Case 11: Ducheene Muscular Dystrophy Flashcards
What is an Enzyme that catalyzes the conversion of adenosine diphosphate (ADP) and phosphocreatine into adenosine triphosphate (ATP) and creatine; expressed in many tissues, but in higher concentrations in the brain, striated muscle, and other tissues that rapidly regenerate ATP
Creatine kinase
What is gower’s sign for DMD
When an individual rises from the floor using a 4-point stance placing hands on the knees and then hyperextending the knees while pushing on the thighs to compensate for hip extension weakness
What is hypercapnia
Excess carbon dioxide in the blood
What is PSEUDOHYPERTROPHY
Increase in the size of a mm that is not due t an increase in the size of the persons mm fibers
In DMD what is mms replaced with
Fibrous tissue and fat
What is an Enzyme that catalyzes the transfer of an amino group from one molecular group to another, which is an important process in forming amino acids in the metabolism of proteins; elevated ____ levels in the blood may indicate ____ dysfunction
TRANSAMINASE
liver
What are the general PT POC/goals for DMD
- maximize safe participation and function at home
- optimize positioning throughout the day and night to minimize contractures
- improve LE strength , ROM and endurance
- maintain respiraotry status
- progress stnading tolerance and ambulation with and without AD
What are the PT interventions from DMD
- practice negotiating WC through tight spaces , over ramps , into and out of home , van and bud
- practice safe and effective transfers
- weight shifting in WC and sitting balance at edge of bed
What are the precautions during PT for DMD
- no resistance or forceful ROM to the extermtieis or trunk due to high fx risk and damage to mm
- pain , weakness, faitgue
- asssitance with WB activities required during transfers
- close monitor of skin due to orthoses
What is the complications interfering with PT for a kids with DMD
- patient discomfort with AFOs and prolonged positioning
- patient and parent anxiety regarding potential fx
What is DMD caused by
Genetic mutation that encodes for a mm protein called dystrophin
These people lack it
What is the benefit of starting glucocorticoids earlier for DMD
More sustained neuromuscular function and prolongs ambulation and QOL
What is an outcome tool that measures the functional ability for non ambulatory people with DMD to perform daily activities
Egen Klassification Scale (EK scale)
T/F: The use of systemic glucocorticoids prolongs ambulation and quality of life in individuals with Duchenne muscular dystrophy.
True
T/F: Scores on the Egen Klassification Scale can predict the need for ventilatory support and ongoing loss of physical function in the DMD population.
True
What does the scores on the Egen Klassificaiotn scale predict
The need from ventilatory support and ongoing loss of physcial function in the DMD population
What does asssitive bicycle exercises of the legs and arms do for people with DMD
Delays the progressive weakness and functional loss characteristic of DMD
What sign is the hallmark of DMD
Gowers
Which distinctive examination sign may be associated with the diagnosis of DMD?
A. Babinski’s
B. Thomas test
C. Gowers’
D. Homan’s
C
Which of the following is appropriate to assess function and participation in the DMD population?
A. Timed Up and Go
B. Range of motion
C. Functional Reach Test
D. Egen Klassification Scale
D
