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Table 6 Impact of Late Onset GM2

From: A feasibility study of mHealth and wearable technology in late onset GM2 gangliosidosis (Tay-Sachs and Sandhoff Disease)

Impact of Late Onset GM2
Question Possible Answer Numerical Value Numerical Value Converted (For composite scale)
This section relates to your ability to walk about from place to place. Please select the option that best describes how you are generally. I have no problems walking about 0 0
I am clumsy 1 0.2
I am unsteady on my feet 2 0.4
I can walk but only with assistance, particularly outdoors 3 0.6
I normally use a wheelchair outdoors. Inside/at home I move about on my feet but need assistance 4 0.8
I cannot walk at all and to get around I always use a wheelchair 5 1
This section relates to your ability to coordinate your movements. Please select the option that best describes how you are generally. I have no problems coordinating movement 0 0
My movements can be shaky or trembling 1 0.25
I have some problems with coordinating movement 2 0.5
I have problems with coordinating movement, but can still feed myself 3 0.75
I cannot perform any activities independently and need help to do everything 4 1
This section relates to your speech. Please select the option that best describes how you are generally. I have no problems with speech 0 0
My speech can be difficult to understand 1 0.333
My speech is very difficult to understand and really only people who know me very well can understand what I am saying 2 0.667
I cannot speak, but can communicate in other ways 3 1
This section relates to your ability to swallow. Please select the option that best describes how you are generally. I have no difficulty in swallowing 0 0
I chew abnormally 1 0.25
I sometimes have difficulty in swallowing, and may occasionally cough while eating or drinking 2 0.5
I have difficulty in swallowing every day, frequently coughing or choking on food or drinks 3 0.75
My difficulties in swallowing are so severe that I now must be fed by nasogastric tube or gastrostomy. 4 1
Do you think your cognitive abilities are impacted by late onset GM2 gangliosidosis? i.e. learning new skills, making decisions, following instructions, focusing your attention Not at all 0 0
A little bit 1 0.333
Quite a bit 2 0.667
Very much 3 1
Total Score 19