Osteogenesis Imperfecta

1. OsteogenesisImperfecta Caring for school aged children in a community program 2012-04-01

2. OsteogenesisImperfecta • 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

3. 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

4. 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

5. 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

6. 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

7. 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

8. 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

9. 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

10. 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.

11. 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

12. 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: • Contact the child’s parent/guardian. • If you are unable to contact the parent/guardian or alternate contact, call 911/EMS. • Inform the paramedics that the child has OI.

13. 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.

14. 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

15. 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

16. Child specific information • Type of OI • Safety precautions • Emergency Response Plan Health Care Plans are located in child file and binder