DMD - L5

Question 1

Topics? (6)

Answer 1

genetic counselling
maintaining ambulation
prevention & treatment of contractures
anticipatory monitoring for DMD complications
medical therapy
adult issues / palliative care

Question 2

How to maintain ambulation? (4)

Answer 2

weight control, diet, exercise
prevent contractures
physiotherapy
corticosteroid therapy

Question 3

Why do contractures arise? (3)

Answer 3

static positioning in a position of flexion
muscle imbalance around joint
fibrotic changes in muscle tissue

Question 4

How to prevent/treat contractures? (4)

Answer 4

maintain ambulation
passive stretching, physio
night-time splints and braces
surgical release of contractures

Question 5

Describe the benefits and dangers of exercise.

Answer 5

-

Question 6

What are the complications for anticipatory monitoring? (4)

Answer 6

learning problems
scoliosis
respiratory muscle weakness
cardiomyopathy

Question 7

Scoliosis?

Answer 7

steroids (less risk, >risk for fractures)
spinal monitoring (Cobb angle >25)
spinal fusion (complications?)

Question 8

Respiratory monitoring?

Answer 8

annual measurement of pulmonary function tests
annual sleep study
check peak cough flow yearly

Question 9

Management of cardiomyopathy?

Answer 9

baseline assessment by 6 years
regular ECG and echocardiography in all boys
monitor for hypertension in boys on steroids
treat signs and symptoms of cardiac dysfunction

Question 10

Medical therapy?

Answer 10

steroids

nutrition/diet

Question 11

Adult issues / palliative care?

Answer 11

-