Case 18: Osteogenesis Imperfecta Flashcards
What is the Drug class that helps prevent loss of bone mass by inhibiting osteoclast activity, which in turn decreases bone resorption; used off-label in individuals with OI
Bisphosphonates
What is the bone mineral density
Amount of calcium and other mineral matter per unit of skeletal area … measurement is typically used to assess fracture risk and bone health
What is a Brace worn on the lower leg and foot that provides circumferential support with anterior and posterior shell components
Clamshell ankle foot orthosis
What is a osteotomy
Surgical fx of a bone , typically done to realign a deformed or rotated bone
What is the general PT POC/goals for OI
- improve LE strength and endurance
- progress standing tolerance and ambulation w and w/o AD
- max safety
What is the PT intervention for OI
- gentle strengthening of LE
- activities to progress stnading tolerance with initiation in pool —> land
- gait training
- stairs
- HEP
- parent eduction on how to safely progress weight bearing in order to mimizie fx risk
What is the precautions during PT for OI
- no passive twisting , rotating or forcefull ROM in the extremities or trunk due to high fracture risk
- pain
- weakness
- faitgue
- close guarding during WB activities due to decreased risk of fall or injury
- close monitoring of skin when wearing AFO
What are the complications interfering with PT for OI
- patient discomfort with AFO due to post surgical LE swelling
- pain may limit activity tolerance
- UE deformities may increased difficulty in using walker
What kind of disorder is OI and in most cases what is it caused by
Inherited disorder that in most cases is caused by autosomal dominant gene mutation that affects type 1 collagen production
What does type 1 collagen make up
Connective tissue in bone s, ligament , tendons .skins , dentin , corneas, and lung
What is the standard treatment of long bone deformity
Surgical placement of a rod thru the intramedullary cavity of the bone
The Wong-Baker Faces Pain Rating scale has been found to be reliable and valid in children from what ages
3-18 y/o
Should u initiate WB on land or in the pool with kids with OI and whjy
Pool bc the water lessens the impact on the joints and decreased stresses on long bones
T/F: Intramedullary rodding surgery decreases fracture recurrence in long bones and minimizes progression of bowing deformity in children with osteogenesis imperfecta
True
T/F: Bisphosphonates increased fracture rate in children with OI.
F
Decreased
What is the most appropriate progression from ambulation with a walker to ambulation without an AD for a child with OI (question 1)
Having the child practice taking a few steps at a time with light hand held asssit in controlled environment
A physical therapist is creating a home exercise program for a child with OI who has just begun weightbearing on land following a rodding revision surgery. What is not an appropriate exercise to strengthen the quadriceps for this child?
Single leg squats holding a walker for support
When would it be appropriate to utilize a partial-weightbearing gait therapy device (e.g., LiteGait) as a therapy intervention for a child with OI who has recently begun weightbearing after a rodding surgery?
Never bc a child with OI shoudl never be placed in a partial weight bearing gait therapy device bc it could fx their bones
