggested that he could operate on her spine and both scapulae at the same time and have her back in college by late January. That, however, wasn’t what happened.

Van Alstyne says she now realizes that the surgeon wasn’t very familiar with FSH dystrophy and didn’t recognize how much leg weakness she had — and that it would worsen, at least temporarily, from lack of muscle use after surgery. completely immobilized from the three procedures, Van Alstyne was in superb pain and unable to do something for herself for months.

She thinks that nurses who were trying to proceed her could possibly have inadvertently broken one of the scapular fusions when they were lifting her inside of a blanket “like a log” shortly after surgery. In any case, one facet failed to heal and had to get fused again later.

Meanwhile, Van Alstyne missed an whole semester of college, graduated late, and had to work for some time to regain her bodily and psychological confidence.

In hindsight, she says, she would have made sure the physician understood a whole lot more about her muscular dystrophy. But she’s glad she had the fusion surgeries.

“Cosmetically, it had been a superb thing,” she says, and she’s gained some range of motion and the ability to lift her arms higher. She does drinking water treatment and walking to sustain her power and is also able to work on a computer all morning and travel as needed for her job.

She says she’d do it all again — but one facet at a time.

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