Melinda Guttry, a physical therapist in Rehabilitation Services at the University of California, Los Angeles Health System, has worked closely with neurologist Susan Perlman and with Friedreich's ataxia (FA) patients since the mid-1980s.
Most of her clients with FA have been young adults, but she also has worked with people in their 40s and 50s. Below, she answers questions about the role of physical activity in FA.
A: It’s usually movement and coordination. The nerves aren’t conducting information consistently, so the muscles don’t receive consistent signals about holding a position or making a limb have a smooth, coordinated movement.
A lot of the problem is in outgoing coordination signals from the nervous system. But if you have a muscle that’s getting weaker because the person is not using it, then the muscle doesn’t activate as readily either. There can be problems with sensing position as well.
A: We try to have people work on exercise and work on stability, and use their muscles so that they’re reminding the muscles of holding the body erect or sitting upright or making smoother movements.
If I have the person moving from a sitting to a standing position, I use the parallel bars and have them practice. I start them out by helping them set their muscles and use their muscles in a sequence. Then, as they practice, they get a better feel for the sequence. They know which muscles they’re using and the feeling of the muscles as they coordinate them.
Parallel bars are heavier than they are, and they’re long. The bars are solid and stable, and people get an opportunity to isolate different muscles, setting their muscles so that their trunk is more stable.
On the parallel bars, if they’re trying to stand up, they might pull on the bars. I teach them to push down instead, because if they want to use a walker and they pull up on the walker, the walker won’t stay on the ground.
I also work with them on range of motion [putting a joint through its normal movement range], to help maintain flexibility.
A: Yes, because there are many, many nerve fibers, and they all don’t degenerate at one time. So it’s the practice of getting all the fibers that are still functioning to do the work, to give as much information to the muscles as possible.
A: I’ve seen good results. It depends on how much the person is able to practice at home.
Sometimes that’s a problem. It depends on the age of the person and how motivated they are to practice versus staying in their chairs and doing something else. Sometimes that’s more important to them than getting up and practicing walking.
But the more they’re up and the more they practice coordinating their muscles, the more they can stay up.