A qualitative evaluation of the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R) by the patient community: a web-based cross-sectional survey

Figures & data

Table 1 Participant characteristics (N = 57).

	M (SD) or n (%)
Age, M (SD)	52.4 (10.5)
Most recent ALSFRS-R score, M (SD)	33.1 (13.9)
Participant type
Person with ALS	41 (71.9%)
Caregiver	16 (28.1%)
Gender
Female	33 (57.9%)
Male	24 (42.1%)
Race/ethnicity
White or Caucasian	55 (96.5%)
Hispanic or Latino	1 (1.8%)
Other	1 (1.8%)
Marital status
Married	45 (78.9%)
Not married	12 (21.1%)
Country
United States	45 (78.9%)
Germany	2 (3.5%)
United Kingdom	2 (3.5%)
Sweden	1 (1.8%)
Canada	1 (1.8%)
the Netherlands	1 (1.8%)
Australia	1 (1.8%)
Belize	1 (1.8%)
Holland	1 (1.8%)
Israel	1 (1.8%)
Portugal	1 (1.8%)

Figure 1 Percent of critiques by item in response to the question, ‘Can you think of a situation where you might not be able to answer this item accurately or that your answer might not reflect your abilities?’ Percentage is out of the total number of participants who responded to any question in this section (n = 57).

Table 2 Contextual themes by item based on qualitative responses.

Theme	Item	Exemplary quote
The question is answered differently depending on the situation or equipment used	Speech (Item 1)	‘Time of day. Better in morning less intelligible in evening.’
	Walking (Item 8)	‘Needs to allow for more options. Stairs? Uphill v downhill? Just getting up v walking for a bit?’ ‘In water, I can 'cycle’, bear some weight and move my legs freely. Out of water, I can only wiggle my toes. What # would that be: 4 or 5?’
	Cutting food/handling utensils (Item 5)	‘I am able to feed myself finger foods. Sandwiches, pizza slices of fruit, etc. but unable to use utensils.’ ‘Yes. Depends on specific food and utensil. My wife and use plastic utensils with foam tubing because they are light and easier to hold. How is she supposed to answer this? The score will depend on who is asking her and how they map her answer to one of these choices.’
	Dyspnea (Item 10)	‘Yes. I don’t get winded walking,  but I do with doing ADLs.’
	Orthopnea (Item 11)	‘What if I sleep just fine laying down on my side with 0 pillows but can’t sleep in a sitting position. They categories can seem arbitrary at times.’
It is difficult to distinguish the difference between choices on the scale	Turning in bed and adjusting bedclothes (Item 7)	‘yes The difference between the second and third choice is very unclear. Is the third choice implying that help is needed? What exactly is "can initiate" mean?’ ‘Yea. This is a weird question. There’s a lot in between the options.’
	Dyspnea (Item 10)	‘I can’t imagine how a responder picks between the last two items. Is the last option only for when mechanically ventilated and under what conditions − 24 hours a day? just at night?’
	Climbing stairs (Item 9)	‘Too much happening between mild difficulty and needs assistance.’
	Walking (Item 8)	‘There is a lot of room between early difficulties and walk with assistance. My son walks, with difficulty it doesn’t need assistance. Surfaces are the issues.’
	Dressing (Item 6)	‘answers 3 and 4 can get blurred.’ ‘I have a great deal of difficulty deciding between #2 and #3, they are not mutually exclusive. If my spouse is not around, I can do everything by myself but she helps if she is there to conserve my energy!’
The structure and/or underlying assumptions of the question makes it difficult to answer	Handwriting (Item 4)	‘legibility of handwriting may have nothing to do with ALS - the item needs to clarify that this is a CHANGE in handwriting that goes from legible in illegible. Someones handwriting may be illegible to begin with regardless of ALS.’ ‘…how does one approach answering the options considering handedness and bilateral differences in the levels of weakness or deficit. How is one supported to answer? Based on handedness? Or based on weakest hand?’ ‘Is there a distinction for handedness. I am left handed. What is my left hand is far more effected than my right hand. Then imagine the reverse. Does that mean the same person with a deficit that is greater in the dominant hand potentially score two different numbers? My right hand isn’t impacted but my handwriting will be very bad. If I had total loss of use of left hand and was forced to use non-dominant hand - how do I score?’
	Swallowing (Item 3)	‘Yes - answer b describes two different problems “early eating problems” which is very vague - too vague. What is an early problem versus a late problem? Clearly dieatary consistency may change before occasional choking events making it hard to respond to this question.’
	Turning in bed and adjusting bedclothes (Item 7)	‘Turning and adjusting sheets are two entirely different activities. Because my legs are compromised, I have to move them with my hands. Adjusting sheets, on the other hand is not compromised in any way.’
	Orthopnea (Item 11)	‘Some people (my wife included) always used multiple pillows and has had trouble sleeping unrelated to ALS.’
	Respiratory (Item 12)	‘I might have Respiratory Insufficiency but have adapted by limiting my activity or changing my position and not need any additional intervention but I still have the deficit.’
	Salivation (Item 2)	‘Ask about dry mouth as well as salivation.’
Language used in the question is of a medical literacy level too high for most non-clinicians	Dyspnea (Item 10)	‘…stop using medical terms in the scale - need to use lay terms given the frequent self report nature of this use of this scale.’
	Swallowing (Item 3)	‘Don’t know what the last option (NPO) means’
Language used is of appropriate literacy level but needs clarity	Turning in bed and adjusting be dclothes (Item 7)	‘I really struggle to understand when the threshold is met to answer “b”. Yes, it’s harder to turn in bed, but is clumsy? When does it become “clumsy” it’s hard to interpret when to start selecting answer b’
	Stairs (Item 9)	‘What do you mean by assistance?’ ‘Yes. Needs assistance--is that the use of a handrail? This question should be revised.’ ‘Very unclear as to what level of assistance is required to answer "needs assistance" - does that include using the handrail? Not clear as to what circumstances need to be to answer the assistance aspect of the response options and there seems to be a large gap in functional decline to go from needs assistance to cannot do…that is - the response options definitely do not appear to be linear.’
	Orthopnea (Item 11)	‘Yes, I use a wedge, not sure if that counts as 2 pillows?’
	Respiratory (Item 12)	‘Yes, Bipap is not an accurate term.’
	Walking (Item 8)	‘"Early ambulation difficulties" is ambiguous. What does "early" mean and what does "assistance" mean? Does it mean I need a handrail on stairs or a walker? Do I put my hand on the wall to steady myself or hold onto furniture for security? Those aren’t absolutely necessary but they do show a loss of skills in balance and ability and would they be considered "assistance"?’ ‘Needs to allow for more options. Stairs? Uphill v downhill? Just getting up v walking for a bit?’ ‘What’s the different between early ambulatory difficulties and using a cane or walker? What is the patient refuses to use assistance, but really should have assistance? How do you answer that situation. Based on what the patient actually is doing, despite the majority of patients with the same level of deficits using assistance? This one is actually quite difficult to answer. I use assistance in many situations, but not all the time - so why do I answer?’ ‘This question is unclear and is vague. What exactly is meant by early ambulatory difficulties.’

Data availability statement

The subset of the data that support the findings of this study have been included in a Supplemental file.