2021 Survey Results

 

Rare disease day is here again, and I am very pleased to publish the results of my 2021 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/1eB5CHux-V-S91vs3DRujuZP-znsHx0u9

In the post below table 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.

An on-line Survey for People with Hereditary Spastic Paraplegia (HSP) linking Wellbeing with Relationships, Acceptance, Life with HSP and Bladder issues.

These are the results of my ninth survey, launched in October 2021. Thanks are due to all the respondents in many countries who took time to respond to this survey, without these excellent answers this analysis would not be possible. I also thank those individuals in different HSP groups who have reviewed and assisted with the translations of the survey questions into different languages.

Respondents

There were 565 respondents who completed the survey, predominantly from the USA, Germany, Brasil, and the UK. Table 1 shows the distribution of respondents’ locations;

Table 1 - Location of respondents

Location		Respondents	Percentage
USA	(USA)	128	23%
Germany	(DEU)	89	16%
Brasil	(BRA)	65	12%
UK	(GBR)	60	11%
Netherlands	(NDL)	45	8%
France	(FRA)	44	8%
Australia	(AUS)	21	4%
Canada	(CAN)	20	4%
Austria	(AUT)	20	4%
Other Europe	-	59	10%
Rest of world	-	14	2%

The number of responses to this survey is higher than in any previous survey, with two key differences in the distribution. This year included a German language translation, and there was a push from the German and Austrian groups. Also, there was a push from the USA group. The ‘Other Europe’ category includes higher numbers of answers than previous years from Italy, Belgium, Spain, and Switzerland. With this survey the analysis separates out results from those nine countries with higher numbers of respondents to identify country-to-country differences, with 87% of respondents coming from these nine countries.

A brief analysis shows that 195 people who completed this survey had also completed at least one of my previous surveys, representing about 35% of respondents. There are 13 people who have completed seven or more of my nine surveys.

HSP Analysis

The first questions in the questionnaire asked if people had HSP. 561 respondents answered this question, selecting from the following options:

Table 2 – HSP Diagnosis

HSP Diagnosis	Respondents	Percentage
I have HSP - genetic test	360	64%
I have HSP - other diagnosis	155	28%
I do not know if I have HSP	8	1%
I am answering on behalf of someone else with HSP	38	7%

Around 64% have had a genetic test. The proportion having a genetic test is lowest in Brasil (48%), and highest in Germany (82%) and Austria (80%).

There were 321 respondents (57%) who knew which type of HSP they had, with the most common answer being type 4 – SPG4, indicating a mutation in the Spast gene. HSP types with at least four people reporting a diagnosis are shown, with the gene which is mutated in that type of HSP:

Table 4 – Type of HSP

HSP Type	Gene	Respondents
SPG 3/3a	ATL1	18
SPG 4	Spast	152
SPG 5/5a	CYP7B1	6
SPG 7	Paraplegin	59
SPG 8	WASHC5	10
SPG 11	Spatacsin	19
SPG 30	KIF1A	7
SPG 31	REEP1	5
Other types	-	45

These results are similar to those in previous years, where SPG 4 and SPG 7 are the most common types of HSP reported. SPG4 is the most common type of HSP across the world.

Wellbeing

In order to assess the wellbeing of people with HSP, respondents completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS). The minimum scale score is 14 and the maximum is 70. A higher score indicates a better wellbeing. There were 543 respondents who answered this question, with the average wellbeing score of 46.0, which is broadly the same as the average scores of 47.4 in 2019 and 45.9 in 2017 and 2018. The average score in 2020 was 42.9, most likely a result of Coronavirus. The scores ranged between 14 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”.

If the average scores are 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, then this is likely to be significant, particularly for groups of over 50 respondents. 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.

Variation in wellbeing by location

The wellbeing for respondents from the countries with higher numbers of respondents has been calculated to identify if there is potential for differences in wellbeing across the world.

Table 7 – Regional Wellbeing

Area	Wellbeing
USA	45.3
Germany	48.9
Brasil	46.5
UK	43.3
Netherlands	48.0
France	43.4
Australia	44.0
Canada	44.8
Austria	51.8

This shows that people in UK and France have a slightly lower wellbeing and those in Austria and Germany have a slightly higher wellbeing. The Austria score is 5.8 points higher than the average, indicating potential for significance, although it is a dataset of 20 people. All other scores are within 3 points of the average suggesting that any regional differences are not significant.

6) Mobility Analysis

Just over half of respondents use walking sticks/poles/crutches/ canes, two fifths use wheelchairs/mobility scoters and just over a quarter use walking frames/rollators. Functional Electrical Stimulation (FES) is the mobility aid used by the least number of people, with a take-up of 5%. 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	290	51%
Using Wheelchair/Mobility Scooter	246	44%
Using Walking Frame/Rollator	168	30%
Using Orthotics/AFO/Insoles	138	24%
Using FES	29	5%

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;

0. No mobility effects
1. Walk without aids, but some mobility effects
2. Orthotics/AFO/FES and/or Sticks/Poles/Crutches/Canes some of the time
3. Sticks/Poles/Crutches/Canes and Frame/Chair some of the time
4. Sticks/Poles/Crutches/Canes most of the time
5. Sticks/Poles/Crutches/Canes all of the time
6. Rollator/Walking frame most of the time
7. Rollator/Walking frame all of the time
8. Wheelchair/Mobility scooter most of the time
9. Wheelchair/Mobility scooter all of the time

The wellbeing scores show a general decrease in wellbeing with increased mobility aid use. The distribution of wellbeing scores is shown for all respondents in the following figure, split by mobility score.

Relationships

This section included questions about relationships with other people to explore feelings around how their HSP impacts on life in a more general sense.  The first six questions are from the Survey on Stigma and Neurological Disorder, published by European Federation of Neurological Associations (EFNA) in 2020[1].

In the EFNA report the questions were general, whereas I made them specific to HSP.

• Have you ever been excluded from invitations to social events by family or friends?
• Do you feel comfortable discussing issues relating to your HSP with your partner/family?
• In a new romantic relationship, how comfortable would you feel discussing your HSP and its impact?

People also answered how much they agree with the following statements:

• My family understand the consequences of my health problems.
• My family can sometimes make me feel like I am exaggerating about my HSP.
• My family think I can do more than I feel able to.

·         In addition, I asked if people are in long term relationships, and if HSP affected those relationships.

Exclusion from invitations to social events

There are 560 respondents who answered: Have you ever been excluded from invitations to social events by family or friends? Results are shown in the following table.

Table 12 – Have you ever been excluded from invitations to social events by family or friends?

Excluded from social events?	Respondents	Wellbeing
All	560	-
Yes	104 (19%)	40.7
No	337 (60%)	48.9
Not sure	119 (21%)	42.4

Around three in five people with HSP feel they have not been excluded from social events, one in five feel they have, with one in five not sure. Those who feel they have not been excluded have a higher wellbeing, and those who feel they have been excluded have a significantly lower wellbeing.

The perceptions of exclusions increase with mobility aid use. Those in Brasil and Canada perceive more that they have been excluded, and those in Germany, Netherlands, Austria, and France perceive more that they have not been excluded.

Comfortable discussing HSP issues with partner/family

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

Table 13 – 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%)	48.7
No, never	41 (7%)	40.2
Sometimes	164 (29%)	41.6

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.

As shown below, those who use a wheelchair all or most of the time are more inclined not to be comfortable discussing their HSP, and those in Austria are less inclined to be comfortable discussing their HSP all of the time.

Comfortable discussing your HSP in a new romantic relationship

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

Table 14 – 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%)	43.6
I would not discuss it	37 (7%)	37.0
Comfortable, it’s not a problem	224 (43%)	50.3

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.

Whilst there is some variation with mobility aids and from country to country, the patterns are similar. Those in Canada are more likely not to have a problem discussing their HSP whereas those in France are more likely not to discuss it.

My family understand the consequences of my health problems

There are 560 respondents who indicated how much they agreed with the statement: “My family understand the consequences of my health problems”, as shown in the following table.

Table 15 – Agreement with “My family understand the consequences of my health problems.”

My family understand the consequences of my health problems	Respondents	Wellbeing
All	560	-
Strongly agree	173 (31%)	50.4
Agree	194 (35%)	46.9
Neither agree nor disagree	115 (21%)	44.1
Disagree	51 (9%)	37.0
Strongly disagree	27 (5%)	37.1

Two thirds of people with HSP agree or strongly agree with this statement. About one in five neither agree or disagree, with the remainder disagreeing or strongly disagreeing. Those who perceive their family understands the consequences of HSP have a higher wellbeing, and those who perceive their family does not understand have a significantly lower wellbeing.

The pattern is relatively similar across mobility aid use. Those in Germany have family who are most likely to understand, whereas those in France are less likely to have family who understand.

My family can sometimes make me feel like I am exaggerating about my HSP

There are 552 respondents who indicated how much they agreed with the statement: “My family can sometimes make me feel like I am exaggerating about my HSP”, as shown in the following table.

Table 16 – Agreement with “My family can sometimes make me feel like I am exaggerating about my HSP.”

My family can sometimes make me feel like I am exaggerating about my HSP.	Respondents	Wellbeing
All	552	-
Strongly agree	40 (7%)	36.2
Agree	89 (16%)	41.5
Neither agree nor disagree	124 (22%)	44.6
Disagree	163 (30%)	48.4
Strongly disagree	136 (25%)	49.9

Over half of people with HSP disagree or strongly disagree with this statement. About one in five neither agree or disagree, with the remainder agreeing or strongly agreeing. Those who think their family feel like they are exaggerating have a likely significant lower wellbeing, and those who think their family do not feel they are exaggerating have a higher wellbeing.

The pattern is relatively similar across mobility aid use. Those in the Netherlands are more likely to have family who do not make them feel like they are exaggerating, and those in Brasil are more likely to have family who think they exaggerate.

My family think I can do more than I feel able to

There are 558 respondents who indicated how much they agreed with the statement: “My family think I can do more than I feel able to”, as shown in the following table.

Table 17 – Agreement with “My family think I can do more than I feel able to.”

My family think I can do more than I feel able to.	Respondents	Wellbeing
All	558	-
Strongly agree	49 (9%)	40.9
Agree	104 (19%)	41.6
Neither agree nor disagree	143 (26%)	44.7
Disagree	151 (27%)	48.9
Strongly disagree	111 (20%)	50.1

Almost half with HSP disagree or strongly disagree with this statement. About a quarter neither agree or disagree, with the remainder agreeing or strongly agreeing. Those who perceive their family thinking they can do more than they are able have a lower wellbeing, and those whose do not perceive this have a higher wellbeing.

The pattern is relatively similar across mobility aid use. Those in the Netherlands, Germany and Austria are more likely to have family who do not believe that they can do more than they can, whereas those in France are less likely. Those in Brasil are more likely to have family who think they can do more than they can, whereas those in the Netherlands and Germany are less likely.

Are you in a long-term relationship?

There are 544 respondents who answered this question, as shown in the following table.

Table 18 – Are you in a long-term relationship?

Are you in a long-term relationship?	Respondents	Wellbeing
All	544	-
Yes	405 (74%)	47.2
No, and that is what I want	27 (5%)	46.4
No, but I would like to be in one	57 (10%)	41.5
I was in the past	55 (10%)	43.5

Three quarters of people with HSP are in long term relationships. There are five percent who are not, and who do not want to be in one, there are 10% who are not, but would like to be in one, and there are 10% who were in the past. Those whose are not in a relationship, but would like to be in one have a lower wellbeing. All others are within 3 points of the average.

There are fewer who are in a wheelchair all or most of the time who are in a long term relationship, otherwise patterns are similar between mobility aid use and from country to country.

Has HSP affected your long-term relationships in the past?

There are 544 respondents who answered this question, as shown in the following table.

Table 19 – Has HSP affected your long-term relationships in the past?

Has HSP affected your long-term relationships in the past?	Respondents	Wellbeing
All	544	-
No	264 (49%)	49.0
Yes, positively	22 (4%)	47.2
Yes, negatively	122 (22%)	40.3
Not relevant	136 (25%)	45.0

There are more people whose HSP did not affected previous long-term relationships than people whose HSP did affect. When HSP has affected relationships, it has affected the relationship negatively more often than positively. Those whose HSP did not affect previous relationships have a higher wellbeing, and those whose HSP negatively affected previous relationships have a significantly lower wellbeing.

HSP can be seen to have affected previous relationships more for those who use mobility aids all or most of the time. There are people at all levels of mobility aid and from all countries whose HSP has positively affected their previous long-term relationships.

Does HSP affect your current long-term relationship?

There are 533 respondents who answered this question, as shown in the following table.

Table 20 –Does HSP affect your current long-term relationship?

Does HSP affect your current long-term relationship?	Respondents	Wellbeing
All	533	-
No	251 (47%)	49.4
Yes, positively	42 (8%)	47.9
Yes, negatively	101 (19%)	40.0
Not relevant	139 (26%)	43.8

There are more people whose HSP does not affect their current long-term relationships than whose HSP does affect them. When HSP affects relationships it does so negatively more often than positively, but there is a higher proportion whose HSP positively affects their current relationships compared with previous relationships. Those whose HSP does not affect relationships have a higher wellbeing, and those whose HSP negatively affects their relationships have a significantly lower wellbeing.

HSP can be seen to affect relationships more for those who use mobility aids all or most of the time.

Do you think HSP will affect your future long-term relationships?

There are 537 respondents who answered this question, as shown in the following table.

Table 21 –Do you think HSP will affect your future long-term relationships?

Do you think HSP will affect your future long-term relationships?	Respondents	Wellbeing
All	537	-
No	182 (34%)	51.5
Yes, positively	42 (8%)	43.0
Yes, negatively	164 (31%)	40.5
Not relevant	149 (28%)	46.4

There are more people who think that their HSP will affect future relationships than who think it affects current relationships. There is also a higher proportion thinking that HSP will affect future relationships negatively. Those who think HSP will not affect future relationships have a significantly higher wellbeing, and those who think that HSP will negatively affects future relationships have a significantly lower wellbeing.

These patterns are similar to the past and current relationships ones, with larger negative effects.

Comments on relationships

Additional comments were made by 152 respondents about relationships. Almost half (43%) were unsure about relationships, and about a third (36%) were negative, with the remainder being either positive or variable. Some comments present these issues in powerful ways, and are included below.  These are the respondents’ own words (or Googles translation of them into English), with occasional minor adjustments for clarity or anonymity. Although these comments are generally cover negative aspects, they contextualise some of the wellbeing results shown.

Relationships Comments

Thank you for asking such important questions, that many times I don't even have the courage to think about it.

These insightful questions make me think more deeply about my feelings which I often brush aside.

My yucky days were when I was at school and away from my family. That was stressful for me and caused some anxiety. It's not the HSP that gets me down.

I feel invisible to my family and some friends.

It has ruined any enjoyment I had in life.

Never had a relationship.

I don't dare to get into a relationship because I don't want to be dropped in the end.

I try to hide my problems as much as possible, out of pride for myself.

I have recently noticed that an awful lot depends on oneself.

I exclude myself from certain things that would put me in an uncomfortable situation.

Hardly any sex since the HSP.

No more sex life, I miss that a lot, my husband no longer desires me.

My family is very physical - hiking, camping, outdoor games. I am trying to not have them quit what they want to do because of me, but I know someday I will have to find another way to enjoy time with them.

I was together with my current partner before being diagnosed with HSP.  We often make decisions based on my past abilities, not my current ones.

It’s hard to feel whole when you can't keep up with your partner. Makes me feel less of a person.

My long term boyfriend said I should consider myself lucky he stayed because no one would with my health problems.

My husband is very understanding. My sons find it difficult and my mother denies my complaints because she has it herself and would rather not see it.

I got divorced from my husband and was diagnosed with HSP not long after that. I choose not to be in a relationship because we took the vow “till death do us part”. That’s my first reason, but now with the things I have to do because of HSP I don’t think I would want to be in a new relationship.

I am currently not in a relationship with anyone by choice. I've never had any problems with it, but with my spasticity getting worse and my walking in the last two years, I prefer to be alone so I don't bother or worry anyone. Unfortunately I cannot accept the current level of my disability.

I really miss not having a partner. As a result, I regularly feel down and this affects the HSP. I often miss this in the information I read. Mental health is very important for how I walk or how I feel.

I don't want to feel like a burden to anyone and at the same time I don't want to feel the fear of losing someone because of HSP progression that I can't control.  I would like to be independent as much as I can.  Maybe just have friends instead of a partner now.  This has been one of the hardest things to accept about HSP, as I know having a partner would have many pros.

I was married for 25 years and he has now left. He says it is nothing to do with HSP but it was definitely a factor, partly because I struggled to come to terms with it.

My husband hates having to be my carer. He feels I am preventing him being free to complete his bucket list.

I’ve been married to the same person for over 20 years. My HSP is causing him LOTS of stress and we fight a lot. I know he resents how much he has to do that I find difficult now.

I have been married for nearly 40 years.  He saw what it did to my mom.  He knows what is coming, but he loves me.

HSP is something my partner and I have to adjust our lives to live with, so we can get the most out of them!!

This reminds me that I am fortunate to have a supportive and understanding family.

I add: Life is beautiful despite everything

Relationships Summary

These question shows that there is a larger proportion of people having better experiences in relationships than having worse experiences. On the whole more than half of respondents have positive experiences, and less than a quarter have negative experiences. There are large differences in wellbeing for those having positive experiences compared with those having negative experiences, demonstrating that these aspects have a strong influence on wellbeing.

Of these questions, the three with the lowest wellbeing are; those who consider that their family thinks they exaggerate their HSP, those who consider that their family don’t understand the consequences of their health problems, and those who would not discuss their HSP in a new romantic relationship.

Similarly, the three with the highest wellbeing are; those who do not consider that their family thinks they exaggerate their HSP, those who are comfortable discussing their HSP in a new romantic relationship, and those who don’t think that HSP will affect their future long term relationships.

There are three areas where there is a 13 point difference in wellbeing score between positive and negative experiences, indicating a significant effect. As these findings present people’s opinions, there is scope for opinion change. People could explore changing opinions through conversations with trusted friends, counsellors, or family. These areas are: Thinking your family thinks you exaggerate your HSP, thinking your family doesn’t understand the consequences of HSP, and discussing HSP in a new romantic relationship.

Of those who are not in long term relationships, there are roughly twice as many people who would like to be in one compared with people who are content not being in one.

Acceptance and Stigma

Acceptance

Part of the journey with a progressive condition like HSP is accepting that you have HSP. Respondents filled out the Acceptance of Illness Scale (AIS) which is tool to measure acceptance of any disease. There are 8 questions, and people select one answer from a choice of five.

Each answer is scored between 1 and 5, so the total score ranges 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. Scores covered the full range between 8 and 40, and the distribution across countries is shown in table form and graphically.

Table 22 – Acceptance of Illness Scale

AIS	All	USA	Germany	Brasil	UK	Netherlands	France	Australia	Canada	Austria
Answers	511	122	84	44	57	41	42	20	18	19
Score:	21.7	20.9	22.8	24.0	20.2	25.3	19.1	20.3	20.5	21.9
<20	40%	44%	33%	23%	56%	12%	52%	60%	39%	53%
20-30	44%	43%	49%	52%	28%	63%	36%	25%	44%	26%
≥30	16%	12%	18%	25%	16%	24%	12%	15%	17%	21%

All country averages are broadly in the range 20-25 points. Brasil and the Netherlands have the greatest proportion with good acceptance. Australia and the UK have the greatest proportion with low acceptance.

Acceptance and Wellbeing

There is a strong link between acceptance and wellbeing. Those who have a low level of acceptance have a wellbeing score of 38.4, more than 7 points below the average and those that have a high acceptance have a wellbeing score of 55.9, almost 10 points higher than the average.

Table 5 – Acceptance of Illness Scale - Wellbeing

AIS	All	USA	Germany	Brasil	UK	Netherlands	France	Australia	Canada	Austria
<20	38.4	36.9	42.0	38.2	37.8	36.6	40.1	37.2	32.9	46.8
20-30	49.3	50.9	50.6	46.1	49.2	47.8	47.5	49.1	50.4	56.2
≥30	55.9	55.3	58.0	56.7	53.7	53.9	51.4	61.0	54.0	57.8

The pattern is similar across countries. Results are more than 5pts from average, and therefore significant.

Stigma

Respondents answered the extent which they feel stigma round their HSP. 503 respondents answered this question, which is also from the Survey on Stigma and Neurological Disorder, published by EFNA in 2020.

Over half (55%) say they feel a little stigma on account of their HSP. About a quarter (28%) feel no stigma at all, and the remainder (17%) feel very much affected by stigma. Overall, nearly three quarter (72%) are affected by stigma, either a little or very much. The patterns are similar between countries.  People identified the factor which they believed to be the greatest cause of stigma:

Table 27 – Greatest cause of stigma

Stigma cause	Respondents	Percent
Lack of understanding about HSP	166	35%
The symptoms of HSP	140	30%
The invisible nature of HSP	107	23%
Because HSP is a nerve/brain disorder	39	8%
Misconceptions or myths about HSP	12	3%
The name HSP	8	2%

People identify a lack of understanding about HSP and the symptoms of HSP to be two main causes of stigma they feel about having HSP, these factors being chosen by about two thirds of respondents.

These patterns are similar around the world except Germany and Austria where the greatest cause is the invisible nature of HSP, accounting for more than half of the responses in each country. It is not clear if there are different viewpoints in Germany and Austria, of if this is an effect of the translation between languages.

Peoples Thoughts on Acceptance and Stigma

Several people left comments about acceptance and stigma. There are some powerful thoughts, and these lived experiences are given below. These thoughts are in the respondents’ own words (or Googles translation of them into English), with occasional minor adjustments for clarity or anonymity.

Acceptance and Stigma comments

By asking a few direct questions about my mobility issues, the other person is able to see me as an equal person with rare powers, not just a person with a rare disease.

I accommodate my limitations as my abilities decline, making my life easier.

People assume you are lazy, drunk or weak. HSP is not a life choice that you have any say in.

I do feel friends backing away as the disease progresses.

I feel like people treat me differently after they see the AFO.

People assume that because HSP affects mobility, I am less human and have less to contribute.

I get exhausted just thinking about how much I need to share information with others.

My worst stigma comes from my children who don't want to be seen with me in public as I embarrass them. (This cuts me deeply.)

It is difficult to accept the decline.

I usually tell people I have MS due to lack of understanding.

I still struggle to use a walking stick and I rather use an umbrella as a walking aid.

I have noticed distrust in people when they see me, a young person, usually on public transport or in social activities.

Before a catheter was in place I had very many embarrassing public wettings, which I detested.

I no longer consider going out alone for the sake of any problem that might arise.

My main problem with HSP is with myself, it's the enormous difficulty it causes in my walk. I don't really accept this deficiency, perhaps because I can still manage on my own most of the time. I really wanted to be able to walk, run, ride a bike, as I used to, but this is currently impossible and my head unfortunately still doesn't accept it correctly.

Personally, the hardest thing is to accept that I am no longer as "able-bodied" as 5 years ago, and people's eyes seem to say "He's not sick, he's not in a wheelchair and he walks..."

My son doesn't understand he has this disease and doesn't understand that people make judgements.  I however feel them a great deal on his behalf.

It's permanent and without a cure so I am stuck in this situation and have a very hard time accepting this. I feel like no one understands my disease, even doctors sometimes. I am tired of being handicapped and wish to have my life back.

I don’t care what strangers think at all.  If I could lose the pain in my legs, and not feel so tired all the time, I could handle the disability and do most of what I miss.  The disability itself is the easy part.  The hard parts are pain, fatigue and depression.

I live very well with it, I don't feel judged by those around me, it's the ignorance and impatience of those who don't know which is hard.

Acceptance and Stigma Summary

The average acceptance score of people with HSP is near the change-over between a low level of acceptance and a moderate level of acceptance. Those who use mobility aids all or most of the time accept their HSP slightly less than others. People generally agree that their HSP means they are not able to do what they like best.

There is a strong link between acceptance and wellbeing. Those who are able to accept their HSP have wellbeing significantly above average, and those who have low acceptance of their HSP have wellbeing significantly below average.

People feel stigma associated with their HSP, and this is most often perceived to be due to lack of understanding about HSP or the symptoms of HSP.

Life with HSP

Do you Consider Yourself to be Disabled?

There are 563 respondents who answered this question, as shown in the following grid.

Table 28 – Identify as Disabled?

Consider Self Disabled?	Yes	No	Wellbeing Yes	Wellbeing No
All	457 (81%)	106	45.0	50.6
Mobility 0-1	39 (44%)	50	47.6	51.7
Mobility 2-3	89 (73%)	33	45.3	51.4
Mobility 4-5	152 (93%)	11	46.0	(46.1)
Mobility 6-7	77 (93%)	6	43.6	-
Mobility 8-9	100 (94%)	6	43.1	-

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. When mobility aids are used all or most of the time the wellbeing is similar, irrespective of if people consider themselves to be disabled.

Those who identify as disabled have an acceptance of illness scale score of 20.8 whereas those who do not identify as disabled have a score of 26.3. This may indicate that people regard themselves as disabled before they accept that they have HSP.

Depression

Respondents answered the two question PHQ2 screening tool for depression. 545 people answered these questions. The total score ranges 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.

Table 29 – PHQ 2 – Screening for depression

PHQ2 score range	Respondents	Wellbeing
All	545	-
Score 0-1	242 (44%)	53.3
Score 2-6	303 (56%)	40.3
Score 0-2	407 (75%)	49.4
Score 3-6	138 (25%)	36.0
Score 2	165 (30%)	43.9

These results show that scores of 0 or 1 have a significantly higher wellbeing and scores of at least 3 have a significantly lower wellbeing. Around a third of respondents have a score of 2 with a wellbeing slightly below average.  This indicates that a cut-on of 3 points is likely to be appropriate as a cut-on point for screening depression for people with HSP.

Trips and Falls

Respondents were asked about their frequency of trips and falls.  At least 520 people answered these questions. The overall summary is shown in the table below. People selected an answer for each question from the following choices:

• Never
• A few times
• More than once a month
• More than once a week
• More than once a day
• In the past, but not any more

Table 32 – Summary of Trips and Falls from HSP

As a result of your HSP, have you ever….	Answers	Frequency
		Never	A few times	Monthly+	Weekly +	Daily+	In the past
Scuffed your feet	520	19%	21%	10%	18%	28%	4%
Lost your balance	547	3%	23%	22%	29%	26%	3%
Tripped up	545	2%	25%	26%	28%	19%	5%
Stopped yourself falling	536	6%	32%	23%	25%	15%	2%
Fallen down	551	9%	55%	22%	12%	3%	4%

This shows that 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.

The higher numbers from these trends are highlighted in bold text in the table

When looking at the different countries, whilst there are some minor variations in the pattern, the general trends are similar.  Those in the USA tend to scuff their feet more on a daily basis, and those in Brasil seem to have to stop themselves falling less frequently. 

Table 33 –Trips and Falls from HSP - wellbeing

As a result of your HSP, have you ever….	Frequency
	Never	A few times	Monthly+	Weekly+	Daily+	In the past
Scuffed your feet	48.4	48.0	44.5	45.4	43.7	(47.5)
Lost your balance	(47.7)	48.2	47.4	44.7	44.4	(43.2)
Tripped up	-	47.8	47.3	44.5	44.4	46.4
Stopped yourself falling	44.9	47.5	48.8	43.6	44.1	-
Fallen down	48.5	47.7	43.3	39.7	(47.6)	47.2

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.

Those who are affected more often by each effect have a lower wellbeing. For those who scuff their feet or fall down the wellbeing drop comes when this happens monthly. For those lose balance, trip up or stop themselves falling the wellbeing drop comes between monthly occurrence and weekly occurrence.

Bladder

Respondents were asked details about urinating. Around 550 people answered these questions, with some people not answering all of these questions. Five questions were asked, with the first four from the ICIQ Overactive Bladder questionnaire, which I used in my 2016 survey.

• How often do you pass urine during the day?
• During the night, how many times do you have to get up to urinate, on average?
• Do you have to rush to the toilet to urinate?
• Does urine leak before you can get to the toilet?
• Does your bladder empty completely when you go to the toilet?

Respondents selected an answer from a pick-list for each question. In the ICIQ questions the answers are scored allowing a total overactive bladder score to be determined.  There are five choices for each question, which are scored from 0 for the smallest answer through to 4 for the highest answer, giving overall scores ranging between 0 and 16.

During the analysis for my 2016 survey a score of 3 was used to mark the onset of overactive bladder effects, and the following ranges were 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.

All four questions were answered by 544 respondents, with an average score of 5.7. Scores ranged from 0 through to 16. Scores have not been evaluated for respondents who did not answer all four of these questions.

Table 39 – Overactive Bladder Score Results

Overactive Bladder Effect Bands	Respondents		Wellbeing
All respondents	544	-	-
0-2: No effects	96	18%	48.0
3-6: Mild effects	230	42%	46.8
7-10: Moderate effects	171	31%	45.3
11+: Severe effects	47	9%	42.9

This table shows that 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.

Table 41 – Overactive bladder score by mobility aid use

Overactive Bladder	Respondents	OAB Score
All respondents	544	5.7
0-1: Those without aids	89	4.8
2-3: Those who use mobility aids some of the time	119	5.1
4-5: Those who use sticks most/all of the time	161	5.8
6-7: Those who use frames most/all of the time	79	7.0
8-9: Those who use chairs most/all of the time	96	6.2

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 only respondents scoring the highest score of 16 use a wheelchair all or most of the time. With all other mobility uses (including no aids) the highest score was 13 or 14. There were people scoring zero points in all mobility aid uses indicating that there are people who use mobility aids all or most of the time who do not have any bladder issues. The distribution of scores is shown:

The survey 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, and were given the opportunity to identify if they used other methods than those in the list or to report that they used more than one option.

Table 48 – Bladder problem management

Main method for managing bladder problems	Respondents		Wellbeing
Do not have bladder problems	90	17%	48.8
Take no action to manage	133	25%	46.0
Medication to relieve spasticity	126	23%	44.0
Drink less fluids	85	16%	43.7
Train bladder muscles	53	10%	47.9
Use an intermittent catheter	33	6%	46.8
Botox injections in bladder	10	2%	(48.7)
Use a catheter which stays in place	9	2%	-

This question was answered by 539 people. Most (59%) use at least one of the methods noted in the table. The most common method used is taking medication to relieve spasticity in the bladder, followed by deliberately drinking less fluids during the day.  All of the wellbeing scores are within three points of the average, suggesting that the method of bladder issue management is not an important factor in wellbeing. All of these measures are used by those with SPG7, however they tend to manage more by drinking less fluid, with this measure being used by a higher proportion of people with SPG7 than the other measures.

Comments were made by 179 people who mostly described either one method in more detail or using more than one method. Of methods listed that were not in the pick-list choices, the most common are:

• 32 people use an incontinence pad
• 14 people plan their drinking – mostly drinking more in the morning and less in the evening
• 12 people use incontinence pants or nappies
• 6 people use a urinal, bottle or similar
• 4 people use Sacral Nerve Stimulation (SNM), also known as a bladder pacemaker

Other methods noted by one or two people include: Changing their posture when going to the toilet or using hands to agitate the bladder. Some take turmeric, cranberries, oregano, or CBD. Some drink less caffeine and/or alcohol. One person had Transurethral resection of the prostate (TURP), with another using biofeedback. Incontinence sheets are also used on some people’s beds.

Bladder Summary

More than 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.

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.

Participation in HSP Research

Respondents were asked about their participation in HSP research. These questions were answered by around 550 people.

Table 49 – Participation in HSP research

Question	Respondents	Yes
Have you ever taken part in a HSP research project?	562	197	35%
Would you like to take part in future HSP research projects?	551	487	87%
Do you know how to find out about upcoming HSP research projects?	554	163	29%

Around a third of respondents have taken part in HSP research studies. Almost 90% of people would like to take part in future projects, but only 30% of people know how to find out about upcoming trials. This indicates that those who are planning HSP research have potential to reach out to a wider pool of people to participate in their studies.

The fourth question in this section of the survey asked people to give their reasons for wanting to take part in HSP research projects. 423 respondents gave answers to this question, and there were several common reasons given. Some people gave a single answer whereas other people mentioned several things.

Learning

The most common answer was around learning, with 170 people identifying this as a reason. There were three types of answer about learning, some people were keen to find out more information about HSP for themselves, others were keen that the research teams would learn more about HSP, and a third group of people mentioned learning or knowledge, but didn’t express who would increase their learning/knowledge.

Treatments and Cures

There were 105 people who want research to identify more/better treatments for HSP and 84 people who want research to identify a cure for HSP.

Helping

Some respondents expressed their desire to help, with 95 using this term. Some simply said they wanted to help, whereas others want to help other people or future generations.

HSP Information Sources

Respondents were presented with a list of potential information sources about HSP. They were invited to identify which ones they used. Results show each one is used by at least half of respondents. For those sources that they used people identified how much they trusted the source. The results are shown below.

Almost everyone with HSP uses their neurologist and doctor as a source of information about HSP. Neurologists are the most trusted source of information, being trusted a great deal or quite a lot by 84%.

The second most trusted source of information, used by 87%, are members of a support group. 83% of people who get information from support group members trust this information a great deal or quite a lot.

Physiotherapists are used as a source of information by 87% of people with HSP, and these are trusted a great deal or quite a lot by 73% of those getting information this way. Medical journals score similarly, used by 78% and trusted a great deal or quite a lot by 71% of these people.

Whilst doctors are used as an information source for HSP by 97% of people, the information given is trusted a great deal or quite a lot by 52% of those people.

Table 51 – HSP Information Sources

Information source	Answers	Used by	Trust level
			Great deal	Quite a lot	Not very much	Not at all
A Neurologist	553	97%	48%	37%	14%	1%
A Doctor	522	97%	16%	36%	41%	7%
Members of a support group	527	87%	30%	53%	14%	3%
A Physiotherapist	517	87%	27%	46%	24%	3%
Another medical professional	483	85%	10%	31%	49%	10%
Medical websites	498	83%	14%	49%	30%	7%
Friends/Family with HSP	501	82%	24%	44%	25%	7%
Medical journals/research articles	486	78%	24%	47%	26%	3%
Online rare disease communities	504	78%	17%	49%	28%	6%
Social media	487	73%	4%	24%	52%	20%
Books/magazines	478	67%	10%	42%	39%	9%
Leaflets/booklets	476	67%	9%	35%	44%	12%
YouTube	485	67%	4%	23%	54%	18%
Other websites	459	64%	2%	17%	60%	20%
Blogs	475	59%	5%	30%	48%	17%

The most obvious gap in trust is with doctors, used as an HSP information source by almost all people, but with whom the level of information trust is lower.  

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 walking distance and needing to rush to the toilet. 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 and medications 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:

• Balance/strength exercises or modifications at home/work to reduce falls
• Checking that mobility aids are still appropriate
• Techniques to help manage bladder issues
• Reviewing factors affecting depression

Positive Wellbeing Effect 

Table 52 – Positive Wellbeing Factors

Factor	Respondents	Wellbeing
AIS score 30+ (good acceptance)	84	55.9	+9.9
PHQ score 0-1	242	53.3	+7.3
Living in Austria	20	51.8	+5.8
Not considering yourself disabled, no walking aids	50	51.7	+5.7
HSP not affecting future relationships	182	51.5	+5.5
Not consider yourself disabled, mobility aids some of the time	33	51.4	+5.4
Walk as far as you want	98	50.8	+4.8
Not considering yourself disabled	106	50.6	+4.6
My family understands my HSP - strongly agree	173	50.4	+4.4
Comfortable discussing my HSP in a new relationship	224	50.3	+4.3
Family feels I'm more able than I feel - strongly disagree	111	50.1	+4.1
Have SPG 3/3A	18	50.1	+4.1
Family thinks I exaggerate my HSP - strongly disagree	136	49.9	+3.9
Not using mobility aids	89	49.9	+3.9
Never have to rush to the toilet	58	49.5	+3.5
PHQ score 0-2	407	49.4	+3.4
HSP not affecting current relationship	251	49.4	+3.4
AIS score 20-30 (moderate acceptance)	225	49.3	+3.3
Bladder empties fully every time	118	49.2	+3.2
HSP not affected past relationships	264	49.0	+3.0

 Negative Wellbeing Effect

 Table 53 – Negative Wellbeing Factors

Factor	Respondents	Wellbeing
PHQ score 3-6 (screen for depression)	138	36.0	-10.0
Family thinks I exaggerate my HSP - strongly agree	40	36.2	-9.8
My family understands my HSP - disagree	51	37.0	-9.0
Not comfortable discussing my HSP in new relationships	37	37.0	-9.0
My family understands my HSP - strongly disagree	27	37.1	-8.9
AIS score <20 (low acceptance)	202	38.4	-7.6
Falling down weekly	64	39.7	-6.3
HSP affects current relationship negatively	101	40.0	-6.0
Not comfortable discussing my HSP with family/partner	41	40.2	-5.8
HSP affected past relationships negatively	122	40.3	-5.7
HSP may affect future relationships negatively	164	40.5	-5.5
Rush to the toilet all the time	48	40.6	-5.4
Have been excluded from social activities	104	40.7	-5.3
Family feels I'm more able than I feel - strongly agree	49	40.9	-5.1
High mobility aid change over 5 years	60	41.2	-4.8
Can walk up to 10m	50	41.5	-4.5
Family thinks I exaggerate my HSP - agree	89	41.5	-4.5
Would like to be in a long term relationship	57	41.5	-4.5
Family feels I'm more able than I feel - agree	104	41.6	-4.4
Sometimes comfortable discussing my HSP with family/partner	164	41.6	-4.4
3 visits to the toilet per night	55	42.4	-3.6
Possible exclusion from social activities	119	42.4	-3.6
Leak urine before toilet all the time	26	42.8	-3.2
Severe effects from overactive bladder	47	42.9	-3.1
HSP may affect future relationships positively	42	43.0	-3.0

The Importance of Wellbeing

Half of the factors in these two tables are from the relationships section, which demonstrates that people’s experiences with relationships is a key influence on their wellbeing.

As most factors shown are not connected with mobility, symptoms or other issues directly related to HSP, wellbeing from these aspects may not form part of routine discussions with medical professionals which frequently focus on mobility, pain, continence and the other direct issues or symptoms.

Some of the factors appear in both the positive and negative tables, indicating that there is potential for large changes in wellbeing if people can change their treatments or perspectives. The factors with the biggest potential for changes are:

Table 54 – Factors with Potential for the Biggest Changes in Wellbeing

Factor	Positive	Negative	Change
AIS (score at least 30 to score less than 20)	55.9	38.4	17.4
PHQ (score 0 or 1 to score between 3 and 6)	53.3	36.0	17.3
Family thinks I exaggerate (st. disagree to st. agree)	49.9	36.2	13.7
Family understands my HSP (st. agree to st. disagree)	50.4	37.1	13.3
Comfortable discussing my HSP in new relationship (yes to no)	50.3	37.0	13.3
Thinking HSP affects future relationships (not to negatively)	51.5	40.5	11.0
Walking (unlimited distance to up to 10m)	50.8	41.5	9.3
Family feels I’m more able than I feel (st. disagree to st. agree)	50.1	40.9	9.2
Toilet rush (not rush at all to rush all the time)	49.5	40.6	8.9

The two areas with the greatest change are measured with the Acceptance of Illness Scale (for acceptance), and the PHQ2 scale (for depression). Those who have low acceptance of their HSP have significantly lower wellbeing and those with good acceptance have significantly higher wellbeing. This level of wellbeing change is also seen with the PHQ2 score for depression. People could consider counselling or taking/changing treatments for acceptance or depression.

There are two key aspects covering the greatest wellbeing changes within relationships:

• Many of the questions are phrased 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, there are some questions showing that people are sometimes not comfortable discussing their situations with important people.

Provided that people feel safe to do so, they could consider having conversations with these important people which initially feel difficult, but which can help everyone improve their understanding, recognise that situations change and be honest about concerns, difficulties and feelings. For some, those conversations may feel more appropriate with a counsellor or trusted friend.

[1] https://www.efna.net/wp-content/uploads/2020/07/SurveyReport2020.pdf