Over the last several issues of SCHOOL BUS FLEET, we’ve looked at a variety of medical conditions and how they affect emergency evacuations from the school bus.

In the fifth edition of the series, we will discuss evacuating students who have osteogenesis imperfecta (OI).

Specifics of OI
OI is a genetic disorder that is commonly called “brittle bone disease.” The disorder is inherited, and both genders are affected. OI is characterized by bones that break easily, often from little to no apparent cause.

It has been found that children who have OI have a problem with the development of collagen in their body. Collagen can be described as the framework upon which bone and tissues are built. If the collagen is defective, bones fracture more easily and muscles lack tone.

There are currently four known types of OI.

Type I OI:
• This is the mildest and most common form.
• These children have half the normal amount of collagen. However, this collagen is structurally normal.
• They may have muscle weakness, joint laxity and flat feet along with dislocations and sprains.
• Life expectancy seems average.

Type II OI:
• This is the most severe form.
• Infants are quite small at birth, and they are born with multiple fractures, an unusually soft skull and an unstable neck.
• Almost all infants with Type II die at or shortly after birth.

Type III OI:
• These children are born with fractures, and X-rays may reveal healed fractures that occurred before birth.
• They may have anywhere from several dozen to several hundred fractures in a lifetime. With these children, surgical correction of long bone bowing and scoliosis is common.
• They may need supplemental oxygen.
• Life expectancy varies.

Type IV OI:
• This is considered the moderate form.
• These children will have frequent fractures that decrease after puberty.
• They have mild to moderate bone malformation.
• Life expectancy appears to be average.

With all four types of OI, fractures decrease after puberty.

Researchers are beginning to identify additional types of OI, namely Type V and Type VI.

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The type of OI is determined based on clinical and radiographic data. When working with a student with OI, it is important to focus on his or her particular abilities, strengths and weaknesses rather than the particular OI type.

OI treatment involves caring for fractures, maximizing independent mobility and function, and developing optimal bone mass and muscle strength.

Orthopedic surgery can be done to insert metal rods into the long bones to reduce malformations and control fractures. Medications developed for perimenopausal osteoporosis are currently being tested for OI.

Implications for evacuation
Here are some key factors to keep in mind for evacuating students with OI from the school bus.

1. Just the impact of an accident may cause a fracture for a student with OI. The staff must be extremely careful in how they move and handle the student.

2. When students with OI have to be evacuated from the bus, they will be best protected in their mobility device (unless it is motorized — see No. 3), especially if it is a customized molded seating system. Bus staff must be extremely careful as they move the mobility device out of the bus.

3. If a student with OI is in a motorized mobility device, it will be necessary to remove the student from the device.

Instead of “dragging” an emergency evacuation device and the student down the bus aisle, lift and carry the student as much as possible to avoid hitting the floor. This will reduce impact to fragile bones. Two people lifting from the same side of the student, while cradling the entire body, may put less strain on any one part of the student’s body.

4. A student with OI would not physically participate in an emergency evacuation drill. However, the student will need to be “walked and talked” through the process to know what would happen in an emergency evacuation.

5. Be sure to let emergency personnel or bystanders outside the bus know about the student’s medical condition. Inform them about the student’s brittle bones, and tell them to be extra careful when touching or moving the student.

6. Immediately let paramedics know about this student. A broken bone could create an injury that would need to be attended to immediately.

Summary
Obviously, students with OI do need extra care while being evacuated from a school bus. In spite of the urgency during an actual evacuation, we must try to lift and move these students slowly and carefully.

Also, as noted above, we would not actually evacuate a student with OI during a drill. Rather, we would “walk and talk” the student and the staff through the evacuation procedures.               

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.