The Assessment of a Patient S EDSS Is Dominated by Their Ability to Walk Unaided Or Without

Appendix 1

The assessment begins with a disclaimer and introduction about the use of the calculator. It next asks patients whether they need assistance completing the assessment, indicating that it may be done with the help of a relative, or by a relative on behalf of the patient. The patient is asked to submit their disease type, relapsing, progressive or relapsing-progressive. If their disease is relapsing, they are asked to record the date of their last relapse, whether they made a full recovery and if their clinical condition has been stable over the past 4 weeks. In this introductory part of the assessment patients are also asked whether they feel that they are ‘disabled or affected in everyday life by their disease’, and, where applicable, whether they feel that their symptoms have changed since they last completed the survey

The assessment of a patient’s EDSS is dominated by their ability to walk unaided or without support. The first question of the second part of the web assessment (Question 12) raises this issue, and filters the patients further questions based on their response. If the patient requires assistance to walk, the next seven questions (Question 12-21) assess the level of assistance required. However if they can walk unaided they are directed to the assessment of the functional systems. If the patient’s disease is more severe as indicated by their responses, they are next asked about their need for a wheelchair, their ability to transfer and whether they are restricted to their bed by MS. (question 22-32) If the patients disease is at this level of severity they are then directed towards questions which assess their ability to articulate speech, to eat and swallow. (Question 33-35)

The assessment then reassesses the patient’s ability to walk with or without support, to ensure that the patient has understood the meaning of the questions answered previously.

The functional systems are next assessed. (Question 37-44) The ophthalmic, brainstem, pyramidal, and cerebellar systems are examined first. Responses are graded as ‘none, mild, moderate, severe, and very severe’ depending on how the symptoms of each affect the patient’s ability to function. Each response is accompanied with an explanation of the symptoms experienced in each category of severity. Cerebellar dysfunction is assessed with two questions relating to balance on walking and limb ataxia, as in the original telephone assessment. Sensation, bowel / bladder function and cognition are assessed with six levels of severity. Again each possible response is explained with relevant symptoms to ensure the patient understands which response is most applicable to them.

Disclaimer and Introduction

Although care has been taken in preparing the EDSS calculator and accompanying survey, Barts and The London School of Medicine and Dentistry, Queen Mary University of London, cannot be held responsible for any errors or omissions in the survey and accepts no liability whatsoever for any consequences arising from completing this survey. The University reserves the right to make changes to or take down this survey, or remove or alter any content at any time without notice. No third party will be given access to the data, nor the personal information, collected as part of this survey.

I understand that relevant sections of my medical notes and data collected during the study may be looked at by individuals from Queen Mary University of London, from regulatory authorities and from the NHS Trust, where it is relevant to my taking part in this research. I give permission for these individuals to have access to my records.

Professor Giovannoni welcomes comments and suggestions about this survey and the proposed study, which can be sent to him using the "additional comments" field at the end of this survey. Anyone wishing to contact him directly can do so using his contact details below.

Professor Gavin Giovannoni, Neuroscience & Trauma Centre, Blizard Institute of Cell and Molecular Science, 4 Newark Street, Whitechapel, LONDON E1 2AT

Tel: +44 20 7377 7472 / Fax: +44 20 7377 7033 / Email:

I have read and understand the disclaimer and wish to proceed with this survey.

Which of the following best describes the person or people who are filling in this questionnaire?

I am a person with MS and I am filling in this questionnaire on my own
I am a person with MS and I am filling in this questionnaire with some help from a carer, relative or friend
I am a carer and/or relative of a person with MS and am filling in this questionnaire on behalf of a person with MS.

1. Do you have relapsing or progressive MS?

Relapsing MS is characterised by definite episodes of worsening neurological functioning that typically lasts days to weeks, followed by a period of partial or full recovery and then a period of remission.

Progressive MS is characterised by a gradual deterioration in neurological functioning without neurological improvement; this can be either primary or secondary progressive MS.

Progressive-relapsing MS is characterised by a combination of the two; i.e. a gradually progressive course with superimposed relapses. If you have this form of MS you won't experience a stable baseline.

Intermittent symptoms that come and go over minutes to hours, possibly in relation to fatigue or heat, should not be considered a relapse. These symptoms typically occur in pathways that have been previously involved by a MS lesion and occur as a result of either a failure of conduction of a nerve impulse or abnormal excitement of a group of nerves.

Relapsing MS
Progressive MS
Relapsing-Progressive MS

When was your last relapse? (Both the month and year should be entered as a number). If you can't remember, please estimate month and year.

Did you make a full recovery from this relapse?

Has your clinical condition been stable over the last 4 weeks?

If you are having a relapse or recovering from a relapse and have not reached a plateau (at least 4 weeks) please answer "no". Intermittent symptoms that come and go over minutes to hours, possibly in relation to fatigue or heat, should not be considered when answering this question.

Do you have intermittent symptoms that come and go over minutes to hours, possibly in relation to fatigue or heat?

These symptoms typically occur in pathways that have been previously affected by MS and occur as a result of either a failure of conduction of a nerve impulse or abnormal excitement of a group of nerves. For example, intermittent blurring of vision, pins and needles, tingling or other odd sensations, muscle or limb jerking, transient double vision, etc.

Do you feel disabled or affected in everyday life, due to your disease?

No; not all
Yes; but only some of the time or very occasionally
Yes; I have some disability

When you doctor last assessed you did he/she inform you of any abnormal physical findings that could be due to your MS but may not be causing you any obvious symptoms?

These findings can be very subtle and you may not be aware of them, for example (1) paleness of the optic nerve disc at the back of the eye, (2) asymmetric or abnormal reflexes, (3) mild weakness, (4) clumsy fine movements, (5) inability to walk on a straight line or hop on either leg unsupported, (6) mild tremor, (7) abnormal sensory testing, etc.

Yes
No
Unsure

If you have completed this survey before, have you observed any change since then in relation to your MS symptoms?

Improved much
Improved a little
Remained unchanged
Got a little worse
Got much worse
I have not completed this survey before

Walking ability and aids

Are you able to walk without aid or support?

Do you use a foot splint?

Yes - both feet
Yes - one foot only
No

How far can you walk without any aid or rest?

Please refer to a known distances, for example post office, shop, church or park, as well as improvement or deterioration as compared with the previous assessment, for a better estimate of your walking distance.

Unrestricted (more than 8-10km or 5-7 miles; 2 - 3 hours)
Clearly more than 500 m
About 500m
About 300m
About 200m
About 100m
Less than 100m
Between 100 and 300m, but not sure

Are you able to do your normal daily work?

Yes, no limitation (but walking distance less than approximately 300m)
Unable to work a full day without special provisions
No, normal daily activities not possible

How long have you needed a walking aid, such as a stick or frame?

Less than 6 months
6 to 12 months
1 to 2 years
2 to 5 years
greater than 5 years

Are you able to walk with unilateral or bilateral assistance?

only unilateral assistance (i.e. only a single stick)
need bilateral assistance or help by other person
not able to walk more than a few steps with bilateral assistance

What kind of walking aid do you use?

Single stick
Two sticks
Crutches
Zimmer frame
Assistance of another person
Other please specify

How reliant are you on your walking aid?

It is not essential, but using it makes me feel more confident
I need it occasionally (<20% of the time)
I need it some of the time (20-50% of the time)
I need it most of the time (50-80% of the time)
I need it almost all of the time, but can do without it for short periods (>80% of the time)
I am unable to walk without it

How many meters can you walk without rest, using constant or intermittent unilateral assistance?

more than 50m
less than 50m
I am unable to walk with only unilateral assistance

How many meters can you walk without rest, using bilateral assistance?

more than 100m
more than 10m but less than 100m
less than about 5m

Do you need a wheelchair at certain times, for example when leaving the house?

For how long have you regularly needed a wheelchair?

Less than 6 months
6 to 12 months
1 to 2 years
2 to 5 years
Greater than 5 years

Can you wheel yourself in a standard wheelchair?

Can you transfer alone (e.g., from/to bed, toilet)?

If you need help with any transfers then please answer "No".

When you are awake and up (out of bed) are you in your wheelchair (or other chair) for less than 8 hours a day?

Can you transfer alone (e.g., from/to bed, toilet)?

If you need help with any transfers please answer "No".

When you are awake and up (out of bed) are you in your wheelchair (or other chair) for less than 8 hours a day?

Are you restricted to bed for part of the day?

No
In bed for less than 3-4 hours during the day
In bed more than 4 hours during the day

Can you use your arms for (1) eating, (2) washing your face and (3) brushing your teeth?

Yes, all three of these tasks
Yes, two of these tasks
Yes, one of these tasks
No, none of these tasks

Are you restricted to your bed due to your MS?

Are people able to understand you when you speak (problems may be due to speech problems such as articulation or language problems)?

Are you able to eat and swallow?

Yes
No, fed by feeding tube

Functional Systems Assessment

Are you able to walk more than 10m with bilateral support?

Yes, I can walk more than 10m with bilateral support.
No, I need a wheelchair or similar device to cover distances greater than 10m.

Do you have visual problems with one or both eyes (not including double vision)?

This could be a very minor problem; for example when looking at colours with either eye they appear "washed out".

1 = none; I have no noticeable problems with vision

2 = mild; I am aware of problems with vision but it does not interfere with my visual function

3 = moderate; it interferes with my visual function to some extent

4 = severe; it interferes greatly with my visual function

Do you have double vision or slurred speech or partial loss of sensation in the face or facial weakness or swallowing problems?

1 = none; I have no noticeable problems

2 = mild; I am aware of problems in these areas but they do not interfere with my functioning

3 = moderate; I have problems in these areas that interfere with my functioning to some extent

4 = severe; problems in these areas interfere greatly with my function

Do you have weakness of both legs or weakness of one half of the body or weakness of only one arm or leg?

1 = I have no noticeable weakness

2 = mild weakness; I am aware of weakness but it does not interfere with my limb function

3 = moderate weakness; it interferes with my limb function to some extent

4 = severe weakness; it interferes greatly with limb function

Do you have any tremor or clumsy movements?

1 = none; I have no noticeable tremor or clumsy movements

2 = mild; tremor or clumsy movements do not interfere with my handwriting, closing buttons or cutting food during meals

3 = moderate; tremor or clumsy movements interfere with my handwriting, closing buttons or cutting food during meals

4 = severe; most functions are very difficult for me to do because of tremor or clumsy movements

5 = very severe; no coordinated movements are possible

Do you have any problems with your balance when walking or sitting?

1 = none; I have no noticeable problems with walking on heels or toes, or walking on a straight line

2 = mild; I lose balance when I walk on my heels or toes, or walk on a straight line

3 = moderate; I lose balance with normal walking or when sitting

4 = severe; I am unable to walk, or I require support by another person or assisting device because of unsteadiness

5 = very severe; I am unable to sit or walk even with assistance

When touching your body, is the sensation normal?

1 = I have normal sensation

2 = I have mild numbness when touching 1 or 2 limbs

3 = I have a clear reduction in sensation in 1 or 2 limbs or numbness in many parts of my body below the head

4 = even with firm pressure I can not feel at all in 1 or 2 limbs or there is a clear reduction in sensation in more than 2 limbs

5 = sensation is essentially lost in 1 or 2 limbs or there is a moderate decrease of sensation for most of my body below the head

6 = sensation is essentially lost below my head

Do you have any problems with passing urine or stool? Please select the answer that is most appropriate for you.

Some definitions:

1. Urinary hesitancy is the term used to describe difficulty initiating urination.

2. Urinary retention means the inability to initiate and empty your bladder.

3. Urgency means you have to get to a toilet quickly to prevent episodes of incontinence.

4. Incontinence means lack of control of passing urine or stool.

1 = I have no problems passing either urine or stool

2 = I have moderate hesitancy, urgency or retention; or rare urinary incontinence; or severe constipation

3 = I have frequent urinary incontinence, but spontaneous urination is generally possible; and/or I need to use enema in order to pass stool

4 = I need almost constant catheterisation

5 = I have lost of bladder function and need permanent catheterisation

6 = I have lost both bowel and bladder function

Do you have any concentration or memory problems?

1 = I have no problems with concentration, memory or mood

2 = I have no problems with concentration or memory, but I do have problems with my mood (such as depression)

3 = I have concentration and memory problems, or decreased ambition, or problems with coping with stress, but I am able to handle my daily routines including completing this survey

4 = I have definite problems with concentration and memory that make completing this survey difficult

5 = I have marked decrease in mentation and need help completing this survey

6 = I have no meaningful conversation due to confusion and/or disorientation and need someone else to complete this survey

Patient Prior Knowledge and Satisfaction

Before being invited and agreeing to do this survey did you know what the EDSS was?

Yes
No
Unsure

Did you need help to complete the questions of the EDSS calculator?

No
Yes, for some of the questions
Yes, for all of the questions
Other, please specify

Do you think there is a need for a standalone web-based EDSS calculator?

Yes
No
Unsure

How did you find the instructions for the EDSS calculator as set out in this survey?

Very easy to follow
Easy to follow
Difficult to follow
Very difficult to follow
Other (please specify)

What applies to you in relation to the time it took you to complete the EDSS calculator?

Short
Just right
Long
Other (please specify)

If available, would you personally use a web-based EDSS calculator?

Yes
No
Unsure

Do you think there is a need for an interactive web-site that would help you monitor the course of your MS?

Yes
No
Unsure

Would you use an interactive website to monitor your disease?

Yes
No
Unsure

Do you have any suggestions how you would improve the EDSS calculator experience and for our proposed interactive website to help people with MS monitor their disease (optional)?