Hand versus mouth for call-bell activation by DMD and Becker patients.

Severe hand dysfunction is common in patients with Duchenne muscular dystrophy (DMD) and may preclude the use of conventional call-bells. We prospectively evaluated a call-bell with two hand-controlled interfaces (push-button and key-pinch) and two mouth-controlled interfaces (sip-or-puff) in 32 consecutive DMD and Becker patients. Patients called intentionally 348 times, using the sip-or-puff device 237 times and the hand-controlled interfaces 147 times. Use of the hand-controlled interfaces correlated with key-pinch strength (R=0.366; P=0.04). Six patients reported being unable to call with the hand interfaces and five patients reported temporary call failure due to inaccessibility of the sip-or-puff interface. Ease-of-use scores on a visual analogue scale were best for puff, followed by sip then key-pinch interrupter and push-button (8.7+/-2.1, 7.5+/-2.7, 6.2+/-3.9, and 0.5+/-2.0 respectively; ANOVA: P<0.00001). In conclusion sip-or-puff devices should be considered more often to provide neuromuscular patients with greater independence.

Optimization of power wheelchair control for patients with severe Duchenne muscular dystrophy.

The extended survival of patients with Duchenne muscular dystrophy (DMD) achieved by the introduction of mechanical ventilation is raising new quality-of-life issues. We evaluated passive range of wrist extension, key pinch strength, and power wheelchair driving in 84 patients. The 47 drivers with restricted driving abilities were older than the 37 unrestricted drivers (27.2+/-5.0 y vs. 22.8+/-3.8 y) and had worse upper limb performance. By multiple logistic regression, only key pinch was significant ( R(2) = 0.224, P = 0.02. Eighteen restricted drivers were reassessed after having swapped a conventional joystick for another control system (mini-joystick, isometric mini-joystick, finger joystick, or pad) and having moved the position of the tested control system so that the patient could use it with different fingers, his chin or his mouth. All of them regained the ability to drive unrestricted. Adults with DMD gradually lose their ability to drive with a conventional joystick but can regain unrestricted driving with alternative control systems.