Lecture 14: Osteogenesis Imperfecta Flashcards
Osteogenesis Imperfecta
* How is it aquierd
* Whats messed up in this disease
Inherited disorder
Connective tissue apthology
also called Fragilitas Ossium or Brittle Bone disease
What happens to the joints w/ osteogensis imperfecta
* what happens to muscles
* What bone disease do they have
* whats the hallmark of this deformity?
*
Lax joints
Weak muscles
Diffuse OSteoporosis
Multiple Recurrent Fractures = halmark
Deformity-Ranges from person to person
Sulcus sign used to be how they tested but this is bad because it can lead to broken bones
What happens to the yes w/ osteogenesis imperfecta?
Blue Sclera
Note they also have:
* Dentinogenesis Imperfecta - teeth issues
* Deafness
* Hernia
* east bruising
* Excessive sweating
What type of osteogenesis imperfecta does 50% of the population have?
type 1
generally short
only 10% have fx at birth
blue eyes
Whats the big thing to know about type 2 osteogenesis imperfecta?
Not compadible w/ life
bones are so fragile that skull litteraly crumbles
What is type 4 osteogenesis imperfecta like?
Very mild
KNOW: in type 5 osteogenesis imperfecta theres lots of bone where theres not supposed to be bone
Whats the big thing to know about type 8 osteogenesis imperfecta
Flat bones - so weird bone shape messes up all the attachments for the muscles
Whats the big thing to know about type 10 osteogenesis imperfecta
extremely fragile
What vitamind would we want to give someone w/ this disorder?
Increase the amount of vitamin D
MEdical maangement:
* No cure
* Whole body Vibration
* Bone marrow transplant and stem cell therapy - used sparingly - risky because immunse system is comprimised, but when it works it works great
* Bisphosphonates = reduced fx / increased bone density
Fractures:
* If a bone fractures is it more or less suseptible to refracture?
* What happens to healing time?
* when does fracture rate slow
* What do they get put in for increased stability
More suseptible (unlike normal people)
* resultatnt callus large and poor quality
Normal healing time
Decreased fractures following puberty
Sometimes get intermedually rods for increased stabilization, however, they’re more at risk for fx above and below rods
PT Evaulation for OSteogenesis Imperfecta
* how do we test ROM?
* Do we MMT?
ONLY do active ROM becuse they won’t break their own bones
Pain testing
No resistive MMT
* Do more functional things (think sit to stand etc…)
Function
Development
Gait
Posture
Cognitive status - shouldnt really be impacted
Hearing - often have conductive hearing issues
Vision - remember blue eyes
Equipment
Goals
Medical status
Medications
Current and prior therapies
Interventiosn for someone w/ osteogenesis imperfecta:
* Strengthening-Non resistive - think functional activities / isometrics - contact physician and ask what they should do
* child and caregiver education
* Orthotic assessment
* Gait training
* Positioning
* Adaptive equipment
* Assistive devices / mobility devices
* Splinting/Bracing
* aquatic therapy - this is good because its decreased wt bearing. However, if were trying to build bone density this isnt our best choice
* No passive ROM/Stretching!!!
Maximize Function
Decrease Fx risk
“Keep it simple stupid”
