1. Osteogenesis Imperfecta
Caring for school aged children in a community program

2. 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
Osteogenesis imperfecta (OI), also known as brittle bone disease, is a genetic disorder characterized by fragile bones that break easily. OI affects both bone quality and bone mass. A person is born with this disorder and is affected throughout his/her life. In addition to fractures, health issues frequently seen in children who have OI include:
Short stature;
Weak tissues, fragile skin, muscle weakness, and loose joints ;
Bleeding, easy bruising, frequent nosebleeds and in a small number of people heavy bleeding from injuries;
Hearing loss may begin in childhood and affects approximately 50% of adults;
Breathing problems, higher incidence of asthma plus risk for other lung problems ; and
Curvature of the spine.
OI is caused by a mutation on a gene that affects the body’s production of the collagen found in bones and other tissues. People with OI have less collagen than normal or a poorer quality than normal. It is not caused by too little calcium or poor nutrition. Approximately 35% of children with OI are born into a family with no family history of OI. Most often this is due to a new mutation to a gene and not by anything the parents did before or during pregnancy.
It is estimated that OI occurs once in every to births. OI occurs with equal frequency among males and females as well as across races and ethnic groups.

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
OI is highly variable, ranging 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). The specific medical problems a person will have depend on the degree of severity. The characteristic features of OI vary greatly from person to person, even among people with the same type of OI, and even within the same family.
OI Type I is the mildest and most common form of the disorder.
OI Type II is the most severe form.
OI Type III is the most severe type among children who survive the neonatal period.
People with OI Type IV are moderately affected. Type IV can range in severity from relatively few fractures, as in
OI Type V is moderate in severity. It is similar to OI Type IV in terms of frequency of fractures and the degree of skeletal deformity.
OI Type VI is extremely rare. It is moderate in severity and similar in appearance and symptoms to OI Type IV.
Recessively Inherited Types of OI (Types VII and VIII):
Some cases of OI Type VII resemble OI Type IV in many aspects of appearance and symptoms.
Other cases resemble OI Type II, except that infants have white sclerae, small heads and round faces.
Cases OI Type VIII are similar to OI Types II or III in appearance and symptoms except for white sclerae.
OI Type VIII is characterized by severe growth deficiency and extreme under-mineralization of the skeleton.

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
OI Type I is the mildest and most common form of the disorder. It accounts for 50% of the total OI population.
Type I is characterized with mild bone fragility, relatively few fractures and minimal limb deformities. The 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. Others experience multiple fractures of the long bones, compression fractures of the vertebrae, and chronic pain. The intervals between fractures may vary considerably.
After growth is completed, the incidence of fractures decreases considerably.
Blue sclerae are often present.
Typically, a child’s stature may be average or slightly shorter-than-average, but is still within the normal range for the age.
There is a high incidence of hearing loss. Onset occurs primarily in young adulthood, but it may occur in early childhood.
Dentinogenesis imperfecta (brittle teeth) is often absent.
People with OI Type I experience the psychological burden of appearing normal and healthy despite needing to accommodate their bone fragility. The absence of obvious symptoms in some children may contribute to problems at school or with peers.
Significant care issues that arise with OI Type I include gross motor developmental delays, joint and ligament weakness and instability, muscle weakness, the need to prevent fracture cycles, and the necessity of spine protection.

5. Treatment There is no cure for OI. Treatment goals Treatments
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
Treatment
Doctors who see children and adults with OI include primary care physicians, orthopedists, endocrinologists, geneticists and rehabilitation specialists.. Other specialists such as a neurologist may be needed.
Treatments focus on minimizing fractures and maximizing independent function and general health.
Treatments include
Physical therapy and safe exercise including swimming;
Casts, splints or wraps for broken bones;
Braces to support legs, ankles, knees and wrists as needed ;
Orthopedic surgery, often including implanting rods to support the long bones in arms or legs;
Medications to strengthen bones; and
Mobility aids such as canes, walkers, or wheelchairs and other equipment or aids for independence may be needed to compensate for weakness or short stature.
There is no cure for OI.

6. 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 
Lifting∕Handling
When handling an infant with OI, all movements should be slow, methodical, and gentle.
Never push, pull, twist, bend, apply pressure to, or try to straighten an infant’s arms or legs.
Lift a baby with OI onto your shoulder by placing one hand under the buttocks and legs, and the other hand under the shoulders, neck and head. Lean over the baby so that there is a shorter distance to lift.
Do NOT lift the baby from under the armpits.
When holding an infant with OI, keep your fingers spread apart to provide a wider base of support and an even distribution of support pressure.
Hydrocephalus occurs in a large percentage of children with Type III OI. Extra care is needed to support the head if it is oversize for the small body.
Be aware of where the baby’s arms and legs are at all times to avoid awkward positions or getting a hand or foot caught.
Infants should be repositioned frequently during the day. Beneficial positions for an infant with OI include being held, carried, held on a caregiver’s shoulder, and side lying.
It is important for babies with OI to held and touched by parents and other caregivers, and that they are allowed to explore independent movement. Supporting infants in a variety of positions on the parent’s shoulder and eventually including side lying, develops muscles that will help with head and neck control and later on with sitting.

7. 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
Diapering∕Burping
Do not lift by the ankles to change a diaper. Lift the baby by the buttocks. Spread your fingers apart as far as possible, and put your hand under the buttocks, with your forearm under the baby’s legs to prevent them from dangling.
Burping an infant with OI should be done very gently, with soft taps, and possibly with padding over the hand, or by gently rubbing the baby’s back.

8. 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
Avoiding head flattening
All infants with OI have soft skulls. To prevent skull malformations, every effort should be made to reduce pressure on the back of the head. The following strategies are beneficial.
Put gel pads under the infant’s head when he or she is on her back.
Position the infant in a propped, side-lying position.
Frequently change the infant’s position throughout the day.
Carry the infant on your shoulder or in an approved sling carrier.
Avoid leaving the infant in a car seat for long periods.
Helmets have been used in some infants who have OI, but they are not universally -recommended. In severely affected infants, the additional weight of a helmet may make the already challenging task of gaining head and neck control even more difficult.

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
Car seats
An approved car safety seat geared to the child’s weight and his or her ability to sit-up is appropriate.
A padded washable cover for the seat is a good idea. However, it is unsafe to add extra padding that was not provided by the seat manufacturer.
Other important car seat features include a well-padded harness and a head-hugger support pillow.
Some severely affected infants with OI may require a car bed.

10. 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.
Physical Environment
For children who are mobile, it is important to avoid cluttering the floor with toys/objects so avoid tripping and falling.

11. 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

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.
EMERGENCY RESPONSE PLAN (Infants and Toddlers )
Any of the following situations may indicate a fracture.
Child resists moving the sore body part
Swelling or bruising over a bone
Child has deformed limb
Child is not using the limb
Fussiness may be a sign of fracture
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.
Do not move the affected area unless it is absolutely necessary to move the child out of harm’s way. If staff needs to move the child, keep the affected area as still as possible and avoid jarring movements.
Make the child comfortable while waiting for a parent or other designated person to arrive.
If the child becomes chilled or nauseated, provide a blanket, a basin, or whatever else the child might need.
Do not provide food or drink; if the child needs surgery to set the fracture, this will interfere with safe administration of anesthesia.
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. Makeshift splints can be formed using a pillow, a towel, or even a magazine wrapped around the affected limb. Splints can be tied on using an elastic bandage or strips of cloth. Take care not to tie the splint on too tightly, as that will cause pain and decrease circulation. Minimize additional pain by applying the splint quickly but very gently, avoiding sudden or jarring movements. Most general first-aid classes instruct people in how to apply a splint. .

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