What is Duchenne Muscular Dystrophy?
An X linked rare, life-threatening neuromuscular disease characterized by rapidly progressing muscle weakness and wasting.
FOUR PHASES
Early Phases
(6 Years)
Clumsy, fall frequently, difficulty jumping or running, enlarged muscles, contractures.
Transitional Phase
(Ages 6-9):
Trunk weakness (Gowers manouvre), muscles weakness, heart problems, fatigue.
Loss of ambulation
(Ages 10-14):
By 12 years most boys use a powered wheelchair. Scoliosis due to constant sitting and back weakness, UL weakness make ADL’s difficult (retain use of fingers).
Late stage
(15+ years):
Life threatening heart and respiratory problems more prevalent, dyspnea, oedema of the LL’s. Average age of death is 19 years in untreated DMD but due to improvements in clinical care in many centers the average age of death is the late twenties or beyond.