Shree Pandya tests Scott's strength.

Say "physical therapy" and most people think of World War II movies with wounded heroes struggling with weights and pulleys, athletes nursing injuries in whirlpool baths, and heart attack survivors sweating on treadmills.

Say "physical therapy" and "muscular dystrophy" in the same sentence and you may get the same uncertainty from anxious parents that you do from skeptical insurance company representatives.

"Does it really help in those diseases?" is a frequent response. "I mean, what can you do in a degenerative, genetic disease?"

That's what Yvonne Nichols of Horseheads, N.Y., heard from her insurance company when she tried to get physical therapy last summer for her 8-year-old son, Scott, who has Duchenne muscular dystrophy (DMD).

"The insurance company termed the therapy 'inactive care,'" Nichols says. "They call it inactive care when it's not going to improve a condition, and they refused to cover it."

Fortunately for Nichols, she and Scott live in an area where the schools are very good about complying with the requirements of the Individuals with Disabilities Education Act (IDEA), which mandates that every child is entitled to a full education, including physical education, with whatever special services and adaptive equipment may be necessary to make that happen (see "Getting Started With Physical Therapy and More").

Scott's DMD was diagnosed in kindergarten, when his physical education teacher noticed that his motor skills weren't developing as expected and encouraged the family to investigate.

That summer, after the diagnosis, Nichols swung into action. "We went through the Committee on Special Education," she says. "I contacted the school, because I had a friend who had a son with behavioral problems. They went through the CSE, so I knew a little about it and what to do."

Scott was classified as "orthopedically impaired" and assigned to a physical therapy program involving a half-hour of therapy two days a week during the school day. The therapy mostly involves stretching his heel cords and the muscles at the backs of his thighs, but Scott is also learning to manage his energy level and stay safe. There's some fun involved, too, like walking on 4-inch stilts.

"I've heard about red tape with the CSE," Nichols says, "but we had no trouble whatsoever. We've had nothing but good experiences."

A New Way to Look at PT

"In the old way of looking at things, rehabilitation meant you were going to get better," says Sheila Hayes, a physical therapist associated with the MDA/ALS Center at Columbia-Presbyterian Medical Center in New York.

That's no longer the case, she says. In fact, the American Physical Therapy Association recently started a special section on degenerative diseases.

Shree Pandya is a physical therapist and educator who's been involved with the MDA clinic at the University of Rochester (N.Y.) for many years. Nowadays, she works mostly with patients - including Scott Nichols - who've been in research studies at the university.

Pandya sees physical therapy as "helping people to remain at their highest functional level possible at any given point in time within the constraints of their disorder."

Physical therapy overlaps a great deal with occupational therapy (see "Skills for the Job of Living," Quest, vol. 6, no. 5; and "Skills for School and Play," vol. 6, no. 6), but there are some differences. For the most part, although both disciplines deal with maximizing function, OT is concerned more with the small muscles, particularly those of the hands, while PT is concerned more with large muscles, such as the legs, and with mobility.

Today, physical therapy is an integral part of the treatment of almost any neuromuscular condition and is usually included in the medical plan for everyone with these conditions and in the school or preschool program for children.

How Much and What Kind?

Unfortunately, there's a lack of research on exactly what kind of PT and how much is ideal in each of the various neuromuscular disorders.

Most therapists agree that a certain amount of stretching and range-of-motion exercises (these ROMs keep joints supple by putting them through their normal range of motion in space) is almost always a good idea in neuromuscular diseases. Such maneuvers tend to slow down or sometimes even prevent the development of contractures, the freezing of joints that aren't moved.

Most therapists also agree that a certain amount of exercise is good for cardiovascular health, and that some weight bearing, where possible, can head off the development of the bone-weakening disorder osteoporosis. Most also agree that strenuous exercise in certain types of metabolic muscle disorders (for example, phosphorylase deficiency, or McArdle's disease) can lead to serious muscle damage and perhaps kidney damage as well. (This is because proteins leaking out of damaged muscle cells reach the kidneys, where they're toxic.)

Shree Pandya shows Yvonne Nichols how to do stretching exercises with Scott.

In periodic paralysis, attacks of paralysis are often brought on by resting after strenuous exercise. Cramping of muscles, paralysis of muscles or cola-colored urine are warning signs to stop exercising immediately in any muscle disorder.

For some people, just activities of daily living, like walking up and down a few stairs, getting in and out of chairs, and turning from side to side in bed have to suffice for physical therapy. Even those simple things can help preserve both strength and flexibility.

But when it comes to more concerted efforts to increase strength or muscle bulk in the muscular dystrophies, disagreements and uncertainty surface.

Several of the dystrophies (Duchenne, Becker, some limb-girdle dystrophies and at least one congenital dystrophy) are known to result from fragile muscle membranes. These membranes are sheaths that surround each muscle fiber (long cell) inside the muscles. In several muscular dystrophies, the sheath is weakened because it lacks one of several membrane proteins. Extra stress on the membranes, many experts have reasoned, may hasten muscle degeneration.

On the other hand, muscles are designed to be stressed, and leaving them alone can also hasten their deterioration and interfere with overall fitness.

Pandya, despite many years of experience in the field, isn't sure about the exercise question. "I would rather be cautious," she says, "but sometimes I wonder if we have been too cautious." She says the most serious quandaries are posed by "college-age kids with facioscapulohumeral dystrophy or limb-girdle dystrophy, who do not have as much of a progressive disorder as Duchenne dystrophy and are into exercise with weights and equipment."

Pandya prefers that they do walking or swimming instead of weight lifting, but she says there aren't a lot of studies. "Basically we try to summarize what we know from the literature, that a certain amount of exercise is good for all of us, as well as keeping weight down, eating a healthy diet, all those things." She's not enthusiastic about exercises that may tear membranes and damage muscles cells that have a hard time renewing themselves (which describes muscles in muscular dystrophy).

She recalls a research study on boys with DMD conducted many years ago. Exercising the thigh muscles three times a week temporarily strengthened these muscles, but when the children were tested six months and a year later, they had lost the gains.

Because of this study and others, Pandya isn't keen on overzealous strengthening exercises in severe muscular dystrophies like DMD. She's more interested in increasing the child's overall flexibility. Of equal concern to Pandya is a youngster's experience of childhood.

"The child has to be a child first and a child with muscular dystrophy next," she says, and she tries to build a PT program around that philosophy.

Don't Overdo It

Jenny Robison, a physical therapist who's long been associated with the MDA clinic at Vanderbilt University in Nashville, Tenn., has much the same philosophy.

"I try not to overload my patients," she says. "But in my years of experience, I can see kids whose parents have done exercises and night splints [to keep feet in proper alignment] and surgeries, and they're in so much better shape than kids who haven't had any intervention. I think it works, but it's a lot to do." Physical therapy "prevents or slows down problems like contractures and keeps people in a better functional position to do things that they want to do, to live life as well as they can. It also helps people by getting them the right equipment," Robison says.

Concerns about exercise also exist in motor neuron disorders like spinal muscular atrophy (SMA) and amyotrophic lateral sclerosis (ALS).

In these disorders, motor neurons, the nerve cells that control muscle movement, are lost, leaving muscle fibers "orphaned" - without a nerve supply. Some investigation of exercise in motor neuron disorders has been done, Hayes says, and the consensus is this: When motor neurons die or don't function, their neighboring motor neurons can take over, at least for a while, supplying more than their share of muscle fibers with nerve signals. But these new connections are fragile and under stress. Overexercising the muscles can stress the new connections still further, perhaps hastening damage (Hayes doesn't think this is likely), but almost certainly making it harder for a person to function by the next day.

Hayes' recent experience is with ALS patients, but she says conclusions about exercise in ALS should apply equally to people with SMA.

In her view and that of other experts in motor neuron disease physical therapy, she says: "It's OK to do moderate strengthening for muscles that are uninvolved, that show no overt weakness. But once a person is starting to exhibit weakness, it's best to stay away from weight machines, free weights or any type of resistance exercises. If the goal is to improve function, then exercising to the point where muscle fatigue impairs function is counterproductive."

Pandya checks Chad O'Connell's wheelchair for comfort and proper body alignment.

A Child First

Children especially need to have a PT program that works for them in the context of their other activities. No one understands Pandya's message about children needing to be children first better than Sherrie Shannon of Fairview, Pa. Her son, Christopher, an 11-year-old with DMD, has had the wrong kind of physical therapy, and too much of it, she says.

Shannon says she was directed to a physical therapist in her area who was approved by Medicaid but knew next to nothing about MD.

"Christopher was only the second kid with muscular dystrophy that she had ever seen," Shannon says, adding that the therapist told her she'd had only about four hours of education on neuromuscular disorders in her five years of PT training.

"She started him on wall squats - where you lean against the wall and squat down and have to get back up - and on sit-ups," Shannon recalls. Christopher has recently begun a pool therapy program, a welcome change, but Shannon says of the therapist, "She's not listening for Christopher's cues saying 'I'm tired.'"

Shannon describes her son's schedule: "From 7:30 to 8 in the morning, he does leg exercises and manual stretches at home; then at a quarter to 9 he gets more stretches at school, at 12:30 more stretches, more when he gets off the bus and then again before bed. On the days when he has PT after school, he goes from school to therapy."

Shannon is all for the stretches - in moderation - and for flexibility exercises in general, but, she says, "I don't see that strengthening has helped him in any way, because he's still declining at an average rate." Fortunately, Shannon has recently been able to bring in a consulting therapist with years of experience in DMD who's been helping their primary therapist. Christopher's program is undergoing modifications and shifting more toward water therapy.

But Shannon is also concerned with the quality of Christopher's life. She describes how the physical therapist had him doing an exercise called "side-lying subluxation - where he lies on his side and has to lift his leg and kick behind him. That's supposed to help strengthen the muscles that bend the hip." Shannon says, "You can do that standing, doing kickball. I'd rather let him kick the ball."

Christopher also likes sled hockey, a game his mother describes this way: "He sits down in a sled, someone pushes him, and he hits the puck." Winners on Wheels, a program she found through her MDA office, sponsors sled hockey in her area.

Nowadays, when Christopher asks if he can play instead of doing exercises, Shannon tells him, "We'll wait a half-hour for the exercises. Go play."

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