Case 18: Osteogenesis Imperfecta Flashcards
Bisphosphonates
Help prevent loss of bone mass by inhibiting osteoclast activity
Decreases bone resorption
Increase BMD
Bone Mineral Density (BMD)
Amount of Ca and other mineral matter per unit of skeletal area
Typically used to assess fracture risk and bone health
Clamshell AFO
Worn on lower leg and foot
Provides circumferential support with anterior and posterior shell components
DEXA / DXA Scan
Measures BMD
Intramedullary Rod
Surgically inserted into medullary canal of a long bone to add stability
Solid piece (intramedullary nail)
OR
Telescoping rod (smaller rod inside larger hollow rod - elongates as bone grows)
Osteotomy
Surgical fracture of a bone
Typically done to realign a deformed or rotated bone
Precautions During PT (Patients with OI)
No passive twisting / rotating / pulling on body part / forceful ROM in extremities or trunk due to high fracture risk
Precautions in Handling Infants w/ OI
Carry with widespread hands over head / shoulders and bottom
Diaper changes done by lifting buttocks, NOT ankles
Complications During PT (Patients with OI)
Patient discomfort with AFO due to postsurgical LE swelling
Pain may limit activity tolerance
Pt and parent anxiety regarding potential fractures
UE deformities may increase difficulty in using a walker
OI affects what type of collagen?
Type I
Type I OI vs. Type II / III / IV
Type I OI allows production of normal collagen but a deficiency in the amount produced
Types II / III / IV have altered collagen formation that creates a deficient bone matrix
Dominant vs. Recessive OI
Dominant: Person had too little / poor quality Type I Collagen due to mutation in a Type I Collagen gene (majority of cases)
Recessive: Mutations in other genes interfere with collagen production
Characteristics of OI
Low BMD that results in fragile bones that fracture easily with little or no trauma
Bone deformities
Hyperlaxity of ligaments
Fragile skin
Poor thermoregulation
Blue Sclerae
Dentinogenesis Imperfecta (issues with teeth)
Does OI affect the rate of bone healing?
No!
However, healing may be affected by amount of pre-existing bowing in long bones
Which type of OI is typically fatal in the neonatal period?
Type II OI
A child may be immobilized for how long following installation of an intramedullary rod?
6-8 weeks to allow sufficient healing
What is the gold standard for measuring BMD in adults?
DEXA
Prior to an Osteotomy, children taking Bisphosphonates may be instructed to discontinue usage for how long? Why?
Discontinue use 3-4 months prior
Medications may delay surgical osteotomy healing
Whenever possible, WB should be initiated ___ in children with OI.
in the pool
Decrease fracture risk and allows child to experience success with low-impact activities
Buoyancy of water lessens impact on joints and decreases stresses on long bones
Wong-Baker Faces Pain Rating Scale
Reliable and valid in children 3-18 years old
What should be avoided related to MMT in children with OI?
Avoid applying resistance with MMT to decrease risk of fracture
MMT only if able to tolerate and perform at mid-shaft of bone
What movement at the trunk should be avoided in patients with OI?
Isolated trunk flexion / extension to assess core strength
Assessing Knee Extension Strength in Children w/ OI
Prompt child to complete a SLR while therapist lightly supports limb
Observe how well the child can maintain knee extension while lifting the leg
OR supported LAQ
Assessing Hip Abduction Strength in Children w/ OI
Supine or sidelying with back supported against the wall
Therapist lightly supports limb during movement
