2023 Survey - Highlights of 10 years of results

Rare disease day 2024 is here, and I am very pleased to publish the highlights from all ten years of surveys. 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/1Yr9-UbbS3QNJwpZIAWIF_aamEoCqLP4o/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!

HSP Survey Highlights. Views of 1,740 People with Hereditary Spastic Paraplegia (HSP) who answered ten on-line surveys 2013-2022, linking Wellbeing, Mobility and Life with HSP.

This paper presents the highlights of all ten surveys undertaken 2013 to 2022, and reported on rare disease days 2014 to 2023. The full details of each survey can be found on my blog.

Overall, there have been 1,740 people who completed at least one survey, predominantly from the USA, UK, Brasil, France and Australia. Of these people, 530 people (30%) have answered more than one survey, and 1,210 (70%) people have answered one survey. In total there are 2,784 answers across all surveys. Table 3 shows the numbers of people completing more than one survey.

Table 3 – Answers from respondents, highlighting those completing more than one survey

No. Surveys?	Total	2013	2014	2015	2016	2017	2018	2019	2020	2021	2022
More than one	1577	47	53	56	115	144	191	246	219	272	234
One	1207	74	44	53	54	78	113	121	98	293	279
Total answers	2784	121	97	109	169	222	304	367	317	565	513

2) Contents and Survey Questions

The following table shows the sections in this report and the year which that topic was dealt with. Some sections have been removed from this blog version, shown in italics. This version includes shorter versions of results. In all cases further information can be found in the full PDF of this writeup, and also in the survey results of that year.

Table 1 – Contents of this report

Section	Topic	Year(s)
1	Introduction (Full report)	-
2	Contents and Survey Questions	-
3	Respondents (Full report)	-
4	HSP Analysis and misdiagnosis	Every year
5	Wellbeing	2017 to 2022
6	Mobility analysis	Every year
7	Change in mobility and Disability (full report)	All years
8	Symptoms	2013 and 2018
9	Medication and Surgery	2014, 2019, 2020
10	Pain	2017 and 2022
11	Bladder	2016 and 2021
12	Bowel issues	2016
13	Fatigue	2016
14	Depression and Acceptance	2015, 2017, 2021, 2022
15	Modifications around the home	2015
16	Walking, Trips and Falls	2017 and 2021
17	Exercise and Relaxation	2014 and 2019
18	Menstrual Health	2022
19	Sleep	2018
20	HSP Research	2020 and 2021
21	Life with HSP	2019 to 2022
22	Needs	2020
23	Employment (Full report)	2017 and 2022
24	Wellbeing Conclusions	2017 to 2022
25	Links to surveys and outputs (Full report)	-
26	Acknowledgements	-

4) HSP Analysis and Misdiagnosis

In each survey the first questions ask if people had HSP and the method of their diagnosis. Overall, 921 (53%) said that they knew which type of HSP they had. HSP types with at least ten people reporting a diagnosis are shown, along with the gene which is mutated in that type of HSP. 47 different types of HSP have been identified by the respondents.

The most common answer is type 4 – SPG4 (indicating a mutation in the Spast gene). There are more than 50 people indicating either SPG7 (a mutation in the paraplegin gene) or SPG11 (a mutation in the spatacsin gene). There is a small number of people who note that they are affected by two or three HSP genes.

5) Wellbeing (2017-2022)

In order to assess the wellbeing of people with HSP, respondents since 2017 have completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS). The table below shows the average wellbeing score from year to year, and the number of respondents answering in each year. Over the six years the average score has ranged between 45.6 and 47.4 except for 2020 when the average was 42.9. It is presumed that the drop in 2020 was as a result of the various lockdowns and other restrictions in place due to coronavirus/Covid-19.  Most respondents have a wellbeing in line with average scores for the UK population.

6) Mobility Analysis

Around half of respondents use walking sticks/poles/crutches/ canes, two fifths use wheelchairs/mobility scoters and three out of ten use walking frames/rollators. The distribution of mobility aid use has remained broadly similar from year to year. Table 9 shows the aggregated total results and the percentage of respondents. These add to more than 100% as some people use more than one type of mobility aid.

Table 11 – Use of mobility aids

Mobility Aids Used - Summary:	Respondents	Percentage
Using sticks/poles/crutches/canes	1439	52%
Using Wheelchair/Mobility Scooter	1145	41%
Using Walking Frame/Rollator	789	28%
Using Orthotics/AFO/Insoles	704	25%
Using FES	113	4%

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 12 – Overview of mobility aids used

Mobility Aids Used - Overview:	Respondents	Percentage	Mobility Score
Those without aids	482	17%	0-1
Those who use mobility aids some of the time	592	21%	2-3
Those who use sticks most/all of the time	867	31%	4-5
Those who use frames most/all of the time	356	13%	6-7
Those who use chairs most/all of the time	482	17%	8-9

Overall, the distribution of these results from year to year are similar. For example, in each year between a quarter and a third of people use sticks all or most of the time.  When looking at wellbeing, the average scores for each of these categories is usually within 3 points of the average. However, those without mobility aids have higher wellbeing, and those who use frames or chairs most or all of the time have the lowest wellbeing.

Favourite mobility aids (2022)

In 2022 respondents were asked what their favourite mobility aids were. There were 386 people who gave an answer. There were 11 answers given by at least 10 people, as shown:

 Table 13 – 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

Disability (2017 to 2022)

In each year since 2017 people answered the question “Do you consider yourself to be disabled?”. Around 80-85% of people with HSP consider themselves to be disabled. The proportion of people who identify as disabled increases with reduced mobility, however 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 mobility is not the only factor in individuals deciding if they are disabled or not. In 2022 respondents described the point at which they have or would describe themselves as disabled, with responses grouped into different categories, shopwing those mentioned by more than 20 people:

Table 18 – Point of considering self as disabled

Disabled point	People
On diagnosis	18
With difficulty walking	64
Needing to use mobility aids, or needing mobility aids all the time	74
When unable to do certain activities	46
Needing help from other people, or needing more help from others	27

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 falling or difficulties walking). 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.

8) Symptoms (2013 and 2018)

The 2013 survey asked people to identify how much each of 13 different HSP symptoms affected them. For the 2018 survey the list was expanded to 36 symptoms, and people were asked how each affects them.

Table 19 – Answers and Symptom Grouping

Answer	Grouping	Factor
“Do not have”	Do not have	1
“Occasional symptom” or “Minor symptom”	Minor symptom	2
“Frequent symptom” or “Regular symptom”	Moderate symptom	3
“Most of the time” or “All of the time”	Major symptom	4 or 5

Table 14 lists symptoms from the 2018 questionnaire which are major for more than 10% of people, ordered by how people are affected by them.

Table 20 – Symptom Impact

Symptom	Major	Moderate	Minor	Don’t have
Difficulty running	86%	5%	9%	1%
Difficulty walking	70%	16%	13%	0.3%
Difficulty using stairs	63%	17%	18%	2%
My muscles are stiff	60%	21%	18%	1%
Loss of balance	55%	24%	19%	2%
Get more stiff when it is cold	55%	19%	20%	5%
My muscles are weak	51%	25%	20%	4%
Fatigue	40%	28%	24%	8%
Affected by bladder problems	37%	19%	23%	22%
Back/hip pain	32%	23%	28%	17%
Leg/foot pain	33%	21%	26%	20%
Clonus (jumping feet or other muscle spasms)	24%	29%	33%	13%
Stress	23%	29%	30%	18%
Regular falls	16%	26%	45%	12%
Poor co-ordination	23%	20%	35%	22%
Pes cavus (arched/high feet)	27%	8%	15%	50%
Depression	15%	21%	35%	29%
Hammer toes (toes curl under)	24%	11%	15%	51%
Affected by bowel problems	18%	16%	24%	41%
Loss of vibration sensitivity in legs	18%	14%	27%	41%
Numbness	13%	20%	33%	34%
HSP affecting sexual function	16%	13%	22%	49%
Feet swell up	14%	13%	29%	44%
Get more stiff when it is hot	11%	14%	32%	43%
HSP affecting learning or memory	12%	12%	26%	50%
Fail to remember dreams	14%	9%	22%	55%

 

There are people who are affected all or most of the time by every one of the symptoms listed (i.e. a major impact). The first five symptoms (loss of balance, muscles being stiff and difficulty running, walking, using stairs) relate to mobility and affect almost everybody. There are people who do not have all of the other symptoms.

Symptom with the Greatest Effect (2018)

Respondents described in a free text answer which symptoms gave the greatest effect or had the biggest impact. 283 respondents answered this question. Some described one or two symptoms and a small number listed many. There were 617 symptoms identified, and the 7 reported by at least 25 people are shown

Table 21 – Greatest Effect Symptom

Symptom with greatest effect	Respondents
Fatigue	99
Difficulty walking	64
Loss of balance	53
My muscles are stiff	41
Affected by bladder problems	40
Back/hip pain	39
Stress	29

This table shows that fatigue is the symptom which has the greatest effect for the greatest number of respondents. Difficulty walking and loss of balance affect a smaller number of people, but still more than 50.

Symptom with Greatest Effect on Wellbeing (2018)

The majority of respondents answered both symptom questions and wellbeing questions. This allows an examination of which symptoms have the greatest effect on wellbeing. The assessment has been limited to symptoms where at least 30 respondents had given wellbeing scores across the impact categories. Depression and stress have the greatest effect on wellbeing. For both of these symptoms, those that have it all or most of the time have a significantly lower wellbeing score, and those that don’t have it have a significantly higher wellbeing score.

9) Medication (2014 and 2019) and Surgery (2020)

In 2019 the survey asked questions about the different medication that people take for their HSP and its symptoms. In total 320 respondents answered this question. There were 274 people who indicated that they took at least one medication and 46 respondents who indicated that they did not take any medication at all. This indicates that around 85% of people are prescribed at least one form of medication for their HSP, and this proportion is slightly higher than reported in 2014. Those who do not take medication either have not started taking medication at all or have stopped taking medicine, either because they do not like the side effects of the medicine or because they are not noticing any benefits of the medicine.  The following grid shows the distribution of the number of medicines against mobility.

Table 24 – Number of respondents, number of medicines against mobility.

Mobility Score	No medicine	1 Medicine	2 Medicines	3 Medicines	4 Medicines	5 Medicines	6 Medicines	Total
0-1	16	26	12	0	2	1	1	58
2-3	14	28	19	9	2	5	1	78
4-5	10	35	22	8	8	2	0	85
6-7	4	9	10	5	2	5	1	36
8-9	2	16	19	15	3	6	2	63
Total	46	114	82	37	17	19	5	320
Wellbeing	+3.2	+1.5	-0.6	-2.7	(-3.2)	(-2.6)	-

This shows that generally those who do not take medication are the most mobile although there are several whose mobility is not affected who take several medications. For those whose mobility is affected by HSP there is a reasonable spread of the numbers of medication taken across the mobility bands. The most common number of medicines taken is 1 or 2.  Those who do not take medicine have a better wellbeing than those who take the larger number of medicines.

The medicines fall into six general groups, with a number of other medications grouped together in a seventh group. The group are:

Table 25 – Medication Groups

Symptom	Number of medicines	Number of people
Spasticity/spasms	21	327
Pain/nerve pain	33	109
Bladder	12	64
Depression/anxiety	10	17
Bowel	3	8
Sleep/fatigue	2	2
Other	19	23
Total	100	550

The table shows that there are more people taking medicine for spasticity or spasms than in the other groups, whereas there is a higher number of types of medicine used to treat pain or nerve pain. Baclofen is by far the most commonly used medication for HSP with around half of people taking it, and accounting for around one third of all medication taken.

The table below shows all of the medications being taken by more than 5 pepole for spasticity and spasms for HSP and selected medication from other areas. The table reports if the medication is noted as being used to treat HSP by reference to HSP support/research group websites and other similar places. The table also identifies if the medicine has been reported in the literature as being used to treat HSP, by search of the PubMed database.

Table 29 – HSP Treatment Review

Medication	People taking medication	HSP Treatment? (HSP groups)	HSP Treatment? (PubMed)
Baclofen	178	Yes – SPF	Yes, many
Botulinum toxin A / Botox	26	Yes – SPF	Yes, many
Gabapentin	24	Yes – SPF	Trial: 17539946
Clonazepam	23	Yes – SPF	No
Oxybutynin	22	Yes – Gene Review	No
Tizanidine / Zanaflex	21	Yes – SPF	Trial: 7035623
Pregabalin	14	Yes -Review	No
Diazepam	12	Yes – SPF	Patient: 2803825
Amitriptyline	11	Yes - SPF	No
Mirabegron	11	Yes – Gene Review	No
Solifenacin	10	Yes – Gene Review	No
Dantrolene	9	Yes - SPF	Review: 20862796
Duloxetine	9	Yes - PLM	No
Ziclague	7	Yes - ASPEH	No
Pramipexole	7	Yes - HSPRF	No

This table shows that there is no consistent source of information for the treatment of HSP symptoms, and to find particular results often requires a lot of hunting around. Several of the more commonly used medications do not have HSP specific trial results published, which may prevent their consideration by medical practitioners who are not so familiar with HSP. There may also be regional variations in availability of or guidelines for prescribing certain medicines, which has not been looked at.

Some of the treatments being tried by individuals are not reported at all, and this shows that some practitioners are being innovative and trying out different medications.

9F) Have you had Surgery for HSP? (2020)

Approximately 300 respondents answered this question in 2020. The table below shows that around 20% of people with HSP have had surgery for their HSP and 80% have not. Around one in 6 have had one type of surgery, and a small number have had more than one type of surgery. Surgery is more common in the USA (27% having had surgery) and Australia (25% having had surgery). Surgery is least common in Brasil and Canada (88% not having had surgery in each country).

Looking at the specific types of surgery, tendon release surgery is the most common surgery for those with HSP, followed by implanting a baclofen pump. Other surgeries mentioned included several which may not be related to HSP. Those mentioned include cataracts surgery, hip or knee replacement, foot reconstruction/toe re-positioning, and gastric sleeves (for weight loss leading to easier mobility).

Table 31 – Have you had surgery?

Have you had surgery?	Yes	No
Tendon release	30 (10%)	271
Baclofen Pump	22 (7%)	278
Muscle release	11 (4%)	283
Selective dorsal rhizotomy (SDR)	3 (1%)	291
Other	13 (5%)	248

10) Pain (2017 and 2022)

Pain from HSP

The 2022 survey 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 32 – Pain from HSP

Pain from HSP?	Yes	No	Wellbeing Yes	Wellbeing No
All	380 (76%)	123	-1.0	+3.2
Mobility 0-1	48 (71%)	20	+3.6	+9.9
Mobility 2-3	71 (76%)	22	-1.1	+3.6
Mobility 4-5	141 (77%)	42	-1.3	+1.9
Mobility 6-7	46 (74%)	16	-3.7	(+0.3)
Mobility 8-9	74 (76%)	23	-1.4	+2.1

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.

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. The 22 items are also grouped into four sub-categories representing continuous pain, intermittent pain, neuropathic pain, and affective descriptors of pain. All scores are out of 10.

Table 33 – 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

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 do not get pain from their HSP score an overall average of 0.37, with higher scores for affective pain descriptors and neuropathic pain.

Pain descriptors

The following table shows the average score for the 6 highest scoring 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 34 – 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%

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.

Pain location

Respondents used pain intensity descriptors for different areas of the body to identify where people perceive their pain to occur. 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.

11) Bladder (2016 and 2021)

Respondents in 2021 and 2016 were asked details about urinating. Around 550 people answered these questions in 2021, with some people not answering all of these questions. Respondents selected an answer from a pick-list for each question. The answers are scored. In the analysis a score of 3 is used to mark the onset of overactive bladder effects, and the following ranges are used:

• 3-6 – mild effects – things happen “occasionally”
• 7-10 – moderate effects – things happen “some of the time”
• 11 or more – severe effects – things happen “all” of “most” of the time.

Table 36 – Overactive Bladder Score Results

Overactive Bladder Effect Bands	2021 Respondents		2021 Wellbeing	2016 respondents
All respondents	544	-	-	165
0-2: No effects	96	18%	+2.0	34   (21%)
3-6: Mild effects	230	42%	+0.8	73   (44%)
7-10: Moderate effects	171	31%	-0.7	43   (26%)
11+: Severe effects	47	9%	-3.1	15   (9%)

This table shows that in 2021 more than 80% of people with HSP have some degree of bladder issues. The majority (42%) of people with bladder effects have mild effects, with less than one in ten (9%) having severe effects. Wellbeing scores decrease with increasing bladder effects. The distribution of people in these four categories is similar in 2016 and 2021.

In both years those who use mobility aids all or most of the time have a slightly higher score than those who do not use aids or who use them some of the time. The following bullets are a summary of the individual questions from the 2021 results.

• Most people (70%) go to the toilet 8 times a day or less.
• Most people (65%) do not go to the toilet during the night or go once.
• Most people (57%) have to rush to the toilet sometimes or most of the time.
• Most people (62%) have urine leaking before they get to the toilet occasionally or sometimes.
• Most people (59%) have their bladder empty completely most of the time or all of the time.

There is a trend showing lower wellbeing for those with increased bladder effects. The biggest issue to affect wellbeing is having to rush to the toilet, those who have to do this all of the time have the lowest wellbeing, and those who do not have to rush have the highest wellbeing. Those who have urine leaking before they get to the toilet all or most of the time also have lower wellbeing.

The survey in 2021 sought to identify how people manage their bladder problems. People were given a picklist of eight options to identify if and/or how they manage their bladder problems, with the top answers shown.

Table 38 – Bladder problem management

Main method for managing bladder problems	Respondents		Wellbeing
Do not have bladder problems	90	17%	+2.8
Take no action to manage	133	25%	0
Medication to relieve spasticity	126	23%	-2.0
Drink less fluids	85	16%	-2.3

Most people (59%) use at least one of the methods noted. The most common method used is taking medication to relieve spasticity in the bladder, followed by deliberately drinking less fluids during the day. 

Around 80% of people with HSP have some degree of bladder issues. Bladder issues can affect people who do not use mobility aids just as there are some who use mobility aids all or most of the time who do not have any bladder issues. Those with SPG7 tend to be more affected by bladder issues than others.

The majority of people with bladder issues have mild effects, with less than one in ten having severe effects. Needing to rush to the toilet and having urine leak before getting to the toilet are common issues. Most people feel their bladder does not empty fully at least some of the time. A range of techniques are used to manage bladder issues for people with HSP, with the most common ones being taking anti-spasticity medication and drinking less fluids.

12) Bowel Issues (2016)

The 2016 survey asked nine questions from the ICIQ bowel survey. The answers are scored giving a total range for these nine questions between 2 and 37. These questions were answered by 155 respondents. The following table gives a summary of the results;

Table 39 – Bowel Results

Mobility Score	Respondents	Minimum Score	Average Score	Maximum Score
Overall	155	2	9.5	20

There are people in each mobility band who have no symptoms or minor symptoms, and there are people within each mobility band who have several symptoms. The minimum and average scores both generally increase as mobility issues increase. For this analysis I am grouping bowel scores as follows;

·          5-11 = mild effects – things occur “rarely”

·          12-20 = moderate effects – things occur “some of the time”

·          At least 21 = severe effects – things occur “most” or “all” of the time 

Table 40 – Bowel Severity

Mobility Score	Respondents	No effects	Mild effects	Moderate effects	Severe effects
0-1	28	5 (18%)	15 (54%)	8 (29%)	0 (0%)
2-3	39	3 (8%)	29 (74%)	7 (18%)	0 (0%)
4-5	51	2 (4%)	32 (63%)	17 (33%)	0 (0%)
6-7	11	0 (0%)	8 (73%)	3 (27%)	0 (0%)
8-9	26	0 (0%)	12 (46%)	14 (54%)	0 (0%)
Overall	155	10 (6%)	96 (62%)	49 (32%)	0 (0%)

This shows that around three fifths of people with HSP have mild bowel effects, and around one third have moderate effects. A few people have no effects at all, and no people showed severe effects. There is a general trend that the milder effects tend to be with those with lower mobility issues, and the effects tend to be more severe with those with higher mobility issues.

Urgency and Hesitancy

The data shows that about a sixth of people with HSP have to rush to the toilet most or all of the time, with a further third of people having to rush sometimes. The data also shows that a third of people with HSP have to strain to open their bowels most or all of the time, with a further third of people having to strain sometimes. There is no clear pattern on how urgency varies with mobility.  There are about a quarter of respondents wear pads or stain their underwear more than once a month. 58% of respondents do not have this problem, and 19% have this less than once a month.

13) Fatigue (2016)

Fatigue is often noted as a symptom of HSP. The 2016 survey asked respondents to complete the Modified Fatigue Impact Scale, the Neurological Fatigue Index (Multiple Sclerosis) and the Brief Fatigue Inventory.

Neurological Fatigue Index - MS

The three statements which the most people agreed with were “If I've overdone things, I know about it the next day” (81% agreeing), “Resting allows me to carry on” (79% agreeing) and “I soon become weak after physical effort” (78% agreeing).

Modified Fatigue Impact Scale 5

The physical questions make the greatest contribution to the fatigue score across all levels of mobility, scoring slightly more than the cognitive questions. From this, we can see that both physical and cognitive factors play a part in the fatigue of people with HSP.

Brief Fatigue Inventory

Following interpretations of this scale by others for cancer, this analysis uses scores less than 3 as mild, between 3 and 7 as moderate and greater than 7 as severe. Overall, 11% have mild fatigue, 62% have moderate fatigue and 27% have severe fatigue. Therefore, around 90% of people with HSP have moderate or severe fatigue.

Fatigue Summary

Fatigue is generally independent of mobility. Respondents who do not use mobility aids tended to score slightly lower fatigue levels than those who use mobility aids all or most of the time, but there is a wide spread of data, and there are some who do not use mobility aids who have higher levels of fatigue than those who use mobility aids all the time 

14) Depression (2015, 2017, 2021 and 2022) and Acceptance (2021)

Depression (2015, 2017, 2021 and 2022)

Respondents have answered the two question PHQ2 screening tool for depression in 2015, 2017, 2021 and 2022. Answers are scored, giving a total score between 0 and 6. My analysis in 2021 indicated that a cut-on point of 3 was likely to be appropriate for those with HSP, based on wellbeing scores.

Around one quarter of respondents scoring 3-6 should be screened further for depression and three quarters scoring 0-2 should not. This pattern is consistent from year to year. Those who score at least three points have a wellbeing which is significantly below average.

Several respondents have answered the PHQ2 questions in more than one survey. The analysis in 2022 examined this, looking at people who scored at least three points in some years and not in other years, indicating that their depression has changed from year to year.

Whilst the proportion of people screening for depression is fairly constant, for some people their score has improved, for some it has gotten worse, and for some their score varies from year to year. This indicates that change in depression may be principally related to factors other than HSP.

Acceptance (2021)

Part of the journey with a progressive condition like HSP is accepting that you have HSP. Respondents filled out the 8 question Acceptance of Illness Scale (AIS) in 2021 which is tool to measure acceptance of any disease. Each answer is scored with total score ranging between 8 and 40.

A low score indicates that the person does not accept their illness whereas a high score indicates greater acceptance. For this analysis scores of 30 points and above are considered to be good acceptance, scores between 20 and 30 points are considered moderate acceptance, and scores below 20 are considered low acceptance.

There were 511 answers to these questions, and the overall average score is 21.7, which is around the point between low and moderate acceptance.  There were 40% of people with low acceptance, 44% with moderate acceptance and 16% with good acceptance. Acceptance of illness is lower when mobility aids are used all or most of the time, and is higher when mobility aids are not used.

There is a strong link between acceptance and wellbeing. Those who have a low level of acceptance have a wellbeing score 7.6 points below the average and those that have a high acceptance have a wellbeing score 9.9 points higher than the average.

15) Modifications Around The Home (2015)

In 2015 I wanted to find out what types of modifications people had made around the home and asked a set of questions about modifications in different areas of the home. Overall, there were 99 respondents who answered these questions. An overview of the data is presented below, noting that respondents may appear in more than one of these categories:

Table 50 – Overview of Modification Data

Situation	Mobility 0-1	Mobility 2-3	Mobility 4-5	Mobility 6-7	Mobility 8-9	Total Answers
Total number of respondents	20	18	24	21	16	99
Zero modifications made	10	6	8	6	1	31
Furniture moved within the property	3	0	2	1	0	6
Have moved to a single storey property	5	2	5	5	7	24
Live on one floor within their property	1	2	0	1	1	5

There were 31 respondents who indicated that they had made no modifications to their properties. These fell into two general groups;

• those that had not made modifications yet, and
• those who didn’t need to make modifications because they had moved into an accessible property which meets their needs.

There were 22 different types of modifications which were mentioned in free-text answers by more than five respondents, as shown in the following table.

Table 51 –Modifications by more than 5 people

Modification	Mobility 0-1	Mobility 2-3	Mobility 4-5	Mobility 6-7	Mobility 8-9	Total Answers
Total number of respondents	20	18	24	21	16	99
Grab rails (all data, in any location)	3	7	8	13	8	39
Ramps (external or internal)	1	2	5	4	5	17
Grab rails (within the shower or bath)	2	5	3	3	1	14
Accessible/raised toilet	2	2	2	2	4	12
Stair lift	0	3	0	3	5	11
Bath seat/shower seat/bath board	2	1	2	2	4	11
Conversion of bathroom to wetroom	2	1	0	3	4	10
Hospital/power/electric/adjustable bed	0	0	1	2	4	7
Widening of doorways	0	0	0	4	1	5
Making the level of the bed lower	2	0	1	2	1	6
Modifications to the kitchen	1	0	1	3	1	6

 

Grab Rails

Grab rails are the most common modifications that are made around homes, and are present in some homes at all levels of mobility. These tend to be the modifications which are made first. The majority of grab rails are installed in bathrooms/toilets although respondents also included them by doors, in bedrooms, kitchens, hallways, garages and other rooms.

Ramps

Ramps are also a common modification, again made by people at all levels of mobility. There are two general types of ramps mentioned, the larger scale purpose built external ramp used for access to the property, and smaller portable ramps which may be for use either outside or inside the property.

Raised/Accessible Toilets

This modification includes toilets that were described either as raised or accessible and has been made across the full range of mobility. Generally this was described as making it easier to stand up/sit down from the toilet and was installed because people were finding it difficult to do so.

Stair lift

The stair lift tends to have been installed by respondents who rely more frequently on mobility aids, although a few respondents have had one installed earlier. Stair lifts are reported as giving access to otherwise inaccessible parts of the property or, installed because it makes access easier to parts of the property by people who have difficulties getting up or down stairs.

Conversion of bathroom to wetroom, or conversion of bath to shower.

The wetroom modification has been made by a number of respondents across the range of mobility. he main reason for making this modification is enabling the respondent to shower because getting in/out of the bath has become difficult or impossible.

16) Walking (2017), Trips and Falls (2021)

Perhaps the most common symptom of HSP is impairment of walking. There are plenty of studies which evaluate walking speed and distance, evaluate gait patterns and muscle problems. The majority of the papers I have seen evaluate walking in a clinical environment. In 2017 respondents selected from pick-lists how much and how often different factors affected them.

Walking Factor Assessment (2017)

For each factor respondents scored how much and how often it affects them. The table below shows the percentage affected by each factor split by maximum walking distance. The “answers” column indicates the total number of people who scored the factor. The highest percentages in each column are highlighted 

Table 52 – Factors Affecting Walking

Factor Group	Factor	Answers	As far as I want to	Exhausted beyond 500m	Up to 500m	Up to 10m
Surface	Down slopes	157	71%	98%	100%	100%
Surface	Up Slopes	155	75%	97%	90%	100%
Surface	Round tight bends/corners	154	59%	84%	82%	97%
Surface	Over smooth ground	153	56%	81%	68%	88%
Surface	Over uneven ground	152	89%	97%	100%	94%
Surface	Where steps/stairs are involved	154	81%	98%	100%	100%
Activity	Talking/chatting	153	65%	62%	61%	82%
Activity	Carrying something	153	89%	97%	96%	94%
Activity	Using the phone	151	63%	58%	48%	73%
Activity	Concentrating	149	42%	76%	67%	81%
Activity	Managing children!	141	52%	86%	73%	72%
Factor	Being in a rush	149	92%	95%	100%	91%
Factor	Need to go to the toilet	154	79%	92%	89%	100%
Factor	Stressed	148	70%	92%	96%	91%
Factor	Upset/emotional	148	65%	92%	88%	88%
Factor	Tired/fatigued	151	89%	98%	100%	97%
Factor	Extra short term illness	137	58%	79%	72%	79%
Factor	Have had alcohol	136	78%	71%	84%	69%
Factor	Have had caffeine	136	29%	36%	52%	45%
Environment	It is a hot day	145	50%	71%	74%	79%
Environment	It is a cold day	151	77%	92%	96%	86%

When the “how much” and “how often” aspects of the question are combined, the analysis shows a similar result. Taking a broad view, the factors which affect people walking the most seem to be:

• Where stairs/steps are involved
• Tiredness/fatigue
• Going over uneven ground
• Carrying somethin 

Combinations of Walking Factors (2017)

The analysis also looked at walking factors in combination. The data shows that if people are affected by fatigue or tiredness, or they are in a rush, then walking is often more difficult. These factors are mentioned in combination with the widest number of factors.

Trips and Falls (2021)

Respondents in 2021 were asked about their frequency of trips and falls.  At least 520 people answered these questions. Over 90% of people with HSP have problems with balance, tripping up and falling, and that 80% of people with HSP scuff their feet. There are some trends:

• Over a quarter of those who scuff their feet tend to do this on a daily basis, with around another quarter doing so on a weekly or monthly basis.
• Almost a third of people who lose their balance with HSP do so on a weekly basis, with around a quarter doing so on a daily basis.
• Around half of people who trip up as a result of their HSP do so on a weekly or monthly basis, with one in five doing so on a daily basis.
•  Around a third of people have stopped themselves falling a few times, with around half of people needing to do this on a monthly or weekly basis.
• Over half of people have fallen down a few times, with around one in five people falling on a monthly basis. A small proportion fall down on a daily basis.

Those who fall down on a weekly basis have the lowest wellbeing, and this is a significant effect. All other wellbeing scores are within 3 points of the average.

17) Exercise and Relaxation (2019 and 2014)

The 2014 and 2019 surveys looked at exercise and relaxation routines. Overall 362 respondents answered this question in 2019, and 90 answered in 2014. The analysis reports the 2019 results, and these are very similar to the 2014 results.

The 2019 question set out 18 different activities, and people selected an answer from a picklist describing how often they do this. In the table “Dont” means the person selected “I don’t do this”, and “Multi-Per-Day” means the person selected “I do this several times a day”. Respondents who undertake activities weekly or more frequently are grouped together as undertaking that activity “regularly” in the last column, showing the percentage of respondents for that activity. The table reports activities undertaken regularly by more than 10% of people 

Table 55 – Exercise and Relaxation

	Multi-Per-Day	Daily	Weekly	Monthly	Occasionally	Dont	Total	Regular
Stretches	45	138	74	2	47	31	337	76%
Walking/Running	42	101	44	1	28	85	301	62%
Physiotherapy	7	25	119	14	48	93	306	49%
Music	44	46	28	12	34	103	267	44%
Cycling/Manual wheelchair	12	37	42	7	35	146	279	33%
Weights	4	22	39	6	31	167	269	24%
Other	12	8	18	7	14	105	164	23%
Swimming	5	9	45	7	65	152	283	21%
Cardiovascular gym machines	2	21	30	2	20	187	262	20%
Yoga/Pilates/Tai Chi	3	13	39	4	21	192	272	20%
Gardening	3	14	34	13	40	159	263	19%
Massage	6	8	29	16	42	170	271	16%
Sauna/Hot bath/Steam room	5	6	21	6	36	187	261	12%

 

This shows that stretches are the most common form of exercise being undertaken with over 90% of respondents doing this, and over 75% doing this regularly. Other activities which are commonly undertaken include walking/running, physiotherapy, music and cycling/manual wheelchair with more than one third doing regularly.

Most Important Activities (2019)

Respondents were asked to identify those activities which they felt were most important for exercise, relaxing and flexibility. The top three activities important for exercise are: stretches, walking and aerobic activities (e.g. gym machines, workouts). The top three for relaxing are: music, stretches and massage. The top three for flexibility are: stretches, physiotherapy and Pilates. 

18) Menstrual Health (2022)

The 2022 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. There were between 180 and 185 people who answered these questions. The following table highlights the proportion of people who feel each symptom changes during their menstrual cycle.

Table 57 – Menstrual health summary

HSP Symptom	Proportion worse or much worse	Proportion about the same	Proportion better or much better
Spasticity	30%	70%	2%
Pain	36%	63%	1%
Fatigue	54%	45%	1%
Mental health	32%	64%	4%

For fatigue, the majority feel this gets worse or much worse, whereas for the other symptoms the majority feel that this does not change. There is little difference in the results across location and use of mobility aids. Those who feel their spasticity and fatigue symptoms get worse have a lower wellbeing, and there may be a significant reduction in wellbeing for those who feel their mental health gets much worse during their menstrual cycle. Several people find that their bladder and/or bowel are more affected during their cycle.

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.

19) Sleep (2018)

The 2018 survey sought to explore how HSP affects peoples’ sleeping patterns. Respondents answered questions from the Global Sleep Assessment Questionnaire (GSAQ) and questions capturing factors relevant to HSP. The questions identified 16 factors with the potential to affect sleep. Respondents selected an answer from a picklist best reflecting their situation. Where a factor affects a person ‘usually’ or ‘always’, it is taken that this affects their sleep quality. 290 respondents answered at least 14 of the questions, and are included in this analysis.

The majority of respondents (80%) are affected by at least one sleep factor. Approximately half of respondents (54%) are affected by 3 or more factors. Grouping the numbers of factors together in similar sized groups allows an analysis of wellbeing: 

Table 58 – Number of sleep factors

Number of sleep factors	Respondents	Wellbeing
0 or 1	92 (35%)	+4.5
2 or 3	73 (25%)	+2.2
4 or 5	47 (16%)	-2.7
6 or more	67 (23%)	-6.0

Those respondents affected by at least 6 factors (approximately one quarter of respondents) are shown to have a significantly lower wellbeing. Those who are affected by no factors or one factor, about a third, have a better wellbeing.

The data allows the sleep factors with the biggest effects to be identified. The table shows factors affecting more than 20% of people, sorted by the proportion of people affected 

Table 59 – Sleep Factor With Greatest Effect

Sleep Factor	Respondents
Did you have difficulty falling asleep, staying asleep, or feeling poorly rested in the morning?	48%
Did needing the toilet disturb you in your sleep?	38%
Did you have repeated rhythmic leg jerks or leg twitches during your sleep?	32%
Did you have restless or "crawling" feelings in your legs at night that went away if you moved your legs?	27%
Did you feel sad or anxious?	26%
Did pain disturb you in your sleep?	25%
Did sleep difficulties or daytime sleepiness interfere with your daily activities?	25%
Did you fall asleep unintentionally or have to fight to stay awake during the day?	23%

 

The factor with the greatest effect, affecting nearly half of respondents is having difficulty falling asleep, staying asleep or feeling poorly rested in the morning. Needing the toilet in the night affects more than a third of respondents and having repeated rhythmic jerks or twitches affects about a third of respondents.

20) HSP Research (2020 and 2021)

Research Priorities (2021)

Respondents in 2021 were asked where they felt research into HSP should be prioritised, and 316 respondents provided an answer. These answers have been grouped into categories, and those mentioned by at least 10 people are shown 

Table 60 – 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

Many of those seeking research into 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.

What would a cure for HSP look like? (2020)  

In 2020 people were asked what they thought a cure for HSP would look like by selecting an option. There were 312 respondents who answered this question, as shown in the following grid: 

Table 61 – What would a cure for HSP look like?

What would a cure for HSP look like?	Respondents
Something which stops the progression of my symptoms	91 (29%)
Something which reverses the symptoms I have	140 (45%)
Something which prevents the next generation from getting HSP	72 (23%)
Other	9 (3%)

This shows that the majority of people would consider that a cure for HSP would reverse the symptoms that they have. There was little variation in wellbeing between these answers. Comments around what a cure would look like generally rephrased one of the three options often with specific details added, or bought two of the options together.

Several went for one-word answers, my favourite of which was that a cure for HSP would be ‘wonderous’. One person expressed that a cure when they first started showing symptoms of HSP would have been reversal, but now their HSP is more advanced they would see a cure as just stopping progression. Another, referring to their adult child with complex HSP, observed that a cure to reverse damage would allow them to meet the adult their child they should have been. 

21) Life with HSP 2019 to 2022

Advice to others (2022)

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 62 – 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. 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.

Questions about life with HSP (2019 and 2020)

Several questions were used to investigate different HSP aspects, allowing people to give yes or no answers.

Table 63 – Life with HSP questions in 2019

Question	Yes	No	Wellbeing Yes	Wellbeing No
Do you consider yourself to be lonely?	116 (32%)	245	-7.9	+3.9
Do you consider yourself to be isolated?	107 (30%)	255	-8.4	+3.5
Do you consider yourself to be vulnerable?	195 (54%)	166	-2.9	+3.2
Have you suffered from abuse?	52 (14%)	307	-4.4	+0.8

One third of people with HSP consider themselves to be lonely and/or isolated. These tend to occur more often in those whose mobility is more restricted. There is a significant difference in wellbeing between those who are affected and those who are not.  

Around half of people with HSP consider themselves to be vulnerable. Vulnerability tends to be similar across mobility bands and in different parts of the world. People identified that they feel vulnerable for two main groups of reasons, one group is around the physical changes of HSP not allowing people to be able to defend themselves or resist attack, the other group is around the consequences of HSP symptoms, including balance problems, incontinence and speed/ease of movement in crowded situations.

Around one in seven people with HSP have suffered from abuse. Those with restricted mobility have suffered abuse more than those who are more mobile. Approximately three quarters of those who suffered from abuse indicated that this was as a result of their HSP, and these come from across the range of mobility scores. 

Table 64 – Life with HSP questions in 2020

HSP Description on diagnosis?	Yes	No	Wellbeing Yes	Wellbeing No
Did you get a description of HSP when diagnosed?	180 (59%)	124	+1.1	-1.4
Delayed/avoided seeking advice due to embarrassment?	48 (15%)	265	-7.3	+1.3
Extent or severity of symptoms not believed by medical professional?	173 (55%)	142	-1.9	+2.4
Inadequate/inappropriate treatment because a medical professional did not take you?	174 (56%)	139	-2.4	+3.1

Over half of respondents got a description on diagnosis, and those who did have a slightly better wellbeing. The proportion getting the description is similar across mobility aid usage and country.

About one in seven people with HSP have delayed or avoided seeking medical advice because they have felt embarrassed about their HSP. These people have a significantly lower wellbeing.

More than half of people with HSP feel that the extents or severity of their symptoms have not been belied by medical professionals. Those who feel this have a lower wellbeing than those who have had the extent/severity of their symptoms believed.

More than half of people with HSP feel that they have not received adequate or appropriate treatment because they have not been taken seriously by a medical professional. Those who feel this have a lower wellbeing than those who have been taken seriously.

Friendships (2021)

Respondents in 2021 were asked “Describe if you have found making or keeping friends difficult because of your HSP?” 408 people provided a free-text answer to this question. These answers have been grouped and categorised into yes or no answers. 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.

Comfortable discussing HSP issues with partner/family (2021)

There are 559 respondents who answered (in 2021): Do you feel comfortable discussing issues relating to your HSP with your partner/family? Results are shown in the following table 

Table 67 – Do you feel comfortable discussing issues relating to your HSP with your partner/family?

Comfortable discussing HSP issues with partner/family?	Respondents	Wellbeing
All	559	-
Yes, always	354 (63%)	+2.7
No, never	41 (7%)	-5.8
Sometimes	164 (29%)	-4.4

Around two thirds of people with HSP are comfortable discussing HSP issues with their partner or family. Less than 10% are not comfortable, with the remainder comfortable discussing some of the time. Those who are comfortable discussing have a higher wellbeing, and those who are not comfortable discussing have a significantly lower wellbeing 

Comfortable discussing your HSP in a new romantic relationship (2021)

There are 518 respondents who answered (in 2021): In a new romantic relationship, how comfortable would you feel discussing your HSP and its impact? Responses are shown in the following table.

Table 68 – Comfortable discussing your HSP and its impact in a new romantic relationship?

Comfortable discussing your HSP in new romantic relationship?	Respondents	Wellbeing
All	518	-
Uncomfortable, but I would discuss it	257 (50%)	-2.4
I would not discuss it	37 (7%)	-9.0
Comfortable, it’s not a problem	224 (43%)	+4.3

Around two in five with HSP would be comfortable discussing HSP issues in a new romantic relationship. Less than 10% are not comfortable and would not discuss. About half would discuss this, but would feel uncomfortable. Those who are comfortable discussing have a higher wellbeing, and those who are not comfortable discussing have a significantly lower wellbeing.

22) Needs (2020)

Questions were asked in 2020 to identify the needs of people with HSP, split into five topics. For each topic people selected their top 5 needs from a picklist for that topic. Picklists were derived from observing people’s stories and concerns over time, and the experiences of people with HSP from different areas.

For each topic below, a table reports the number of people choosing the need as their most important need (1^st rank), the total number of people choosing the need in any rank including first rank (total votes), the weighted score (score), and the weighted score expressed as a percentage of the highest scoring need (percent). The tables report any needs identified as 1^st choice by at least 10 people.

The background of the percent column has been shaded to show the relative importance of each, with the more important needs being shaded darker green. Similar scoring needs are grouped using light shading. The full report for this survey also includes a breakdown of needs identified by the people in each country.   

Living with HSP   

This topic included 20 needs.

Table 69 – Living with HSP needs

Living with HSP Needs	1st Rank	Total Votes	Score	Percent
Stretches and exercises that help	129	211	907	100%
Information on appropriate physical activities to take part in	34	135	500	55%
Information on tailoring physical activities to suit HSP	22	109	367	40%
Information on how best to walk	19	104	324	36%
Information on living with HSP & other long-term conditions	30	92	305	34%
Understanding if changes/new symptoms are to do with HSP	15	86	246	27%
Information on staying healthy with reduced mobility	12	86	242	27%

Diagnosis and Inheritance   

This topic included 11 needs:

Table 70 – Diagnosis and Inheritance needs

Diagnosis and Inheritance Needs	1st Rank	Total Votes	Score	Percent
Explanation of HSP on diagnosis	126	234	924	100%
Signposting about potential future needs	36	207	652	71%
Signposting about HSP information	21	144	433	47%
Reduced clinical diagnosis time	27	121	393	43%
Having genetic tests available for more types of HSP	29	120	360	39%
Understanding what a genetic diagnosis means	17	128	343	37%
Information on the likelihood of others in family having HSP	15	114	325	35%
Information on the likelihood of next generation having HSP	12	113	314	34%
Fewer clinical misdiagnoses	14	87	267	29%

Wellbeing  

This topic included 12 needs.

Table 71 – Wellbeing Needs

Wellbeing Needs	1st Rank	Total Votes	Score	Percent
Information on improving mental health/wellbeing	136	229	957	100%
Information on finding/accessing relevant healthcare professionals	45	188	614	64%
Information on obtaining benefits	22	165	512	54%
Information on communicating with healthcare professionals	30	143	473	49%
Understanding your rights	12	138	366	38%
Information on asking for adjustments at work	18	108	354	37%
Information on finding HSP patient communities	12	110	300	31%
Information on maintaining a good sleep routine	12	99	258	27%

Treatments   

This topic included 19 needs:

Table 72 – Treatment needs

Treatment Needs	1st Rank	Total Votes	Score	Percent
Treatment options for spasticity	146	219	953	100%
Treatment options for pain	32	141	510	54%
Treatment options for bladder issues	26	131	443	46%
Treatment options for fatigue	21	142	445	47%
Better information on existing treatments	24	139	418	44%

Information about HSP  

This topic included 9 needs:

Table 73 – Information about HSP needs

Information about HSP Needs	1st Rank	Total Votes	Score	Percent
Predicting how HSP will progress in individuals	76	228	838	100%
Understanding the burden of HSP symptoms	68	194	713	85%
Understanding what the affected genes do	71	170	595	71%
Understanding the day-to-day variation in HSP symptoms	16	194	515	61%
Predicting if other HSP symptoms will start over time	13	154	446	53%
Understanding the prevalence of HSP symptoms	26	130	428	51%
Setting up a patient registry for understanding HSP	21	123	326	39%

24) Wellbeing Conclusions

Since 2017 the effects of different factors of HSP have been highlighted in terms of wellbeing. Those effects with wellbeing higher or lower than the average for each year have been highlighted. The tables below draw together the factors reported since 2017 which have the strongest associations with people’s wellbeing, either positive or negative.

The tables show the year each effect was reported in, a description of the factor, the proportion of people who are affected by the factor, irrespective of if they gave a wellbeing score, and the difference from the average wellbeing of that year. The factors shown are all those affecting at least 20% of people in that year. Positive wellbeing effects are shown where the wellbeing is at least 3.5 points better than average, and negative effects are shown where the average is at least 5 points below average.

Positive Wellbeing Effect

Table 77 – Positive Wellbeing Factors

Year	Factor	Proportion	Wellbeing
2021	No depression (PHQ score 0-1)	42.8%	7.3
2018	Not suffering from depression	26.0%	6.8
2021	HSP not affecting future relationships	32.2%	5.5
2022	No depression (PHQ score 0-1)	47.2%	5.5
2018	Not suffering from poor-coordination	20.4%	4.6
2018	Good sleep (affected by up to 1 sleep factor)	32.9%	4.5
2021	My family understands my HSP - strongly agree	30.6%	4.4
2021	Comfortable discussing my HSP in a new relationship	39.6%	4.3
2021	Family thinks I exaggerate my HSP - strongly disagree	24.1%	3.9
2019	Not feeling lonely	66.8%	3.9
2021	No difficulty making friends	34.3%	3.7
2017	No depression (PHQ score 0-2)	22.1%	3.5
2018	Not having difficulty falling/staying asleep	49.3%	3.5
2019	Not feeling isolated	69.5%	3.5

 Negative Wellbeing Effect

Table 78 – Negative Wellbeing Factors

Year	Factor	Proportion	Wellbeing
2021	Screen for depression (PHQ score 3-6)	24.4%	-10.0
2022	Screen for depression (PHQ score 2-6)	48.5%	-9.4
2017	Screen for depression (PHQ score 3-6)	59.0%	-9.0
2018	Being sad/anxious interfering with sleep always/usually	24.3%	-8.6
2019	Feeling isolated	29.2%	-8.4
2019	Feeling lonely	31.6%	-7.9
2021	Low acceptance (AIS score <20)	35.8%	-7.6
2018	Suffering from stress all or most of the time	22.4%	-6.9
2018	Worries affecting sleep always/usually	20.7%	-6.1
2018	Poor sleep (Having 6+ sleep factors always/usually, sleep)	23.0%	-6.0
2020	Medical professionals not understanding your HSP at all	20.8%	-5.8
2021	HSP affected past relationships negatively	21.6%	-5.7
2021	HSP may affect future relationships negatively	29.0%	-5.5
2017	Not content with employment/occupation situation	31.1%	-5.5

The HSP symptoms with greatest potential impact on wellbeing are pain, bladder, co-ordination and learning/memory. These each affect more than 20% of people. As these factors can change over time there may be decreases in wellbeing over time. 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 areas affecting at least 20% of people, where changes could be investigated include:

• Reviewing factors affecting depression
• Reviewing factors affecting sleep
• Reviewing factors affecting stress
• Frequency of seeing specialists, and getting appropriate treatments

Those who have low acceptance of their HSP have significantly lower wellbeing and those with good acceptance have significantly higher wellbeing. Similarly those who understand HSP well have a higher wellbeing than those with a limited knowledge of HSP. People could consider counselling for changing their acceptance, and could pro-actively seek to understand more about HSP.

The Importance of Wellbeing

he area with the greatest number of positive and negative effects is the social/family/relationships area. There are two key aspects covering the greatest wellbeing changes within relationships:

• Many questions are around people feeling that their family or partners do not understand their experiences with HSP, sometimes making assumptions about HSP which don’t reflect peoples’ own perceptions.
• Secondly, people are sometimes not comfortable discussing their situations with important people.

Overall, there are factors to do with feelings, relationships, comments and opinions. Conversations around these topics can be difficult, and people could consider discussing these issue with people they care about to help their burden (provided they feel safe to do so). These conversations can help everyone improve their understanding, recognise that situations change, and allow honesty about concerns, difficulties and feelings. For some, those conversations may feel more appropriate with a counsellor or trusted friend.

The other aspect affecting at least 20% of people is: people who are not context with their employment situation have a lower than average wellbeing. People could either review transferrable skills to explore new/alternative jobs, or engage more with their employer about the day-to-day reality of their situation.

26) Acknowledgements

Most importantly I thank the 1,740 respondents in many countries who took time to respond to my surveys since 2013, some once or twice and others many times. Without these excellent answers my analyses and these reports would not have been possible. I appreciate everyone sharing their details to allow me to report on these important factors.

Thanks are also given to HSP support groups (who I thank for letting their members know about my surveys) and HSP groups on Facebook (whose admins I thank for letting me post about these). Finally, I also thank those individuals in the HSP world who have reviewed and assisted with the translations of the survey questions into different languages, and other individuals who have given feedback and comments on early drafts of these reports over the years.