Rare disease day 2023 is here, and I am very pleased to publish the results of my 2022 survey.
This post is an abbreviated version of the full results. If you wish to read the full version of the results please follow this link: https://drive.google.com/file/d/1eWWNwPDR6IcCHT6a6aXHRCoy3Trw1zZe/view
In the post below table and section numbers are not sequential. There are other tables of data in the full version of the results, and this approach avoids confusion between people referring to results from this post against the full results just by table number. Blogger is not very good at transposing the formatting and alignment from Word here. Please use the link above for a nicely presented version!
2022 Survey Results. An
on-line Survey for People with Hereditary Spastic Paraplegia (HSP) linking
Wellbeing with Mobility, Pain, Menstrual Health and life with HSP.
This
post presents the results of my tenth survey, launched in September 2022. Help from
other HSP groups around the world was invaluable in translating the questions
to these other languages, and Google Translate is used to translate answers to
English for analysis.
There were 513
respondents who completed the survey, predominantly from the USA, UK, Brasil,
and France. Table 1 shows the distribution of respondents’ locations;
Table 1 - Location of respondents
Location
|
|
Respondents
|
Percentage
|
USA
|
(USA)
|
134
|
26%
|
UK
|
(GBR)
|
80
|
16%
|
Brasil
|
(BRA)
|
56
|
11%
|
France
|
(FRA)
|
53
|
10%
|
Netherlands
|
(NLD)
|
43
|
8%
|
Spain
|
(ESP)
|
38
|
7%
|
Germany
|
(DEU)
|
26
|
5%
|
Australia
|
(AUS)
|
17
|
3%
|
Canada
|
(CAN)
|
17
|
3%
|
Italy
|
(ITA)
|
14
|
3%
|
Other Europe
|
-
|
29
|
6%
|
Rest of world
|
-
|
6
|
1%
|
The number of responses
to this survey is similar to the last two years. In terms of distribution
changes, this survey has fewer responses from Germany and Austria, and more
responses from Spain. The ‘Other Europe’ category includes answers from
Belgium, Austria, Sweden and Switzerland. With this survey the analysis separates
out some results by country to explore country-to-country differences.
A brief analysis shows
that 234 people who completed this survey had also completed at least one of my
previous surveys, representing about 35%
of respondents. There
are 19 people who have completed seven or more of my ten surveys and 64
people who have completed previous surveys across five or more years.
HSP Analysis
The first questions in
the questionnaire asked if people had HSP. 504 respondents answered this
question, selecting from the following options:
Table 2 – HSP Diagnosis
HSP Diagnosis
|
Respondents
|
Percentage
|
I have HSP - genetic test
|
329
|
65%
|
I have HSP - other diagnosis
|
129
|
26%
|
I do not know if I have HSP
|
11
|
2%
|
I am answering on behalf of someone
else with HSP
|
35
|
7%
|
Around
65% have had a genetic test. The proportion having a genetic test is lowest in
Brasil (51%), and highest in Netherlands (79%) and Canada (76%). There are 158
people who reported a genetic diagnosis who had also completed a previous
survey. 103 of these have always reported having a genetic test, and 55 have
previously reported an other diagnosis, indicating that they have had a genetic
diagnosis since they have started completing these surveys.
Wellbeing
In order to assess the
wellbeing of people with HSP, respondents completed the Warwick-Edinburgh
Mental Well-being scale (WEMWBS). The scale is scored by summing the response
to each item answered on a 1 to 5 Likert scale. The minimum scale score is 14
and the maximum is 70. The
total score is higher for those with a better wellbeing. The average score across
England and Scotland populations is 51-52 with most scoring between 41 and 59.
There were 486
respondents who answered this question, with the average wellbeing score of 45.6,
which is broadly the same as the average scores of previous years. The scores
ranged between 15 and 70. The average is lower than UK population norms, however
it is still within the “average” definition and is also similar to those with a
self-perceived health status of “poor”.
The number of
respondents in this survey means that changes in score can be regarded as
significant in the ranges between +/-2 points and +/-5 points. Dealing with
sub-sets of the overall data, if the average is within +/-3 points of the
overall average then the effect is not significant. If an average is more than
5 points from the overall average, this is likely to be significant,
particularly for more than 50 respondents. Therefore, if there are more than 5
points between averages of different groups, then the effect may also be
significant.
Wellbeing scores have
not been evaluated for small groups of respondents (less than 10). Wellbeing
scores for groups of less than 20 people are shown throughout this document in
brackets, and these cannot be reliably assessed in respect of the significance.
The overall translation of significance is shown in Table 6, also shown with the
difference in points from the average:
Table 6 – Assessing WEMWBS Scores
|
Significantly worse
|
Worse, not significantly
|
Average score
|
Better, not significantly
|
Significantly better
|
Score
|
<40.6
|
42.6
|
45.6
|
48.6
|
>50.6
|
Diff.
from average
|
<-5
|
-3
|
0
|
+3
|
>+5
|
To aid the reviewing
of wellbeing scores throughout this report those scores that could be
significantly different from the average have been shaded yellow or light
green, and those that are likely to be significantly different from the average
are shaded orange and green.
Mobility Analysis
All 513 respondents gave
answers to this question and were able to choose as many of the following 13
options as they wanted:
- HSP does not affect my mobility
- I walk without aids, but there are
some effects
- I use walking sticks/poles/crutches
some of the time
- I use walking sticks/poles/crutches
most of the time
- I use walking sticks/poles/crutches
all of the time
- I use FES
(Functional Electrical Stimulation)
- I use orthotics/AFO (Ankle-Foot
Orthosis)
- I use a rollator/walking frame some
of the time:
- I use a rollator/walking frame most
of the time:
- I use a rollator/walking frame all
of the time:
- I use a wheelchair/mobility scooter
some of the time:
- I use a wheelchair/mobility scooter
most of the time:
- I use a wheelchair/mobility scooter
all of the time:
Types of mobility aid used
More than half of
respondents use walking sticks/poles/crutches/ canes, nearly half use wheelchairs/mobility
scoters and nearly a third use walking frames/rollators. These results are similar
to previous years. Table 9 shows these results, including the percentage of
respondents. These add to more than 100% as some people use more than one type
of mobility aid.
Table 9 – Use of mobility aids
Mobility
Aids Used - Summary:
|
Respondents
|
Percentage
|
Using sticks/poles/crutches/canes
|
282
|
55%
|
Using Wheelchair/Mobility Scooter
|
229
|
45%
|
Using Walking Frame/Rollator
|
157
|
31%
|
Using Orthotics/AFO/Insoles
|
152
|
30%
|
Using FES
|
19
|
4%
|
The results also allow
the distribution of respondents within a scale of mobility to be understood. I
have devised an “HSP mobility score” which then allows me to cross-reference
mobility against the other questions in the questionnaire. The definition of
the HSP mobility score is;
- No mobility effects
- Walk without aids,
but some mobility effects
- Orthotics/AFO/FES
and/or Sticks/Poles/Crutches/Canes some of the time
- Sticks/Poles/Crutches/Canes
and Frame/Chair some of the time
- Sticks/Poles/Crutches/Canes
most of the time
- Sticks/Poles/Crutches/Canes
all of the time
- Rollator/Walking
frame most of the time
- Rollator/Walking
frame all of the time
- Wheelchair/Mobility
scooter most of the time
- Wheelchair/Mobility
scooter all of the time
In the remainder of
this paper, whenever “sticks” are referred to as a mobility aid, this term
includes poles, crutches, and canes. Whenever “frames” are referred to this
includes both walking frames and rollators. Whenever “chairs” are referred to
this includes both wheelchairs and mobility scooters. Whenever AFO is referred
to it includes orthotics, AFO and insoles. The results are simplified into five broader groups;
Table
10
– Overview of mobility aids used
Mobility
Aids Used - Overview:
|
Respondents
|
Percentage
|
Mobility Score
|
Wellbeing
|
Those without aids
|
70
|
14%
|
0-1
|
51.0
|
Those who use mobility
aids some of the time
|
95
|
19%
|
2-3
|
45.5
|
Those who use sticks
most/all of the time
|
186
|
36%
|
4-5
|
45.0
|
Those who use frames most/all
of the time
|
64
|
12%
|
6-7
|
42.9
|
Those who use chairs
most/all of the time
|
98
|
19%
|
8-9
|
44.8
|
This
shows that there is a wide range of mobility aids used by the respondents to
the questionnaire, covering all mobility scores. Overall, the results are
similar to previous years although this year has a slightly higher number of
people who use sticks all or most of the time.
Favourite mobility aids
There
were 386 people who gave an answer for their favourite mobility aid. There were
11 answers given by at least 5 people, as shown:
Table 11 – Favourite mobility aids
Mobility
aids
|
Respondents
|
Walking sticks/canes
|
105
|
Walking frame/rollator
|
78
|
Wheelchair
|
59
|
Mobility scooter
|
35
|
Crutches
|
25
|
Electric wheelchair
|
24
|
Walking poles
|
18
|
Orthotics/AFO
|
16
|
Bikes (recumbent, tricycle, electric,
balance)
|
6
|
FES
|
5
|
Insoles
|
5
|
A few
gave the answer of a shopping trolley, including one person who had bought
their own. A couple of people identified an Alinker, and a couple said specific
shoes. Of answers given by one person only, one noted a handle to put in their
car door mechanism to help them out of their car, and one noted a concession
card giving them reduced prices.
Several
people also identified why the aid they specified was their favourite. Common
reasons included:
increasing their confidence and/or independencereducing their pain or fatigue,helping with their balance or reducing their fallsbeing able to get out and enjoy taking part in activities,increasing the distance they can travel or their ease of travel
One
person noted that their aids helped other to know they had mobility problems,
and several who said frames/rollators noted that they found the seat useful.
These reasons demonstrate that people do get benefits from their aids, and that
the aids can help some peoples symptoms.
Thoughts on using mobility aids
Respondents
described how they felt about using mobility aids, with 479 people giving an
answer. There were several common themes, as shown here:
Table 12 – How do you feel about using mobility
aids?
Feel
about using mobility aids?
|
Respondents
|
Wellbeing
|
Positive comments
|
64
|
46.7
|
Negative comments
|
73
|
38.7
|
Mixed comments (some positive aspects,
some negative aspects)
|
102
|
44.2
|
Neutral comments (comments without
obvious positives or negatives)
|
127
|
47.1
|
This
table shows that people who express negative comments about using mobility aids
have a lower wellbeing than other people, which is significant. Some example
comments are shown below.
When
looking at future changes in mobility aids there is a similar spread of views,
with some being positive and some being negative. Several people are scared,
apprehensive, worried or uncertain about future changes. Some express hope that
their HSP doesn’t progress such that they will need to change mobility aids.
Exercise and stretching is mentioned by several as techniques to slow down
progression. Others do not know how their HSP is likely to progress and give
the impression that they don’t know what types of mobility aid they might need
in the future.
Some
note that they are already using power chairs, and don’t expect to need to
change from this in the future. Quite a few don’t really want their HSP to
progress such that they need a wheelchair. Others note that they might change
the style of their chairs or type of chair that they have as their needs
change. There are similar ranges of view from people about using/changing sticks
and walking frames.
Quite
a few people are open to using whatever mobility aids give them the most
independence or flexibility, are happy to consider trying new things, and keep
themselves safe. Several note other
things to help their mobility like changes to vehicles, or like to think that
there will be technology improvements in the future.
The
table below shows a range of the comments made on feelings about using mobility
aids. These comments are in the respondents’ own words (or Googles translation
of them into English), with occasional minor adjustments for clarity or
anonymity:
Feelings
about using mobility aids
|
I dont want to, but i have no choice so i will use them
|
I feel good now because they help me, but at first I was
embarrassed
|
I need them, but my pride keeps me from using them.
|
Happy to have anything that keeps me independent and able to get
around
|
It is a necessary evil
|
They are a lifeline. I
now love my wheelchair - it’s my freedom machine for being part of the world!
|
Positively. I know that I
cannot fulfil much without them, and intend to "struggle on" for as
long as I can.
|
I have a love/hate relationship with my mobility aids
|
Ashamed and embarrassed
|
At first I was embarrassed or ashamed when people would stare at
me, but now it doesn’t bother me at all.
|
After years of being vain and struggling without a walking
stick, I realise I now have to walk with one.
|
Not good at all. I feel like I'm too young for this kind of thing
|
My power wheelchair gives me independence, I love it.
|
It’s unfortunate, but life is better with them.
|
Embarrassed, but have to or I fall.
|
I alternate between being okay with them and resenting
them.
|
I wish I didn’t have to use them
|
I prefer to use them rather than struggle so much like my mother
did.
|
I've become comfortable using waking sticks with help of my physical
therapist. This was a difficult transition
|
Don’t like to in public
|
For me, mobility aids are tools to maintain my quality of life.
They allow me a stroll through the city or longer "walks". I'm glad
these things exist.
|
It helps me, I also have less pain because of it
|
I use everything to stay independent as long as possible
|
My mobility scooter is my freedom, I use it a lot
|
Comments about using mobility aids
There
were 414 people who answered the question “Have you had any negative
comments/behaviours from people about using mobility aids?”
Table 13 – Comments about mobility aids
Mobility
aid comments
|
Respondents
|
Percentage
|
Wellbeing
|
No negative comments
|
276
|
67%
|
45.7
|
Some negative comments
|
113
|
27%
|
44.2
|
Mixed answer
|
25
|
6%
|
41.1
|
Generally,
most people’s wellbeing is not affected by these comments. Those who have experienced mixed comments have
a slightly lower wellbeing. The table below shows a range of the comments made.
The table includes both positive and negative comments to give an indication of
the range of experiences that people have (similarly in respondents’ own words
or translation into English, with some minor adjustments):
Comments
made about using mobility aids
|
Several ‘friends’ told that I would be ‘giving up’ if I use a
power/wheel chair. I also had someone question the fact that I got out of my
chair and walked into a shop with my crutch - according to them, if you can
walk you don’t need a chair and are a ‘fake’.
|
My significant other broke up with me because others teased them
about my use of a wheelchair. They didn’t tease me directly, but I lost a
relationship of more than five years.
|
Children think I’m an old lady. They refer to me as my daughters’
grandmother because of the stick. They’re only little, they don’t know.
|
Quite often when out shopping, I have had folks mutter and tut
if they have to move around you, making you feel that you are in their way. Folks
seem to expect you will move out of their way as if they have 'right of way'.
If anyone has been verbal, I have simply smiled and said "I wish I was
able to walk like you!"
|
I have sometimes been ignored or talked over.
|
My best friend has told me he doesn't want to be my care giver.
|
They don’t say anything, but some are thinking stuff behind my
back.
|
I’ve had great responses from people who care and understand - ”What
a perfect machine”
|
I thought people would avoid talking about it to me, but my
colleagues have been asking me how I’m getting on with the wheelchair. Folk at the HSP support group zoom meeting
were congratulating me and people on Facebook helped me to choose the wheelchair
over a mobility scooter.
|
I have people ask all the time ‘what did you do?’
|
I am amazed at how wonderful people are about it in
general. It is very common after
shopping, when I am loading my purchases into my van, for people to stop and
ask if they can assist me. It is very
common for people to go out of their way to open a door for me when they see
how difficult it might be for me to hold it open for myself.
|
The majority of people are very supportive and welcoming. But
there are a few times people are unkind
|
Often children ask their parents, who answer not to look at me! When
I have time, I explain in a few words that I just have a disease that
prevents me from walking. It's simple to say and the child has their
explanation! We are regarded as beasts of fair!
|
In a wheelchair, if I am pushed by someone, I become
"transparent" - most often, people talk with the person who pushes
me and do not even look at me.
|
Some people find it strange when I get up from the wheelchair
and walk a bit.
|
Some
people answered a question on balancing negative comments about mobility aids and
using the same aids. Most people who answered said that they need their aids to
be able to get out and about, so there is no balance to be made, or that the
benefits using aids outweigh the comments. One person represents the views of
several with “what people say because of their fear and ignorance is none of my
concern.” There are a small number of
people who are more affected by the negative comments, either going out less to
avoid comments or using their walking aids less to avoid comments. A few say
that they don’t like to have their mobility aids included in photos of them.
Mobility Summary
The
results show a general decrease in wellbeing with increased mobility aid use. Those
who do not use mobility aids have a significantly higher wellbeing. There is a
broad spread of mobility aids used by people, with many people identifying
which their favourite aids are. The most common favourite aids fall into a
similar pattern as aids used. Several people note the benefits they get from
their favourite aids which include increasing independence, reducing symptoms and
being able to enjoy doing more.
When exploring
people’s thoughts about using mobility aids, there is a wide range of views
that people have. Those who gave generally negative comments about using aids
had a significantly lower wellbeing than others. There is a range of
uncertainty about changing mobility aids in the future, and a corresponding range
of opinions on how people feel about future change. There is a similar spread
of positive and negative views that people have heard from other people about
their use of mobility aids, which affects how people feel about themselves.
Once
peoples walking distance begins to be limited there is a drop-off in wellbeing,
and similarly once people begin to have difficulties standing up their
wellbeing is lower.
7) Change in Mobility
In all
previous surveys, respondents answered how their mobility has changed over the
last five years. For this tenth survey, mobility aid usage of any people answering
more than one survey are examined.
Table 16 – People completing more than one
survey
Range of survey
years
|
Respondents
|
Percentage
|
1 year (2022 and 2021 only)
|
73
|
31%
|
2-3 years (2022 and 2020/2019)
|
75
|
31%
|
4-5 years (2022 and 2018/2017)
|
45
|
19%
|
6-7 years (2022 and 2016/2015)
|
22
|
9%
|
8-9 years (2022 and 2014/2013)
|
22
|
9%
|
Total
|
234
|
100%
|
This
shows that there are 4-9 years of mobility aid use for about two fifths of
people (89 people, 38%) and 1-3 years of data for three fifths of people (148
people, 62%). The table does not show how many times people completed the
surveys for years in between those shown.
No changes in aids
Of the
234 people, 92 report the same mobility aid use in every year they have
completed the survey. Of these there are 15 people who use no aids and 77 who
use some mobility aids.
In this
section, the percentage shows the proportion of people in that group. For
example, in Table 17 there are 62% of people with data across 1 year with no
change in mobility aid use (45 is 62% of 73).
Table 17 – No change in mobility aid use
Range of
survey years
|
Respondents
|
Percentage
|
1 year (2022 and 2021 only)
|
45
|
62%
|
2-3 years (2022 and 2020/2019)
|
27
|
38%
|
4-5 years (2022 and 2018/2017)
|
15
|
33%
|
6-7 years (2022 and 2016/2015)
|
3
|
14%
|
8-9 years (2022 and 2014/2013)
|
2
|
9%
|
The five people in the
6-9 year range are: 1 with no aids, 2 with walking sticks, 1 with a wheelchair
and 1 using orthotics and/or mobility aids some of the time.
No or small changes in mobility aid use
There
are 8 people who do not use mobility aids, but have noticed that their mobility
has changed since they started completing these surveys.
There
are 18 people who report using FES/AFO/Orthotics and/or mobility aids some of
the time in each year. The majority of these are in the one year group, but
there are a few people who use orthotics/AFO/FES and/or who mobility aids some
of the time over longer periods. Two people indicate a change in mobility aid
use across the years, signifying that they have started using a wheelchair or
frame some of the time.
Table 18 – AFO/FES/Mobility aids some of the
time
Range of
survey years
|
Respondents
|
Percentage
|
1 year (2022 and 2021 only)
|
12
|
16%
|
2-3 years (2022 and 2020/2019)
|
4
|
6%
|
4-5 years (2022 and 2018/2017)
|
1
|
2%
|
6-7 years (2022 and 2016/2015)
|
1
|
5%
|
8-9 years (2022 and 2014/2013)
|
0
|
-
|
There
are 51 people who report using walking sticks all or most of the time in each
year. These results are spread throughout the years, indicating that people can
spend many years using walking sticks most or all of the time. 17 people
identified changing between using their sticks most of the time and all of the
time.
Table 19 – Walking sticks most or all of the
time
Range of
survey years
|
Respondents
|
Percentage
|
1 year (2022 and 2021 only)
|
20
|
27%
|
2-3 years (2022 and 2020/2019)
|
10
|
14%
|
4-5 years (2022 and 2018/2017)
|
16
|
36%
|
6-7 years (2022 and 2016/2015)
|
2
|
9%
|
8-9 years (2022 and 2014/2013)
|
3
|
14%
|
There
are 10 people who report using walking frames all or most of the time in each
year. There are not many people who report using a walking frame every time
they complete the survey. This may indicate that a walking frame is more of a
transitional mobility aid. 2 people identified changing between using their
frame most of the time and all of the time.
Table 20 – Walking frames/rollators most or all
of the time
Range of
survey years
|
Respondents
|
Percentage
|
1 year (2022 and 2021 only)
|
7
|
10%
|
2-3 years (2022 and 2020/2019)
|
1
|
1%
|
4-5 years (2022 and 2018/2017)
|
2
|
4%
|
6-7 years (2022 and 2016/2015)
|
0
|
-
|
8-9 years (2022 and 2014/2013)
|
0
|
-
|
There
are 25 people who report using wheelchairs all or most of the time in each
year. These results are spread throughout the years, indicating that people can
spend many years using wheelchairs most or all of the time. 6 identified
changing from using their chair from most to all of the time.
Table 21 – Wheelchair/mobility scooter most or
all of the time
Range of
survey years
|
Respondents
|
Percentage
|
1 year (2022 and 2021 only)
|
10
|
14%
|
2-3 years (2022 and 2020/2019)
|
9
|
13%
|
4-5 years (2022 and 2018/2017)
|
0
|
-
|
6-7 years (2022 and 2016/2015)
|
3
|
14%
|
8-9 years (2022 and 2014/2013)
|
3
|
14%
|
Beginning to use aids
There
are 27 people who reported using no mobility aids in their first survey answers
and have transitioned into using mobility aids since then.
Table 22 – Beginning to use aids
Range of
survey years
|
Some of the time/AFO/FES
|
Sicks most/all
|
Frames most/all
|
Chair most/all
|
1 year (2022 and 2021 only)
|
2
|
1
|
0
|
0
|
2-3 years (2022 and 2020/2019)
|
7
|
4
|
0
|
0
|
4-5 years (2022 and 2018/2017)
|
2
|
3
|
0
|
0
|
6-7 years (2022 and 2016/2015)
|
1
|
1
|
0
|
1
|
8-9 years (2022 and 2014/2013)
|
3
|
2
|
0
|
0
|
Total
|
15
|
11
|
0
|
1
|
This
data shows that the majority of people who have started using mobility aids in
the time that they have been completing surveys currently use Orthotics/AFO/FES,
any of the mobility aids some of the time, or walking sticks most/all of the
time. There are two people who use a wheelchair some of the time and one person
who uses a wheelchair most of the time.
Walking frames transitions
There
are 6 people who have moved from using walking frames/rollators to using
wheelchairs/mobility scooters. There are 27 people who were using other aids in
earlier surveys and now use walking frames/rollators.
Table 23 – Transition through walking frames
Range of
survey years
|
Frames to chairs
|
Aids some to frames
|
Sticks to frames
|
1 year (2022 and 2021 only)
|
2
|
0
|
4
|
2-3 years (2022 and 2020/2019)
|
2
|
5
|
5
|
4-5 years (2022 and 2018/2017)
|
1
|
1
|
3
|
6-7 years (2022 and 2016/2015)
|
1
|
2
|
2
|
8-9 years (2022 and 2014/2013)
|
0
|
5
|
0
|
Total
|
6
|
13
|
14
|
The 6
people who were using walking frames most or all of the time and now use a
wheelchair all or most of the time made that transition in less than 7 years.
These six are shown again in the next section.
The
27 people who were using other aids before their walking frames/rollators split
roughly into two equal groups, one who used Orthotics/AFO/FES and/or mobility
aids some of the time, and the other using walking sticks most or all of the
time. The group using sticks most or all of the time tends to have moved to
using frames most or all of the time in less time than the other group. Information
on how long people were using their aids prior to their first survey is not
available and introduces some uncertainty.
Increased stick usage
There
are 20 people who have moved from using walking aids some of the time into
using walking sticks all or most of the time.
Table 24 – Increased stick usage
Range of
survey years
|
Sticks some/AFO/ FES to sticks more
|
Chair/frame some to sticks
|
1 year (2022 and 2021 only)
|
4
|
1
|
2-3 years (2022 and 2020/2019)
|
4
|
1
|
4-5 years (2022 and 2018/2017)
|
6
|
2
|
6-7 years (2022 and 2016/2015)
|
1
|
1
|
8-9 years (2022 and 2014/2013)
|
0
|
0
|
Total
|
15
|
5
|
There
are 15 people who now use walking sticks most or all of the time who previously
used these some of the time or who used other mobility aids like orthotics or
FES. There are 5 people who now use walking sticks most or all of the time who
previously used a combination of walking aids some of the time including a
wheelchair and/or frame. Of the mobility aid scale used, this is the most
challenging area to interpret as there are several different types of mobility
aids that give rise to a low score, and demonstrates there are several combinations
that people use, ending up using walking sticks most or all of the time.
Into wheelchairs
There
are 33 people who were using other aids in earlier surveys and now use wheelchairs
or mobility scooters most or all of the time. Six are described above (in
frames), and 27 are not in any previous table.
Table 25 – Increased chair usage
Range of
survey years
|
Aids some/AFO to chairs
|
Sticks to chairs
|
Frames to chairs
|
1 year (2022 and 2021 only)
|
0
|
5
|
2
|
2-3 years (2022 and 2020/2019)
|
0
|
9
|
2
|
4-5 years (2022 and 2018/2017)
|
2
|
3
|
1
|
6-7 years (2022 and 2016/2015)
|
3
|
2
|
1
|
8-9 years (2022 and 2014/2013)
|
2
|
1
|
0
|
Total
|
7
|
20
|
6
|
Seven
people previously used orthotics/AFO/FES and/or mobility aids some of the time
and now use a wheelchair all or most of the time. These transitions tend to
have occurred over at least 4 years.
There
are 20 people who were walking sticks most or all of the time in earlier
surveys and now use wheelchairs or mobility scooters most or all of the time. These
changes have taken place over all timeframes reported. Four of these people
report using a walking frame at some point in their answers to these surveys,
whereas the other 16 report only using sticks or chairs. Of these 16 people,
six report changes where they move from sticks most or all to chairs most or
all of the time, whereas 10 people report variation between their aids in different
surveys switching their predominant mobility aids from year to year.
The
transition into using wheelchairs most or all of the time is unlikely to be a
straight switch for many people, and predominant mobility aid use will vary
from year to year during this period.
Mobility change summary
The analysis
highlights the varying rates of change of mobility aids, as summarised, with
the percentage column relating to the total column:
Table 26 – Mobility use change summary
Change
|
Total
|
1-5 years
|
6-9 years
|
Percentage
|
No change in aid use
|
92
|
87
|
5
|
39%
|
Small changes in aid use
|
27
|
19
|
8
|
12%
|
Beginning to use aids
|
27
|
19
|
8
|
12%
|
Change to sticks most or all the time
|
20
|
18
|
2
|
9%
|
Change to frames most or all the time
|
27
|
17
|
10
|
12%
|
Change to chairs most or all the time
|
33
|
24
|
9
|
14%
|
No aids but small change
|
8
|
5
|
3
|
3%
|
Total
|
234
|
189
|
45
|
-
|
This tables shows that
the pattern of mobility aid use in people with HSP is highly variable. Many
people experience a gradual change in their mobility over time, with
corresponding small changes in mobility aid use. There are others who either
have stopped changing their mobility aids, or have not been answering surveys
for long enough to capture their change. For some people their changes in
mobility aids are more rapid indicating that their HSP progresses at a higher
rate than experienced by many.
8) Pain
Pain from HSP
This section included
questions about pain. People answered if they got pain from their HSP, and then
the completed the Short-form McGill Pain Questionnaire 2. 503 respondents
answered if they got pain:
Table 27 – Pain from HSP
Pain from
HSP?
|
Yes
|
No
|
Wellbeing Yes
|
Wellbeing No
|
All
|
380 (76%)
|
123
|
44.6
|
48.8
|
Mobility 0-1
|
48 (71%)
|
20
|
49.2
|
55.5
|
Mobility 2-3
|
71 (76%)
|
22
|
44.5
|
49.2
|
Mobility 4-5
|
141 (77%)
|
42
|
44.3
|
47.5
|
Mobility 6-7
|
46 (74%)
|
16
|
41.9
|
(45.9)
|
Mobility 8-9
|
74 (76%)
|
23
|
44.2
|
47.7
|
This
shows that around three quarters of people get pain from their HSP, and pain is
independent of use of mobility aids. Those who do not get pain from their HSP
have a higher wellbeing than those who get pain from their HSP. The highest
wellbeing is from those who do not get pain from HSP and who do not use
mobility aids. The lowest wellbeing is from those who use walking frames all or
most of the time and who get pain from their HSP. There are differences in the
proportion who get pain from their HSP by location:
Average pain score
The
Short-form McGill Pain Questionnaire 2 (SF-MPQ-2) comprises 22 different
descriptions of pain, and respondents are asked to score each of these on a
scale of 0 to 10, where 0 represents no pain at all and 10 is the worst
possible pain. The overall score is the average of all 22 items, and ranges
from 0 to 10. In addition to the overall score the 22 items are grouped into
four sub-categories representing continuous pain (6 items), intermittent pain
(6 items), neuropathic pain (6 items) and affective descriptors of pain (4
items).
Around
315 people who said that they got pain from their HSP completed the pain
questionnaire, and 47 people who said that they did not get pain from their HSP
completed the questionnaire. The guidance for the questionnaire indicates that
results are still valid if there are a few items not completed, and results have
been included in the analysis where scores have been given for at least 19 descriptors.
For the four subcategories results have been included where a score is given in
all or all but one descriptor.
Table 28 – SF-MPQ-2 score, with pain from HSP
Mobility
|
Overall
|
Continuous pain
|
Intermittent pain
|
Neuropathic pain
|
Affective pain descriptors
|
All
|
3.02
|
3.58
|
2.86
|
2.79
|
3.10
|
Mobility 0-1
|
2.49
|
3.43
|
2.22
|
2.25
|
2.51
|
Mobility 2-3
|
3.20
|
3.67
|
2.79
|
2.76
|
3.31
|
Mobility 4-5
|
3.10
|
3.68
|
3.01
|
2.60
|
3.20
|
Mobility 6-7
|
2.69
|
2.60
|
2.42
|
2.96
|
3.04
|
Mobility 8-9
|
3.18
|
3.78
|
3.30
|
3.07
|
3.07
|
Respondents
|
312
|
314
|
315
|
319
|
318
|
This
shows that the overall average score for people who get pain from their HSP is
3, generally increasing once mobility aids are used. Scores for continuous pain
and affective pain descriptors are generally higher, and scores for
intermittent pain and neuropathic pain are lower. Those not using mobility aids
have the lowest overall score, with the highest score for their continuous pain
sub-category.
Those
who use mobility aids have similar scores for continuous pain and affective
pain descriptors and lower scores for intermittent pain and neuropathic pain.
Scores for continuous pain are the highest for all groups except those who use walking
frames all or most of the time, where affective pain scores highest.
Those
who do not get pain from their HSP score an overall average of 0.37, with
higher scores for affective pain descriptors and neuropathic pain. All average
scores are between 0.1 and 1, with the highest score for affective pain
descriptors from people who use wheelchairs all or most of the time scoring
0.98.
Pain descriptors
The
following table shows the average score for each of the 22 pain descriptors. The
table also shows the proportion of people who scored more than 0 (no pain) for each
descriptor, grouped into three categories. A low score is for anyone who scored
that pain item between 1 and 3 points, a medium score for people scoring
between 4 and 7 and a high score for those scoring between 8 and 10.
Table 29 – HSP pain descriptors and scores
SF-MPQ-2
Pain descriptors
|
Score
(pain=yes)
|
Score
(pain=no)
|
Low score
(1-3)
|
Medium score
(4-7)
|
High score
(8-10)
|
Tiring, exhausting
|
6.0
|
1.6
|
11%
|
35%
|
37%
|
Cramping pain
|
5.0
|
0.7
|
15%
|
35%
|
25%
|
Aching pain
|
4.7
|
0.3
|
12%
|
34%
|
24%
|
Tingling or “pins and needles”
|
3.9
|
0.7
|
22%
|
29%
|
17%
|
Numbness
|
3.9
|
1.1
|
24%
|
30%
|
15%
|
Shooting pain
|
3.7
|
0.2
|
19%
|
31%
|
13%
|
Sharp pain
|
3.6
|
0.1
|
20%
|
24%
|
15%
|
Stabbing pain
|
3.2
|
0.2
|
18%
|
23%
|
12%
|
Heavy pain
|
3.1
|
0.1
|
15%
|
21%
|
15%
|
Tender
|
2.9
|
0.4
|
21%
|
23%
|
10%
|
Throbbing pain
|
2.9
|
0.2
|
20%
|
27%
|
8%
|
Hot, burning pain
|
2.9
|
0.3
|
16%
|
22%
|
12%
|
Gnawing pain
|
2.5
|
0.0
|
18%
|
20%
|
8%
|
Electric shock pain
|
2.4
|
0.2
|
14%
|
17%
|
10%
|
Fearful
|
2.3
|
0.6
|
15%
|
19%
|
9%
|
Cold, freezing pain
|
2.3
|
0.3
|
13%
|
18%
|
9%
|
Punishing, cruel
|
2.2
|
0.0
|
12%
|
14%
|
10%
|
Piercing
|
1.9
|
0.1
|
12%
|
14%
|
8%
|
Sickening
|
1.8
|
0.2
|
12%
|
16%
|
5%
|
Itching
|
1.7
|
0.3
|
14%
|
16%
|
5%
|
Splitting pain
|
1.6
|
0.1
|
13%
|
13%
|
6%
|
Pain caused by light touch
|
1.5
|
0.3
|
16%
|
14%
|
3%
|
The
majority of people (84%) identify that their pain from HSP is tiring or
exhausting, with more than a third of people scoring that high or medium.
This is the highest scoring of any of the 22 descriptors, with over three
quarters scoring medium or high. The average score for people who get pain from
their HSP is 6.0, and the average score for people who do not get pain from
their HSP is 1.6.
Cramping pain and aching pain are identified by more than 70% of
respondents, with about a quarter scoring high and more than a third scoring
medium. About 60% of people score high or medium, with average scores of around
5 for people who get pain from their HSP.
The
next grouping, with around two thirds of people scoring, are numbness, tingling
and shooting pain. Some 15% score these high and about a third score
medium. With these three descriptors almost half of people score medium or high,
and average scores approaching 4. Numbness is the second highest score for
people who do not get pain from their HSP, with an average score of 1.1.
Although
there are some who score high on all descriptors, the six highlighted ones
describe the way the majority describe pain from HSP. These six descriptors include
all of the four sub-categories:
Table 30 – Sub-categories of the highest scoring
descriptors
SF-MPQ-2
Pain descriptors
|
Sub-category
|
Tiring, exhausting
|
Affective
descriptor
|
Cramping pain
|
Continuous
pain
|
Aching pain
|
Continuous
pain
|
Numbness
|
Neuropathic
pain
|
Tingling or “pins and needles”
|
Neuropathic
pain
|
Shooting pain
|
Intermittent
pain
|
The
other descriptors where over half of respondents scored more than zero are:
Sharp pain, Tender pain, Throbbing pain, Stabbing pain, Heavy pain and Hot,
burning pain. Average scores for people with pain from their HSP are between
3.6 and 2.9.
Pain intensity and location
Respondents
identified where they felt their pain, choosing to describe their pain as ‘No
pain’, ‘Mild’, ‘Discomforting’, ‘Distressing’, ‘Horrible’ or ‘Excruciating’ in
different areas of the body. Around 350 people gave answers to these questions,
with about 300 who say that they get pain from their HSP and about 50 who say
that they do not get pain from their HSP.
Table 31 – Overall pain intensity
SF-MPQ-2
Overall pain intensity
|
Respondents (all)
|
Respondents (pain from HSP)
|
Wellbeing
(pain from HSP)
|
No pain
|
67
|
34
|
50.3
|
Mild
|
77
|
73
|
43.8
|
Discomforting
|
127
|
124
|
44.9
|
Distressing
|
39
|
38
|
41.3
|
Horrible
|
16
|
16
|
(37.5)
|
Excruciating
|
1
|
1
|
-
|
This
table shows that most people describe their pain intensity from HSP as
discomforting. Those who said that they do not get pain from their HSP
described their pain intensity as no pain, mild or discomforting. None of the
people who do not get pain from their HSP describe their pain intensity as distressing,
horrible or excruciating.
People
who describe pain as distressing, horrible or excruciating have a lower
wellbeing than others, and those describing pain intensity as ‘no pain’ have a
higher wellbeing. Overall, around one fifth say that they get no pain from
their HSP, which is a similar result to the yes/no question “do you get pain
from your HSP?”
Respondents
also used pain intensity descriptors for different areas of the body to
identify where people perceive their pain to occur. This table shows results
for people who say that they get pain from their HSP.
Table 32 – Pain intensity and location
Pain
location
|
No pain
|
Mild
|
Discomforting
|
Distressing
|
Horrible
|
Excruciating
|
Overall
|
34
|
73
|
124
|
38
|
16
|
1
|
Feet
|
35
|
67
|
108
|
55
|
29
|
9
|
Legs
|
11
|
45
|
139
|
65
|
32
|
12
|
Hips
|
64
|
67
|
82
|
50
|
30
|
8
|
Stomach
|
201
|
37
|
44
|
14
|
5
|
2
|
Chest
|
242
|
28
|
21
|
11
|
2
|
0
|
Back
|
56
|
54
|
104
|
44
|
29
|
15
|
Neck
|
145
|
54
|
61
|
25
|
14
|
3
|
Head
|
190
|
46
|
32
|
22
|
10
|
4
|
Arms
|
153
|
63
|
58
|
15
|
12
|
3
|
Hands
|
149
|
70
|
52
|
19
|
14
|
0
|
Without treatment
|
28
|
55
|
101
|
47
|
27
|
22
|
This
table shows that the areas of the body where people perceive the least pain
from HSP are the chest, stomach and head. People perceive the greatest pain in
their legs, feet, back and hips. For these four areas where people feel the
greatest pain over one quarter of people describe their pain as distressing,
horrible or excruciating.
The
last line in the table shows how people do or would perceive their pain if they
were not taking any treatments for their pain. This result shows that almost
twice as many people would describe their pain as distressing, horrible or
excruciating, which indicates that treatments for pain are helping many
respondents in the management of their pain.
Change in pain since 2017
8.19
There
are around 50 respondents who answered the pain questions in both 2017 and
2022. 47 people answered ‘do you get pain from your HSP’ in both years:
Table 33 – Individual responses – pain from HSP
Pain from
HSP?
|
Respondents
|
Yes in 2017 and in 2022
|
37
|
No in 2017 and in 2022
|
7
|
Yes in 2017 and No in
2022
|
2
|
No in 2017 and Yes in
2022
|
1
|
In
2017 81% of people identified that they had pain from their HSP, which compares
with 76% from this survey. This may suggest that around four in five people
have pain from their HSP and that generally pain with HSP is likely not to
change from over the years for individuals.
When
looking at the specific descriptors of pain the results are very similar. The
descriptor selected by the highest number of people in both years was
“Tiring-exhausting”, with similar average scores, going from 6.9 in 2017 to 6.0
in 2022.
The 2nd
and 3rd ranking descriptors have swapped order in the two years,
cramping pain and aching pain, and the next three descriptors, tingling,
numbness and shooting pain are in the same order. This indicates that the
overall descriptions of pain from HSP have not changed since 2017.
When
looking at the pain locations, these have not changed since 2017, with more
people finding pain from their HSP in their legs and feet, followed by their
back and hips. Chest and stomach are the two locations where the least HSP pain
is felt in both years.
Comments about pain
Some
respondents also gave comments about their HSP pain. There were quite a few
common threads in the comments:
- Some
report discomfort from their HSP but do not regard it as pain
- Some
note that their pain varies, sometimes with time of day, sometimes with
temperature
- Some
are unsure if their pain comes from their HSP or from some other condition
- There
are overlaps between some peoples pain and their fatigue
- Some
report spasms instead of pain
- Some
report pain increasing with activity, and others report pain increasing when they’re
sedentary
- Some
are reporting pain in parts of their body they are now using more with their altered
gait
- Some
report that their pain is the worst part of their HSP
- Some
report that their pain makes it more difficult for them to sleep, affecting their
rest and recovery
- Some
have pain in specific areas of their body, whereas others describe a general
levels of pain
- Some
say that they don’t get much help from their pain medications
- Some
report that massage, stretching, acupuncture and/or exercise help relieve their
pain
Pain Summary
Around
three quarters of people with HSP identify that they get pain as one of their
symptoms. The majority identify that their as tiring or exhausting, including
both those who say they get pain from HSP and those who do not. The other pain
descriptors identified by the greatest number of people are: Cramping pain, aching
pain, numbness, tingling pain and
shooting pain. Using the SF-MPQ-2 scoring those who get pain from their HSP
have an average score of 3 out of 10, compared to 0.9 for people who do not get
pain from their HSP.
People
perceive the greatest pain in their legs, feet, back and hips. For these areas
over one quarter of people describe their pain in these areas as distressing,
horrible or excruciating. There is a wide range of views about peoples pain
from HSP. People who describe their overall pain from HSP as horrible or
excruciating have a lower wellbeing, and those who get no pain from their HSP
have a higher wellbeing.
Comparing
these results with those in 2017 shows that for many people pain does not
change over time. There is a slight decrease in SF-MPQ-2 scores for most people
answering in both years which may indicate that people perceive their pain to
be slightly lower over time. However, there are also people whose pain
increases slightly over time so it is not a consistent pattern.
9) Menstrual Health
These
questions explored if there were issues with menstrual health alongside HSP.
Respondents were asked if commonly occurring HSP symptoms (spasticity, pain,
fatigue and mental health) changed during their menstrual cycle. 511
respondents indicated their sex, with 273 females, 236 males and 2 who
identified as something else.
There
is a small difference in wellbeing between males and females, with females
having a slightly lower wellbeing and males having a slightly higher wellbeing.
Table 36 – Wellbeing and sex
What is
your sex?
|
Respondents
|
Wellbeing
|
Female
|
273 (53%)
|
44.8
|
Male
|
236 (46%)
|
46.7
|
Something else
|
2
|
-
|
There
were between 170 and 175 female who answered these questions, and around 10
males who answered these questions. Those identifying as male did not affect the
results, and these have been included in the analysis as it is not clear why
these questions were answered. Nobody identified as male, answered these
questions and was answering the questions on behalf of someone else.
Spasticity
The
first question explored changes in spasticity. 185 people answered this
question, comprising 173 female and 12 male.
Table 37 – Spasticity changes during menstrual
cycle
Level of
change
|
Respondents
|
Percentage
|
Wellbeing
|
Much worse
|
22
|
12%
|
43.0
|
Worse
|
33
|
18%
|
42.0
|
About the same
|
129
|
70%
|
45.1
|
Better
|
1
|
1%
|
-
|
Much better
|
1
|
1%
|
-
|
These results
show that 30% of people feel their spasticity gets worse or much worse during
their menstrual cycle, and there is a corresponding reduction in wellbeing. The
majority of people feel that their spasticity did/does not change during their
cycle. A very small number of people feel their spasticity gets better during
their cycle. There is little difference in results across location and use of
mobility aids.
Pain
The
second question explored changes in pain. 183 people answered this question,
comprising 173 female and 10 male.
Table 38 – Pain changes during menstrual cycle
Level of
change
|
Respondents
|
Percentage
|
Wellbeing
|
Much worse
|
22
|
12%
|
43.0
|
Worse
|
44
|
24%
|
44.1
|
About the same
|
116
|
63%
|
45.2
|
Better
|
1
|
1%
|
-
|
Much better
|
0
|
-
|
-
|
These
results show that 36% of people feel their pain
gets worse or much worse during their menstrual cycle, but changes in wellbeing
are not significant. The majority of people feel that their pain did/does not
change during their cycle. A very small number of people feel their pain gets
better during their cycle. There is little difference in results across
location and use of mobility aids.
Fatigue
The
third question explored changes in fatigue. 187 people answered this question,
comprising 175 female and 12 male.
Table 39 – Fatigue changes during menstrual
cycle
Level of
change
|
Respondents
|
Percentage
|
Wellbeing
|
Much worse
|
36
|
19%
|
42.5
|
Worse
|
65
|
35%
|
45.4
|
About the same
|
85
|
45%
|
44.6
|
Better
|
1
|
1%
|
-
|
Much better
|
0
|
-
|
-
|
These
results show that 54% of people feel their fatigue gets worse or much worse during
their menstrual cycle, with those affected the most having a corresponding
reduction in wellbeing. A very small number of people feel their fatigue gets
better during their cycle. There is little difference in results across
location and use of mobility aids.
Mental health
The
fourth question explored changes in mental health. 183 people answered this
question, comprising 172 female and 11 male.
Table 40 – Mental health changes during
menstrual cycle
Level of
change
|
Respondents
|
Percentage
|
Wellbeing
|
Much worse
|
17
|
9%
|
(35.6)
|
Worse
|
42
|
23%
|
40.7
|
About the same
|
118
|
64%
|
46.5
|
Better
|
6
|
3%
|
-
|
Much better
|
2
|
1%
|
-
|
These
results show that 32% of people feel their mental health gets worse or much
worse during their menstrual cycle, with a corresponding reduction in
wellbeing. The reduction in wellbeing for those affected the most is likely to
be significant. The majority of people feel that their mental health did/does
not change during their cycle. A few people feel their mental health gets
better during their cycle. There is little difference in results across
location and use of mobility aids.
Other symptoms
Several
respondents noted that they also had worse bladder and/or bowel issues during
their cycle, with bladder issues affecting 9 people and bowel issues affecting
3. A small number of people noted spasms, changes in mood, and weakness.
Hormonal contraceptives and/or hormone
replacement therapy
Respondents
were asked if their HSP symptoms varied when using hormonal contraceptives and/or
when using hormone replacement therapy. One person identified that hormone
replacement therapy made everything better, and another noted that these always
worsened symptoms. All other respondents noted that they do/didn’t have or do/didn’t
notice any changes.
Menstrual hygiene products
Respondents
were asked if their HSP restricted the types of menstrual hygiene products they
would like to use. Most people said no, their HSP did not restrict the type of
product they could use.
Several
respondents noted that their HSP diagnosis had occurred after their menopause,
so they did not need to consider this.
Some
15 respondents indicated that they could not use tampons for a variety of
reasons, or they preferred to use pads. Some said that it was difficult or
uncomfortable to use tampons, others noted they could not use tampons because
of spasms or increased spasticity. One noted that they prefer pads, which they
have to use because of their bladder issues.
Menstrual health comments
Several
respondents made comments about their HSP and their menstrual health. There
were many people who did not comment, or commented with a simple ‘no’ to these
questions. A few people noted that they had not previously thought about if
their HSP symptoms changed during their cycle. Some people noted that they have
had a hysterectomy, with one or two noting this was the best thing they had
done. One or two people use an IUD or contraceptive injections which has
stopped their menstruation.
A few
people noted that their HSP has affected their pelvic floor muscles, and there
are one or two people who feel that their menstrual cycle is either longer, shorter
or more varied as a result of their HSP. One person thought their menopause was
bought forward because of their HSP.
Menstrual health summary
Between
about a third and half of people experience worse spasticity, pain, fatigue
and/or mental health during their menstrual cycle. Those with worse spasticity,
fatigue and/or menstrual health tend to have a lower wellbeing than others. Several
people also find that their bladder and/or bowel are more affected during their
cycle.
Those
with later onset HSP have relatively little overlap between their HSP symptoms and
their pre-menopause menstrual cycle. For those with earlier onset HSP there are
a few reasons why some people avoid using tampons.
Several
respondents made comments about their HSP and their menstrual health. Some had
not previously thought about if their HSP symptoms changed during their cycle.
A few people noted that their HSP has affected their pelvic floor muscles or changed
their menstrual cycle.
10) Life with HSP
Advice to others
Respondents
were asked “If you were giving advice to someone else with HSP, what is the
most important thing you would say?” 407 people provided an answer to this
question. These answers have been grouped into categories. Some respondents
answered with a couple of words, whereas others answered with sentences. This
table shows advice offered by at least five people.
Table 41 – Advice to others
Advice
|
People
|
Stay positive
|
101
|
Keep moving
|
100
|
Be kind to yourself
|
37
|
Find others
|
26
|
Focus on what you can do
|
23
|
Start treatments
|
22
|
Inform yourself about HSP
|
22
|
Accept that you have HSP
|
18
|
Regular stretching
|
18
|
Find expert advice from
healthcare professionals
|
16
|
Seek help when you need
to
|
13
|
Physiotherapy
|
13
|
Go at your own pace
|
10
|
Don’t be scared of using mobility
aids
|
9
|
The
two pieces of advice offered the most are around staying positive and keeping
moving, covering around half of the answers given. These results are discussed
below, but the following table gives some of the comments made by respondents
These comments are in the respondents’ own words (or Googles translation of
them into English), with occasional minor adjustments for clarity or anonymity:
Advice
Comments
|
Even though having HSP is tough, remember that things could
always be worse.
|
Enjoy today, deal with whatever as and when, and exercise/rest
when needed.
|
Do everything you can, while you can. Take every day as it
comes. Enjoy life to the full and don’t worry about petty things.
|
Get support. Learn about your body. Do/ learn about whatever makes it feel
better/healthier.
|
Stay positive, adapt, and never give up.
|
Stretch! Use it or lose it
|
Know things will be tough, accept your HSP, and life will be
easier for you
|
Don't be overwhelmed about your condition, concentrate on those
things that you can do rather than those you can't.
|
Be an ‘expert’ patient - you have to be your own advocate in the
rare disease world. Learn about your condition first and foremost. And travel
- continue to explore the world in a new way.
|
Keep calm and learn about your new self
|
Live your life to the full. You have it, it does not have you
|
Don't allow yourself to become physically or emotionally
isolated. Use help whenever you can
find it to get out of home as often as is reasonable.
|
Get over your preconceived notions about using something that
preserves your “gas” so you can participate in more and longer.
|
Look at the possibilities, when a door closes, open another one.
Do not feel sorry for yourself.
|
Accept that you’re like you are, and try to make the best out of
it - life is to be lived
|
Make a plan for the future. Do what you can before you can’t do
it in the future
|
Be patient and enjoy what you can do today.
|
Keep smiling!
|
Don't focus on your goals. Focus on your values and take every
day as it comes.
|
Don’t be afraid to use aids.
|
Keep active, try and remain positive, join an HSP group, use
aids if and when necessary, disregard negative comments if they happen
|
One step at a time, always forward
|
Your attitude/mindset is very important. You can do anything - you may just have to
do it differently!
|
Don't quit! Don't let other people's ignorance keep you from
living the best life you can live. I wish I had the support and encouragement
when I was growing up. I was very shy and my father treated me like a China
doll. I felt I wasn't good at anything and was too scared to try.
|
Get out of the house every day.
Don’t be afraid to ask for help
|
Talk with others. Find out what works. Get plenty of rest.
Stretch. Make the best of everything
|
Although it takes longer to get ready for things, there's still
life to live. It can be a shift to
accept slower, but hopefully joy can be still captured.
|
Try to have more positive thoughts and always think about being
grateful for the good things in your life
|
Review outdoor mobility aids to stay independent longer
|
Think about taking the time to do what you love
|
No matter how much fun your work is, don't keep working full
time
|
Accept yourself and accept that you have a disease
|
Generally,
staying positive comments were either around keeping a positive attitude and
enjoying life, or around not letting life get you down and not giving up.
Comments around keeping moving were around doing exercise, stretching,
remaining active, walking (where possible), physiotherapy and not sitting
still.
Being
kind to yourself comments are mostly around taking each day as it comes, not
worrying about what other people think, listening to your body and having a
good quality of life. Focussing on what you can do comments are around
maintaining independence, not worrying about things you can no longer do,
enjoying what you can do, and living your life in the current moment.
Finding
others comments are around joining support groups or facebook groups, and also
having good friends, being open to talking about things with others, and
connecting with other people.
Treatment
comments are mostly around stretching and physiotherapy, with others talking
about rehabilitation and only one or two people talking about drug treatments.
Do you Consider Yourself to be Disabled?
There
are 505 respondents who answered this question, as shown in the following grid.
Table 42 – Identify as Disabled?
Consider
Self Disabled?
|
Yes
|
No
|
Wellbeing Yes
|
Wellbeing No
|
All
|
425 (81%)
|
80
|
44.6
|
51.5
|
Mobility 0-1
|
44 (65%)
|
24
|
49.1
|
54.9
|
Mobility 2-3
|
70 (74%)
|
24
|
44.0
|
50.7
|
Mobility 4-5
|
166 (90%)
|
18
|
44.6
|
(48.5)
|
Mobility 6-7
|
56 (89%)
|
7
|
42.6
|
-
|
Mobility 8-9
|
89 (93%)
|
7
|
44.1
|
-
|
Four
fifths of respondents consider themselves to be disabled and one fifth do not.
Although the proportion of people who identify as disabled increases with
reduced mobility, there are both people who do not use mobility aids who
consider themselves disabled and those who use a wheelchair all or most of the
time who do not consider themselves to be disabled. This indicates that
mobility is not the only factor in individuals deciding if they are disabled or
not.
Those
who consider themselves to be disabled have a lower wellbeing score than those
who do not. Once mobility aids are taken into account there is a clearer
distinction on wellbeing, with those identifying as disabled and who use
mobility aids having a lower wellbeing. 275 respondents described the point at
which they have or would describe themselves as disabled.
Table 43 – Point of considering self as disabled
Disabled
point
|
People
|
On diagnosis
|
18
|
With difficulty walking
|
64
|
Needing to use mobility
aids/needing mobility aids all the time
|
74
|
When unable to do certain
activities
|
46
|
Needing
help from other people/needing more help from others
|
27
|
In
receipt of benefits/parking badges
|
12
|
Having to stop work
|
12
|
Regular falls
|
11
|
Affected
by HSP symptoms other than mobility
|
7
|
Affected by the
perceptions of others
|
4
|
This shows that the most common reasons for people considering themselves
to be disabled are points along their use of mobility aids or points along
their changes to their mobility (including difficulties walking or falling).
Other reasons include being no longer able to undertake certain activities or
needing help from other people to be able to do certain things.
Some people regard themselves as disabled at their point of diagnosis, at
the point that they need benefits or when they have to stop working. A few
people use other symptoms or the perceptions of others as the point they regard
themselves as disabled.
Friendships
Respondents
were asked “Describe if you have found making or keeping friends difficult
because of your HSP?” 408 people provided an answer to this question. These
answers have been categorised into yes or no answers. People who answered with
partly, sometimes or other similar answers were grouped into the yes category,
and those that answered not really and similarly were grouped into the no
category.
Table 44 – Difficulties making and/or keeping
friends
Difficulties?
|
Respondents
|
Wellbeing
|
Yes
|
214
|
41.5
|
No
|
194
|
49.3
|
This table shows that
there are similar numbers of people who have difficulties making or keeping
friends as a result of their HSP. Those that have these difficulties have a
lower wellbeing than those who do not.
What would you like to do, but find hard
or impossible?
Respondents
shared their views on things that they would like to do, but that they find
hard or impossible. 406 respondents gave an answer to this question. Some
people gave a single answer and some people listed several items. A few people
said that they find everything hard or impossible, and a few people said that
they are able to do everything that they want. The following table shows
answers given by at least 5 people. The details given by some people is
included in brackets for some of the categories.
Table 45 – What is hard or impossible to do?
Things
that are hard or impossible
|
People
|
Walking (with aids, without
aids, speed, with dog, without support)
|
98
|
Travel (holidays, trips,
planning, on coaches, by plane)
|
44
|
Running
|
40
|
Outdoor activities
(hiking, climbing, camping, on the beach, boating)
|
40
|
Sports (football,
weightlifting, handball, gymnastics, boules)
|
25
|
Dancing
|
20
|
Shopping
|
16
|
Work
|
14
|
Gardening
|
11
|
Get out (out by self, out
with friends, social life, theatres)
|
11
|
Independence (being able
to live without assistance, being autonomous)
|
10
|
Play with kids (some with
their own children, some with their grandchildren)
|
9
|
Stairs
|
8
|
Biking
|
8
|
Activities with friends
or family
|
8
|
Swimming
|
7
|
Standing up
|
6
|
Driving a car
|
5
|
Doing housework
|
5
|
Carrying things
|
5
|
Of
answers given by fewer than 5 people there are some interesting points to note,
a few people note that they find sex difficult, some would like to be able to
get dressed by themselves, one person wishes they could cut their toenails, and
a few would like to be able to bath or shower by themselves. Whilst there are a
few people who wish they could work (or work better) one person wished that
they could quit their job.
It is important to note that many of the activities listed above are
hobbies and pastimes that people enjoy doing, often with family or friends. As
people find these activities harder to do there is potential to negatively
affect their wellbeing. There may be some overlap between these answers and the
previous friendship answers.
Working with HSP
Respondents
answered the question “How has HSP affected your ability to work?” 436 people
answered this question, and all but one of these respondents also gave their
age bracket. The answers were initially divided into four main categories –
those who don’t report any issues, those who work and report issues of one kind
or another, those who are retired, and those who say that they don’t work.
Several
respondents indicated that they were able to work and that their employer had
agreed to various adaptions and alterations at work. A few said that they were
able to work apart from travelling, and a few said they were still in education
and had not worked.
Table 46 – HSP impacts at work
Level of impact
|
Respondents
|
Percentage
|
Wellbeing
|
No impact
|
77
|
18%
|
52.7
|
Some impact
|
157
|
36%
|
44.2
|
Do not work
|
81
|
19%
|
41.9
|
Retired
|
81
|
19%
|
46.2
|
Working with flexibility/adaptions
|
24
|
6%
|
45.3
|
Issues with travelling
|
8
|
2%
|
-
|
Not yet in work
|
8
|
2%
|
-
|
This
table shows that those who are in work and do not have any issues have a
significantly higher wellbeing than others, and those who do not work have a lower
wellbeing.
95%
of those who are retired are over the age of 50. 2% of those who do not work
are over the age of 75. All those not yet in work are under 25. The other
categories are spread across the age brackets.
Those
who report that they have no issues at work have similar answers. Several
people note that their jobs are undertaken sitting down or working on
computers, others note using mobility aids to allow them to work.
There are several
themes in those who report problems or issues at work. Many of these are to do
with HSP symptoms, including:
- Having
to stand for a long time
- Having
to sit for a long time
- Fatigue,
tiredness or concentration problems at work
- Pain
from HSP
- Difficulties
moving about or lifting/carrying things.
Other
people noted that their problems were more down to the attitudes of people they
work with, sometimes their employers, sometimes their colleagues and sometimes
people they meet whilst undertaking their jobs. Those with
flexibility/adaptions note that they have been able to change roles within
their job, alternatively some have reduced the hours that they work or are able
to work remotely. Overall, the adaptions described have allowed people to carry
on working.
People
who do not work have several themes. Some people have quit their work either
because they were no longer able to do their job or because of the attitude of
their employers. Several report that they are on long-term disability insurance/payments
and others note that they were made redundant. People also report that they are
unable to find a job, generally mentioning their HSP symptoms as a reason.
Those
who are retired fall into two main groups, those whose HSP didn’t affect them
until around the point they retired, and others who chose to retire early due
(in part) to the impact of their HSP symptoms. Readers should note that people
identifying as being forced to retire rather than choosing to retire are
classified under “do not work” rather than “retired”.
Depression
Respondents
answered the two question PHQ2 screening tool for depression. 491 people
answered these questions. Answers
are scored giving a total score
between 0 and 6. Several studies regard a score of 3 as the cut-on point for
further assessment of depression, although some studies also suggest a cut-on
point with a score of 2, with a risk of some false positives. My survey results
in 2021 indicated that a cut-on point of 3 was likely to be appropriate for
those with HSP, based on wellbeing scores.
Table 47 – PHQ 2 – Screening for depression
PHQ2
score range
|
Respondents
|
Wellbeing
|
All
|
491
|
-
|
Score 0-2
|
373 (76%)
|
48.4
|
Score 3-6
|
118 (24%)
|
36.2
|
Score 0-1
|
242 (49%)
|
51.1
|
Score 2
|
131 (27%)
|
43.4
|
Score 2-6
|
249 (51%)
|
39.9
|
Using a
cut-on point of 3 points to screen depression, these results show that one
quarter of respondents should be screened further for depression and three
quarters should not. Those who score at least three points have a wellbeing
which is significantly below average, and those who score less than three
points have a wellbeing which is nearly 3 points above average.
These
results show that scores of 0 or 1 have a significantly higher wellbeing and
scores of at least 2 have a significantly lower wellbeing. Around a third of
respondents have a score of 2 with a wellbeing slightly below average. This years results confirm that a cut-on of 3
points is likely to be appropriate for screening depression for people with
HSP.
When
looking at mobility aid use, the results are similar, with those not using aids
having slightly fewer people reaching the 3-point cut-on. When looking at the
country results, those in Italy have less than 10% of people reaching the
3-point cut-on score, and in Spain and Brasil there are between 30 and 40% of
people reaching the 3-point cut-on.
Several
respondents have answered the PHQ2 questions in more than one survey. 12 people
answered in both 2015 and 2022, 45 people in both 2017 and 2022, and 159
answered in both 2021 and 2022. Overall 7 people have answered the questions 4
times, 20 have answered three times and 155 have answered 2 times, giving 182
people with more than one answer.
Of
these 182 people, most score less than 3 in each year. Several (24) score at
least three in each year. There are 32 people who have scored at least three in
some years and not in other years, indicating that their depression has changed
from year to year. For some it has improved, for some it has gotten worse and
some vary from year to year. The following table reports these numbers:
Table 48 – PHQ2 Scores in 2015, 2017, 2021 and
2022
Score
|
People
|
At least 3 in all years
answered
|
24
|
Less than 3 in at least
one year but more than in 2022 (getting worse)
|
11
|
At least 3 in some years
but not others (variable)
|
4
|
At least 3 in at least
one year but less than in 2022 (getting better)
|
17
|
Less than 3 in all years
answered
|
126
|
Research Priorities
Respondents
were asked where they felt research into HSP should be prioritised, and 316
respondents provided an answer. These answers have been grouped into
categories. Some respondents answered with a single word, whereas others
answered with several areas. Each answer has been grouped into one category,
which may not reflect the primary importance of the answer intended by the
respondent.
Table 49 – Research priorities
Priority
|
People
|
Treatments
|
96
|
Cure
|
41
|
Spasticity
|
40
|
Genetics
|
28
|
Cure and/or treatment
|
23
|
Symptoms
|
18
|
Pain
|
17
|
Improved understanding
|
14
|
Gene therapy
|
13
|
More information
|
10
|
Diagnosis
|
7
|
How HSP affects the young
|
6
|
The cause of HSP
|
6
|
Fatigue
|
5
|
Accessibility
|
5
|
Mental health
|
5
|
General health
|
4
|
Cognitive impairments
|
4
|
Complex HSP
|
3
|
There
is some overlap between these categories, with some people noting just these
words, with others identifying which aspects they want treatment research for,
and others inferring they want research into areas without specifically
mentioning treatments.
Many
of those seeking treatments talked about slowing the progression of HSP down, or
stopping the progression. Others sought more/better medications, or combatting
symptoms.
Some
people who identified cure as their priority were angry or frustrated at the
research currently undertaken. They have been told that research is “close” to
finding a cure, but they are annoyed that the “close” isn’t likely to be soon
enough help them.
Those
identifying spasticity identify a range of areas, including reducing camps,
spasms and muscle stiffness, with others wanting to be able to walk
better/again or generally improving their mobility.
Missing Information
Respondents
also identified what information is missing about HSP. There are many
similarities between these answers and the answers given for research
priorities.
Several
people, however, note that information about HSP in doctors and other
healthcare professionals is low, and they feel that this should be improved. Some
are after summaries of HSP research around the world.
Many are seeking
information about treatment options for specific symptoms and how to improve
their understanding of HSP. Several find that the information they get is not
written in a way that they find easy to understand, and note that awareness of
and information about complex HSP is small.
Several
people seek information about the rate of progression of HSP and more
information about their future, which their doctors are reluctant to give them.
Others are looking to improve their mental health, their exercise routines/physiotherapy,
and how to remember that their lives don’t have to be all doom and gloom. Some
are keen to understand how HSP affects their relationships, their family and
other people.
Life with HSP summary
The
two pieces of advice that people with HSP would most often give to others is to
stay positive and to keep moving. Other pieces of advice often given would be
to be kind to yourself, find other people you can talk with, and focus on what
you can do.
People
who are able to find or keep friends tend to have a higher wellbeing than
people who cannot, and people who are able to carry on working have a
significantly higher wellbeing than those who are no longer able to work. Whilst
walking was the activity that the most people identified as being difficult,
there are also many hobbies and interests that people find more difficult to do
with their HSP.
Around
80% of people with HSP consider themselves disabled, with around 20% not considering
themselves disabled. Those who consider themselves disabled have a lower
wellbeing than those who do not. There are wide differences in mobility aid use
in the answers to this question and people gave a wide range of different
reasons why they would consider themselves disabled or not.
Around
a quarter of people screen for depression, but it is noted that depression
varies from year to year for some people, indicating that depression is not
solely linked to HSP and its symptoms. Those who screen for depression have a
significantly lower wellbeing than those who do not.
11) Disclosure
Around 500 people answered questions about disclosing their HSP to other
people. Five questions were asked, with people selecting if they would tell
those people about their HSP.
Employer
This question was
relevant for 341 people who answered. Almost 10% of people said they would not
disclose their HSP to their employer, with the 90% who would split similarly
between yes they would and yes they already have disclosed.
Table 50 – Disclose HSP to your employer
Disclosure
|
Respondents
|
Percentage
|
Wellbeing
|
Yes
|
161
|
47%
|
47.2
|
No
|
31
|
9%
|
45.3
|
Already told
|
149
|
44%
|
45.4
|
There are no differences in wellbeing for people in respect of disclosing
their HSP to their employer. Respondents in France or respondents who do not
use mobility aids are less likely than others to disclose their HSP to their
employer. Differences between age are small.
People you work with
This question was
relevant for 376 people who answered. Less than 10% of people said they would
not disclose their HSP to people they work with, with the 90% who would split
similarly between yes they would and yes they already have disclosed.
Table 51 – Disclose HSP to people you work with
Disclosure
|
Respondents
|
Percentage
|
Wellbeing
|
Yes
|
182
|
48%
|
47.2
|
No
|
28
|
7%
|
40.9
|
Already told
|
166
|
44%
|
46.5
|
People who feel that they would not disclose their HSP to people they
work with have a lower wellbeing that people who have or would disclose. Differences
between mobility aid use, location and age are small.
Close friends
This question was
relevant for 504 people who answered. Around 2% of people said they would not
disclose their HSP to their close friends, with everyone else split similarly
between yes they would and yes they already have disclosed.
Table 52 – Disclose HSP to close friends
Disclosure
|
Respondents
|
Percentage
|
Wellbeing
|
Yes
|
247
|
49%
|
45.5
|
No
|
12
|
2%
|
(42.3)
|
Already told
|
245
|
49%
|
45.7
|
People who feel that they would not disclose their HSP to their close
friends with have a lower wellbeing that people who have or would disclose. Differences
between mobility aid use, location and age are small.
Other people you know
This question was
relevant for 490 people who answered. Around 10% of people said they would not
disclose their HSP to other people they know, with 60% saying they would
disclose and 30% saying they already have disclosed.
Table 53 – Disclose HSP to others you know
Disclosure
|
Respondents
|
Percentage
|
Wellbeing
|
Yes
|
284
|
58%
|
46.3
|
No
|
53
|
11%
|
42.9
|
Already told
|
153
|
31%
|
45.6
|
People who feel that they would not disclose their HSP to other people
they know may have a lower wellbeing that people who have or would disclose. Those
who do not use mobility aids are less likely to disclose and those who use
wheelchairs all or most of the time are more likely to disclose than others. Differences
between location and age are small.
People you don’t know
This question was
relevant for 416 people who answered. Around a third of people said they would
not disclose their HSP to other people they did not know, with just over half
saying they would disclose and less than 10% saying they already have
disclosed.
Table 54 – Disclose HSP to people you don’t know
Disclosure
|
Respondents
|
Percentage
|
Wellbeing
|
Yes
|
234
|
56%
|
46.7
|
No
|
148
|
36%
|
43.6
|
Already told
|
34
|
8%
|
45.2
|
There are no difference in wellbeing for people in respect of disclosing
their HSP to people they do not know. Those who do not use mobility aids are
less likely to disclose and those who use wheelchairs all or most of the time
are more likely to disclose than others. Differences between location and age
are small.
Comments
The numbers of comments made around disclosure generally reflect that
more people are able to disclose than do not want to disclose. The following
table gives some of the comments made highlighting some of the issues and
benefits from disclosure. These comments are in the respondents’ own words (or
Googles translation of them into English), with occasional minor adjustments
for clarity or anonymity.
Disclosure comments
|
I
find it difficult to talk about HSP when you don't know the people well.
Having just started a new job in a different city, it is very difficult to
talk about HSP to my new employer because I am still in a trial period. This
puts me in a difficult situation because I am undergoing a lot of stress and
have to do many long trips but cannot disclose my health situation in case it
scares the employer and they decide not to keep me.
|
May
be slightly reticent to disclose to potential parent in laws given the
hereditary nature and the possibility of passing on to offspring
|
It
makes me feel better to be open about it and freely discuss instead of
keeping it a secret
|
The
name HSP prevents me from telling other people about my HSP but I understand
certain people may need to know
|
I
can disclose my HSP to people who are of positive mind.
|
I
welcome strangers asking me what is ‘wrong’ with me. I prefer to be asked
directly rather than people talking behind my back. It gives me a chance to talk about HSP and
raise awareness - though I find it is more often children that ask rather
than adults.
|
Nobody
ever really asks
|
It's
generally uncomfortable to bring up the subject of HSP, but I do tell people
to explain why I fall down and walk into walls!
|
People
are very helpful when they realise I'm not drunk but disabled
|
It’s
harder to explain than people realize
|
I
am still struggling with being open about having HSP, even to my family
members. Since I don't live in my home country I could get away with not
telling so far, but I feel I will need to do this sometime soon as I started
getting questions about the way I walk. I also noticed that being open about
it to people helps me deal with it mentally, perhaps as it normalises it.
|
Disclosure
of HSP is not always good. Negative the points of attack are then known.
Bullying is the order of the day
|
It
is important that you "come out". Otherwise you will be
pigeonholed, how lazy, comfortable, boring, uninterested, ...
|
Disclosure summary
More people would or have disclosed their HSP than would not. Those who
would not disclose to the people they work with or to people who they know tend
to have a lower wellbeing.
13) Wellbeing
Conclusions
Throughout this report
the factors shown to have the greatest association with people’s wellbeing have
been identified. The tables below draw together the factors which have the strongest
associations with people’s wellbeing, either positive or negative. The number
of respondents given is the total number which have or don’t have the factor,
irrespective of if they gave a wellbeing score.
The
HSP symptoms covered in this survey with greatest potential impact on wellbeing
are pain, walking distance and mobility aids. As these factors change over time
there can be decreases in wellbeing. People can review their situation with
their neurologist, physiotherapist or doctor to ensure their exercise routines,
medications, treatments and mobility aids are appropriate for their stage of
HSP, and be confident that they are managing their changes over time as well as
they can.
Within
the HSP sphere other potential areas where changes could be made include:
Techniques to help manage how HSP symptoms vary during the menstrual cycleReviewing factors affecting depressionReviewing how things to do with HSP are fundedPositive Wellbeing Effect
Table 60 – Positive Wellbeing Factors
Factor
|
Respondents
|
Wellbeing
|
No pain from HSP, no mobility aids
|
20
|
55.5
|
+9.9
|
Not disabled, no walking aids
|
24
|
54.9
|
+9.3
|
Can stand up 'normally'
|
51
|
53.4
|
+7.8
|
HSP not affecting ability to work
|
77
|
52.7
|
+7.1
|
Not consider yourself disabled
|
80
|
51.5
|
+5.9
|
No depression (PHQ score 0-1)
|
242
|
51.1
|
+5.5
|
Not using mobility aids
|
70
|
51.0
|
+5.4
|
Not disabled, use aids some of the time
|
24
|
50.7
|
+5.1
|
Living in the Netherlands
|
43
|
50.5
|
+4.9
|
Pain from HSP but pain intensity = "no pain"
|
34
|
|