Osteogenesis Imperfecta

Caring for school aged children in a community

program

2012-04-01

Osteogenesis Imperfecta

• Brittle bone disease• genetic disorder • Characterized by fragile bones that break

easily. • Affects both bone quality and bone mass. • Other health issues frequently seen in

children with OI:– Short stature– Weak tissues, fragile skin, muscle weakness,

and loose joints – Bleeding, easy bruising, frequent nosebleeds– Hearing loss– Breathing problems– Curvature of the spine

Types of OI

• 8 types• OI types range from a mild form

with no deformity, normal stature and few fractures to a form that is lethal during the perinatal period (prior to and after birth).

• Medical problems a person will depend on the type of OI

• OI varies greatly from person to person, even among people with the same type of OI, even within the same family

Type I

• mildest and most common form• 50% of the total OI population• mild bone fragility• relatively few fractures• minimal limb deformities• child might not fracture until he or she is

learning to walk. • Shoulders and elbow dislocations may occur

more frequently than in healthy children• Some children have few obvious signs of OI or

fractures while others experience multiple fractures of the long bones, compression fractures of the vertebrae, and chronic pain.

• Appear healthy yet need to accommodate for bone fragility

Treatment

• There is no cure for OI.

• Treatment goals– Minimize fractures– Maximize independent function and general health

• Treatments– Physical therapy and safe exercise– Casts, splints or wraps for broken bones;– Braces to support legs, ankles, knees and wrists as needed – Orthopedic surgery– Medications to strengthen bones– Mobility aids

• Some children may need physical or occupational therapy to maximize their skills and independence

SAFETY PRECAUTIONSSchool setting

Physical barriers should be addressed if they interfere with a child’s participation– Stairs– Restrooms with narrow or heavy doors– High sinks, stalls too narrow for a wheelchair– Play structures with stairs– Inaccessible hands-on work areas

Possible strategies• providing an aide to assist a child in the

restroom• portable ramps and wheelchair lifts• lowering lockers, shelves, soap dispensers• providing a low desk or work surface

SAFETY PRECAUTIONSClassroom/hallway

• Crowded hallways and classrooms may pose problems

• Suggested strategies– Allow child to leave class several

minutes early– In multi-level school buildings, allow

child to use elevator – Allow the child to select a seat that

is easy to get to– Provide an extra set of books

SAFETY PRECAUTIONSGym

• Participation is very important for children with OI. Make every effort to involve the child in the same activities as everyone else with appropriate modifications

• If you are not certain if a child with OI should participate discuss it with the parent/guardian.

• May be restricted from playing contact sports

• Avoid activities that jar or twist the spine

• Wearing a helmet and knee/elbow pads for sports like bike riding and roller blading, is recommended.

• Good fitting shoes help to support the ankles, and prevent tripping/slipping

SAFETY PRECAUTIONS Recess

• Special playground equipment for children with disabilities

• Children with OI may also be able to use traditional equipment, such as slides or jungle gyms, with or without adult assistance.

• remind all children that safe and considerate play is important for preventing injury

SAFETY PRECAUTIONSFire evacuation

• An evacuation plan should be established and practiced during routine drills

• Suggested strategies– assign a particular staff person to

accompany a child with OI – In a multi-level school building, a

specific plan for evacuation must be made.

– It is possible for two adults to carry a child in a wheelchair down steps safely.

SAFETY PRECAUTIONSTransportation

• May need someone to assist them on and off the bus

• Because most school buses do not have seat belts, may be at increased risk during an accident or if the bus stops short.

• If the child’s need for a seat belt is included in the written plan, this should be discussed with the school.

• Back of the bus often provides a bouncier ride than the front of the bus. This may be dangerous for a child with OI

EMERGENCY RESPONSE PLAN

The following situations may indicate a fracture– Child complains of pain in a bone that gets

worse with movement– Swelling or bruising over a bone– Child has deformed limb– Child is not using the limb – Child winces or looks like that may be

uncomfortable during routine play or exercises

If any of the above situations occur:1. Contact the child’s parent/guardian.2. If you are unable to contact the

parent/guardian or alternate contact, call 911/EMS.

3. Inform the paramedics that the child has OI.

EMERGENCY RESPONSE PLAN

• Do not move the affected area unless it is absolutely necessary

• Listen to the child’s advice. • Make the child comfortable. • Provide a blanket, a basin, or

whatever else the child might need. • Do not provide food or drink• Staff should only apply a splint if the

parent has instructed them to do so or if the child must be moved before a parent or other caregiver arrives.

Health Care Plans

• Type of OI• Safety precautions• Emergency Response Plan

Health care plans are located in the office

Document relevant events and actions in health care plan

Health Care Plans

• Type of OI• Safety precautions• Emergency Response Plan

Health care plans are located in the office

Document relevant events and actions in health care plan

Child specific information

• Type of OI• Safety precautions• Emergency Response Plan

Health Care Plans are located in child file and binder