Previously, we’ve discussed cerebral palsy — the disability itself and how it affects evacuations from a school bus.

We also discussed Duchenne muscular dystrophy (DMD). DMD is characterized by progressive weakness of muscles, eventually leading to death in the individual’s late teens or early 20s.

In this article, we will discuss spinal muscular atrophy (SMA).

Specifics of SMA
SMA is a group of inherited diseases that cause progressive muscle degeneration and weakness which, like DMD, leads to death, but at a much earlier age.

The difference between DMD and SMA is that DMD is actually a disease of the muscle. With SMA, the problem is with the nerves that fail to function normally and do not send messages to the muscles to move. This causes the muscles to deteriorate.

SMA is a degenerative disease characterized by a dramatic loss of spinal motor neurons (the nerve cells in the spinal cord that cause the muscle fibers to function), resulting in muscle weakness, atrophy and, in the worst cases, loss of ability to swallow and breathe.

Sensation and the ability to feel are not affected. Intellectual activity is normal, and it’s often observed that children with SMA are unusually bright and sociable. Many of these children often have the ability to draw very detailed drawings.

There are four types of SMA, ranging from the more involved type where there is early infant death, to normal adult life with only mild weakness. The child is affected with SMA when the defective gene is inherited from both parents.

Those of us working with students who have SMA must realize the comprehensive medical approach that these children receive. This is important, as it might be necessary to consult with one of the medical specialists in regard to the student’s bus ride and an emergency evacuation.

These specialists can include pediatric neurology, pediatric pulmonology and pediatric orthopedic surgery, which may entail follow-up orthosis (i.e., braces) for the lower extremity and spine. Although physical and occupational therapy cannot change the progression of the muscle atrophy, therapy is important to prevent contractures of muscles and tendons and abnormal curvature of the spine (i.e., scoliosis).

A student with SMA is often seen by a respiratory therapist and a clinical nutritionist. We need to be aware that students with SMA will have difficulty protecting themselves from choking and that breathing complications are common.

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Implications for an emergency evacuation
The following must be taken into account when doing an emergency evacuation for a student with SMA:

• These students are usually in a motorized mobility device at a very
young age.
• These students will need to be either carried out of the bus or dragged out of the bus on an emergency evacuation device.
• These students will have extreme weakness of their trunk muscles and often cannot sit by themselves. Be sure that during an evacuation you protect and support the student’s head and back.
• As a student with SMA gets older, a tracheostomy and ventilator (i.e.,
a breathing machine) may become necessary to help the student breathe.

Plan well in advance to provide manual respirations for this student during an emergency. Also, practicing this drill will be critical to the student’s safety.

• Often, a nurse will ride the bus with a student who has a tracheostomy and ventilator. During an evacuation, the student will need to be disconnected from the ventilator. Respiration will be performed manually by the nurse. However, the transportation staff will also need to know how to perform manual respirations in case the nurse is unconscious due to the accident.

For a student being dragged out of the bus who needs to breathe with an Ambubag, use this recommended procedure:

• During the dragging process, the bus staff members drag the student, then pause to allow the nurse to use the Ambubag to breathe for the student, then resume dragging, pause again to let the nurse use the Ambubag, etc.
• Repeat this process until the student has been safely evacuated. The nurse will continue operating the Ambubag until medical help arrives. For this reason, it is critical that the nurse be involved with emergency evacuation drills.

Summary
It is important to recognize that students with SMA are aware of an evacuation, but physically they are unable to help themselves. During evacuations, it is critical to be in constant verbal communication with these students. They will need reassurance that we will be doing everything we can to get them out of the bus safely.

Jean M. Zimmerman is supervisor of occupational and physical therapy for the School District of Palm Beach County (Fla.). She is the author of Evacuating Students With Disabilities, a comprehensive manual and training course written in conjunction with the Pupil Transportation Safety Institute. The program can be purchased at www.ptsi.org or by calling (800) 836-2210.