STAYING FLEXIBLE, UPRIGHT AND MOBILE
Part 1

Although there is no cure for DMD, the disorder's impact can be changed significantly by keeping the body as flexible, upright and mobile as possible. This is accomplished with a combination of physical therapy, exercises, bracing and the use of a wheelchair. Surgery may also be used.

How Contractures Happen

As a boy with DMD gets older, his weakness progresses. The weakness in Duchenne is generally considered symmetrical, meaning that both sides of the body get weaker at an approximately equal rate. However, the muscles on each side of a particular joint don't necessarily get weak at the same rate. The stronger muscles pull the joint in their direction and, along with gravity and the usual position of the body (for example, sitting), joints become fixed in a certain position over time. These fixations of the joints are known as contractures.

In DMD, the knees and hips usually become flexed in the sitting position. The feet usually end up pointing inward and downward. Elbows may also end up flexed. Hands may point downward because of wrist contractures, and fingers may stay flexed because of contractures in the finger joints.

Preventing and Treating Contractures

Contractures can be a devastating complication of muscular dystrophy because they interfere with normal functioning of the affected body part. They can usually be postponed or slowed down, if not entirely prevented.

Exercise to keep joints as supple as possible is a crucial part of a Duchenne child's life. Your doctor or physical therapist will show you how to gently take each joint through its range of normal positions (known as range of motion exercises) on a regular schedule. This kind of exercise, which should be done only after careful guidance (doing it wrong can do more harm than good), can help keep tendons from shortening prematurely.

Sometimes, surgery is done to relieve contractures. One type of surgery, called a tendon release, is often done to treat ankle and other contractures while the child is still walking (around age 8 to 10). A tendon is the fibrous tissue that connects muscle to bones. For ankle contractures, the Achilles tendon at the back of the heel is cut, allowing the foot to resume a natural position. After the surgery, the child is usually put into a cast and then into leg braces to keep the contracture from immediately reforming.

As the front calf muscle weakens, the stronger rear calf muscle pulls the heel upward, pointing the toes. Surgery to cut the Achilles tendon prevents permanent contracture.

With the braces, the child may be able to walk for a while longer than he would have otherwise.

Some doctors don't recommend this type of surgery because they believe it may do more harm than good to subject the child to this kind of stress and pain and to have him off his feet even for a short time during the recovery period, which itself can lead to additional muscle weakening.

Dr. Irwin Siegel, an orthopedic surgeon and MDA clinic co-director at Rush-Presbyterian-St. Luke's Medical Center in Chicago, has had decades of experience operating on boys with DMD. He says it's essential that they stand and walk the very day of their tendon release surgery. That way, he says, they avoid the complications associated with immobility and gain the benefit of the surgery.

Talk to your doctor and to people who've had or haven't had surgery for contractures. Factors to consider include how well your child tolerates discomfort, how motivated he is to wear braces to continue walking and how much time he's likely to continue walking after the surgery. If he's old enough, the boy himself should be involved in the decision.

Spinal Curvatures (Scoliosis)

A lateral (side-to-side) curvature of the spine is known as scoliosis. This kind of problem is common in muscular dystrophy (and in many other conditions), and its origins are somewhat similar to the origins of joint contractures. Kyphosis, a "hunchback" type of curvature, is also often present in DMD. The two together are known as kyphoscoliosis; for simplicity, the problem is usually just called scoliosis. (The third kind of spinal abnormality, lordosis, is a "swayback" curvature. It's usually seen while the boy with Duchenne is still walking.)

The muscles of the back weaken, but not always symmetrically. The muscles on one side may be a little weaker than those on the other, pulling the flexible spine out of alignment. The spine is made up of a string of bony disks known as vertebrae, and these can bend or be rotated, within limits, in all directions. If a boy is right-handed, he'll usually reach for things from his wheelchair with his right hand and may lean in that direction. He may control his service dog with one hand or use the controls for the power wheelchair with one hand on one side. (Physical therapists recommend that a power wheelchair for a boy with muscular dystrophy be designed with the controls in the center, to discourage this constant leaning.)

The best time for scoliosis surgery in DMD is between ages 11 and 13, before "vital capacity" (the volume of air that can be exhaled after a deep breath) falls too far.

During scoliosis surgery, metal Luque rods are inserted to hold the spine in place while the vertebrae fuse.

Severe scoliosis can interfere with sitting, sleeping and even breathing, so it should be prevented. If the curve is progressing rapidly, surgery is often advised, usually between ages 11 and 13.

Next... Staying Flexible, Upright and Mobile, Part 2