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July 29, 2011  |   Comments (1)   |   Post a comment

Specific Special-Needs Evacuations: SMA

Part 3 of this series focuses on spinal muscular atrophy, which is a group of inherited diseases that cause progressive muscle degeneration and weakness. Knowing how to carry passengers with this condition on an evacuation device and including a nurse in training drills will help to ensure their safety.

by Jean M. Zimmerman


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Katie, who has spinal muscular atrophy, received her first motorized wheelchair at the age of 2.

Katie, who has spinal muscular atrophy, received her first motorized wheelchair at the age of 2.

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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Read more about: evacuation drills

This all means that the school bus driver must be diligent in their duties and strong. Is there a weight lifting requirement for school bus drivers? not in most states. most school bus drivers are considered OLD and have diminished capacities. How are they going to help our children? Something to think about.

lexismom    |    Aug 24, 2011 09:03 AM

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