At present, there’s no cure for most neuromuscular diseases — although the day is rapidly approaching when genetic and drug therapies will change that situation. For immune-mediated disorders, drug therapies can be very effective for treating or reversing symptoms (see “Neuromuscular Disease Descriptions”).

Medical interventions have increased the life span and improved quality of life for many medically fragile children. These interventions focus on treating or delaying symptoms, enhancing physical mobility and social interactions, and preventing heart and lung complications.

Some common interventions include:

Assistive Equipment

• Communication devices allow students with weak speech muscles to convey their needs and thoughts.
• Computer adaptations and software allow those with limited movement to type and surf the Internet.
• Small adaptations help with everyday tasks: special feeding utensils and cups, straws, a foam rubber cylinder for grasping pens and pencils, tape recorders, etc.
• Standing frames allow nonambulatory students to continue to bear weight on their legs, promoting healthier bones, better circulation and a straighter spine.
• Transfer boards and mechanical lifts make it easier and safer to move a nonambulatory student.
• Walkers, wheelchairs and foot, ankle and leg braces keep children safely mobile and part of the social scene.

Cardiac Care

When the muscle layer of the heart weakens and doesn’t pump effectively (cardiomyopathy), children may experience fatigue and lethargy, swelling in the legs and feet, cold extremities, digestive problems and other symptoms of poor circulation. Drug treatments available through a cardiologist may enhance heart muscle function. Some children benefit from a pacemaker, and some may even undergo a heart transplant.

Nutritional Support

When the muscles used in swallowing and chewing are weakened, there’s a risk of dehydration, malnutrition, choking or respiratory infections caused by inhaling food or liquid into the lungs (aspiration).

• Gastrostomy tube (g-tube, feeding tube or PEG) is a tube permanently placed through the stomach wall, so a liquefied diet can be fed directly into the stomach. Those who use a g-tube because eating is difficult and time-consuming still can have food and drink by mouth for pleasure and extra nutrition. But those who use a tube because of choking and aspiration probably shouldn’t eat anything by mouth.

Physical and Occupational Therapy

Keeping the body flexible, upright and mobile combats some of the side effects of neuromuscular disease. Such therapy also benefits academic performance by allowing children to do more and stay healthier. Your student’s IEP may specify that a certain time must be set aside each day for him or her to work with a physical or occupational therapist. PT or OT interventions include:

• Range-of-motion exercises and stretches prevent contractures or freezing of the joints of the knees, hips, feet, elbows, wrists and fingers.
• Hand splints keep the wrists and fingers in a good position.
• Swimming pool exercise, especially in a warm pool, is often easier for children with weak muscles.
• Proper body mechanics ensure safe transfers of nonambulatory students by aides, teachers and other caregivers, so neither party gets hurt.

Medications

Although only a few drugs are approved for use against the effects of neuromuscular disease, ongoing clinical trials are constantly seeking to expand that number.

One medication that has proven effective in some neuromuscular diseases is prednisone. This steroid may be taken by children with Duchenne muscular dystrophy (DMD) and some other neuromuscular conditions because it slows the loss of muscle function and increases muscle strength, providing for a few more months to a few more years of leg and arm use.

Prednisone’s undesirable side effects include weight gain, loss of bone mass, thinning of the skin, raised blood pressure and blood sugar, depression, and difficulties with thinking, sleeping and controlling behavior.

It’s important to carefully monitor the diets of children on prednisone, and to be aware of its behavioral effects.

Respiratory Care

When weak respiratory muscles make it difficult to effectively move air in and out of the lungs, children may experience headaches, mental dullness, difficulty concentrating or staying awake, and nightmares. In addition, weak chest muscles make it hard to cough effectively, leaving the lungs more susceptible to infection. In some children, a simple cold can rapidly progress into pneumonia.

• Assisted ventilation: Help with breathing may be given either through an external mask or “sipper” tube, or by way of a tracheostomy, in which a tube is inserted directly into the airway to deliver air to the lungs.
• Cough assist machines: These machines help bring up lung secretions to keep the bronchial system free of infection.

Surgery

• Heel cord and foot joint surgeries are used to treat ankle contractures and joint deformities in order to prolong walking. Students usually are out of school less than two weeks.
• Scoliosis surgery is performed on older children and adolescents with serious spinal curvature. Timing of the surgery hinges on the student’s growth, lung function, discomfort level and how fast the curve is worsening. Metal rods with hooks are inserted into the spine, and students can spend 6 to 10 weeks out of school. (When possible, these surgeries are scheduled during the summer.)

After scoliosis surgery, students sit much straighter and have fewer problems with breathing. They often require a new wheelchair and adjustments in classroom desk height. Unfortunately, some abilities, such as reaching, may be negatively affected by this surgery.