STAYING FLEXIBLE, UPRIGHT AND MOBILE
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.
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.)
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.
Treating Scoliosis
Exercises to keep the back as straight as possible and advice about sitting and sleeping positions can be obtained from your physical therapist. Professional opinions about the value of special spine-straightening wheelchair contours are not consistent. Most physical therapists believe the usual "hammock" seat and sling back in the standard wheelchair are bad for a boy with muscular dystrophy, but not all agree about custom-designed, contoured seats or back inserts. Ask for a few opinions, and beware of miraculous claims from companies that make these products.
Likewise, opinions are divided on the use of corsets, jackets and back braces to prevent or slow the development of scoliosis. Some doctors and therapists like them and prescribe them, but others are afraid they may give everyone a false sense of security that "something is being done" about the spinal curvature and therefore postpone the definitive treatment -- surgery -- until it's too late.
Surgery for scoliosis is usually done when the doctors judge that the curve is progressing fairly quickly but the boy's respiratory function is still fairly good. If it's found that the curve is progressing quickly but at the time the boy's respiratory function is already severely affected, the risk of the surgery may be too great, and the boy will be told he has to live with his spinal curvature, however far it progresses.
This is a situation to avoid. If your child is doing exercises, wearing a corset or brace or sitting in a specially designed wheelchair to treat his scoliosis, make sure the doctor is keeping a close eye on things just the same. A growing boy with DMD and a curving spine needs frequent examinations and X-rays of his back, along with close monitoring of his respiratory function and suitability for surgery. The X-rays should be done with the boy sitting up to show what happens to the spine in this position.
Spine-straightening surgery, which is done by inserting rods with hooks into the spine, is much less risky than it was just a few years ago. However, there are risks, and these should be discussed with your doctor. Make sure the boy understands what to expect and, if appropriate to his age, has a say in the decision-making process.
There is an increased risk of complications from anesthesia in boys with Duchenne, so the anesthesiologist should be fully informed about this condition by the boy's neurologist.
In rare situations, straightening the spine worsens breathing or swallowing, while at the same time alleviating other problems. A careful evaluation of which muscles a young man is using for these functions should be done before surgery is undertaken.
After the surgery, the spine will be straight and rigid, and adjustments will have to be made. For example, the wheelchair will need alterations, the van's head height may no longer fit (the boy will seem taller), and different ways of transferring the boy to and from the wheelchair or bed will have to be learned.
The ability to sit up straight, look better, breathe more easily and sleep more comfortably can bring about a marked improvement in a boy's attitude toward life.
Braces and Standing Frames
Braces and standing frames can help prolong walking and standing in muscular dystrophy. Braces used in DMD include the ankle-foot orthosis, or AFO, and the knee-ankle-foot orthosis, or KAFO, also known as a long leg brace.
Early in the disease, an AFO can help in walking and can slow the development of contractures. AFOs are sometimes prescribed for night wear to keep the foot from pointing downward while the child is sleeping.
Later in the disease, long leg braces may be prescribed. The boy with Duchenne at this stage walks by swinging his braced legs forward from the hip, a somewhat precarious and cumbersome maneuver. Walking by this method is actually much slower than using a wheelchair, and many boys therefore prefer the wheelchair, since they can keep up better with their friends. They also avoid falls by using the chair. Because of their concerns about injury liability, schools sometimes require a boy with DMD to use a wheelchair.
Standing for a few hours each day, even with minimal weight bearing, is good for the body in general, promoting circulation, the health of bones and a straight spine. If a boy has lost his walking ability and chooses not to use long leg braces, a standing walker or standing frame can be used for at least part of the day. There are wheelchairs available that will tilt into a standing position.
Wheelchairs
Sooner or later, all boys with DMD will need wheelchairs. A wheelchair has to fit the child's needs and those of the parents who'll be taking care of him and transporting the chair.
The physical or occupational therapy department of the medical center where you have your MDA clinic appointments will help you choose the best wheelchair for your child.
Your child may not need a wheelchair until sometime between the ages of 10 and 12, and even then may not need it full time, but you may want to prepare him for this eventuality ahead of time. If the child senses that a wheelchair is something the parents have long dreaded or that every therapy has been directed at "keeping him out of a wheelchair," then the event almost has to seem like a defeat. Dr. Michael Brooke, who heads the MDA of Canada, advises adults to help the child view the wheelchair as a form of transportation not unlike a bicycle, the main difference being that a bicycle has wheels in a line and the wheelchair has wheels side by side.
Of course, there are parents and even some children who lean toward the other extreme, wanting a wheelchair as soon as possible because they fear falling or don't want to make the effort to use braces. There's nothing inherently wrong with using a wheelchair a little sooner than absolutely necessary, except that a constant sitting position hastens the development of contractures and other complications of immobility, such as fat accumulation and loss of bone mass.
Discuss your feelings and let your son discuss his feelings with the doctor and therapist. Perhaps a compromise can be reached, such as using a wheelchair in school or at the mall but getting some walking in at other times.
Other Mobility and Positioning Aids
There is an almost endless array of mechanical and other assistive devices available today that aid mobility and save wear and tear on a parent's back.
Among the simplest and least expensive devices is a transfer board. You can also get mechanical (usually hydraulic) lifts, shower chairs and electronic beds, some of which can turn a person from side to side as well as sit him up or down. Needless to say, some of these are very expensive. Insurance policies vary as to which aids they cover. (This guide's Resources section lists some sources of these items; others are advertised in MDA's Quest magazine.)
Choose products wisely, and check with your local MDA office to see if their "loan closet" has any equipment you can use.
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