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
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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 PRECAUTIONS
School 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 PRECAUTIONS
Classroom/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 PRECAUTIONS
Gym
• 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 PRECAUTIONS
Fire 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 PRECAUTIONS
Transportation
• 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
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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