A journey to HSP/FSP

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 independence

reducing their pain or fatigue,

helping with their balance or reducing their falls

being 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 2^nd and 3^rd 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 cycle

Reviewing factors affecting depression

Reviewing how things to do with HSP are funded

Positive 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

A journey to HSP/FSP

Pages

Sunday, 19 March 2023

Update on Patient Journey

Tuesday, 28 February 2023

2022 Survey Results