Thursday, 28 February 2019

2018 Survey Results

Rare disease day is here again, and I'm very pleased to be able to publish the results of my sixth survey.

This survey was launched in the autumn of 2018, and once again I have had a fantastic response from people around the world completing the questions and contributing to my analysis of HSP.

This survey repeats some of the questions asked in my original 2013 survey, and offers the opportunity to look at some results longitudinally. This survey also marks another new variation. I used Google Translate to translate the questions into other languages to allow people who do not speak English to complete the survey. I also used Google to translate their answers into English for this analysis.

In this post is a short version of the results. The full version can be found by clicking the link here and that will take you to a PDF with the full results and full analysis. There is more detail in each of the sections, so if you're after more detail, I suggest you look here: https://drive.google.com/file/d/1YaQsuXV7yhz1b5gVsUYLJT_rcAed-g0q/view?usp=sharing

There were 304 respondents who completed the survey, predominantly from the USA, UK and Brazil. 
A brief analysis shows that 97 people who completed this survey had also completed at least one of my previous surveys, representing about a third of respondents. A small number of people, five, have completed all six surveys. There are 14 respondents who completed both the 2013 survey and this 2018 survey.

There were 156 respondents (51%) who knew which type of HSP they had. SPG4 and SPG 7 are the most common types of HSP, with SPG11 taking third place.

Misdiagnosis
Around one third of respondents had been diagnosed with another condition before HSP.

Most respondents listed other conditions that they were diagnosed with before their HSP diagnosis. In total 122 other conditions were described. Reading the list it is likely that some of these will be misdiagnoses whereas others are diagnoses of another condition which occurs in parallel with HSP. Six conditions are listed which were diagnosed at least five times and are likely to be misdiagnoses:

 Table 1 – Potential misdiagnosis
Potential misdiagnosis
Respondents
Cerebral Palsy
15
Multiple Sclerosis
12
Ataxia
10
Arthritis
8
Herniated disc
6
Neuropathy
5

Two respondents report that their diagnosis was their doctor saying it was all in their head and there was nothing wrong with them. A small number of people were diagnosed with Primary Lateral Sclerosis, Charcot Marie Tooth disease, Fibromyalgia and Ehlers Danlos Syndrome

These results are similar to those found in the 2013 survey – where Multiple Sclerosis and Cerebral Palsy were the most frequent misdiagnoses.

Multiple Long-Term Health Conditions
Around one third of respondents are living with multiple long-term health conditions. There were five conditions listed which affect at least five people:

 Table 2 – Other Long-term Health Conditions
Heath Conditions
Respondents
High blood pressure
14
Depression
10
Arthritis
10
Asthma
7
Diabetes
5

A small number of people report having HSP along with Ehlers Danlos Syndrome, Ataxia or Parkinsons. Several people listed pain, bladder and bowel issues as other long-term health conditions. It is not clear if these are symptoms of HSP or separate, different health conditions experienced by those people.

Whilst more than half of these people listed one other condition, a third listed two or three other conditions, and the remainder (less than 10%) had between 4 and 7 other long-term health conditions. This analysis shows that some people may have much more complicated journeys managing the symptoms and effects of multiple long term heath conditions.  

Wellbeing

In order to assess the wellbeing of people with HSP respondents completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS). The average wellbeing score is 45.9, which is the same average score as shown last year. The average is lower than UK population norms, however it is still within the “average” definition and is also similar to those with a self-perceived health status of “poor”.

The average score for those with HSP answering this survey is more than 5 points below the UK population norms (51-52) suggesting a significant effect.

There was no significant difference in wellbeing for those people who have additional long-term health conditions compared with those who do not.

Those who have SPG4 tended to have a slightly better wellbeing than those with SPG7, the two biggest groups, but neither average is more than 3 points from 45.9. Although it is a small sample size, those with SPG11 appear to have a lower wellbeing score, with their average being 40.4. Noting the small sample size, this effect might be significant.

Mobility Analysis
Just over half of respondents use walking sticks/poles/crutches/ canes and almost two fifths use wheelchairs/mobility scoters and walking frames/rollators. FES is the mobility aid used by the least number of people, with a take-up of less than 5%. Broadly these results are similar to those from previous years. Table 8 shows these results, including the percentage of respondents.
  
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;
  1. No mobility effects
  2. Can walk without aids but some effects
  3. Orthotics/AFO/FES and/or Sticks/Poles/Crutches/Canes some of the time
  4. Sticks/Poles/Crutches/Canes and Frame/Chair some of the time
  5. Sticks/Poles/Crutches/Canes most of the time
  6. Sticks/Poles/Crutches/Canes all of the time
  7. Rollator/Walking frame most of the time
  8. Rollator/Walking frame all of the time
  9. Wheelchair/Mobility scooter most of the time
  10. Wheelchair/Mobility scooter all of the time

In the remainder of this paper, whenever “sticks” are referred to as a mobility aid, this term includes poles, crutches and canes. Whenever “frames” are referred to this includes both walking frames and rollators. Whenever “chairs” are referred to this includes both wheelchairs and mobility scooters. Whenever AFO is referred to is includes both orthotics and AFO.

The detailed distribution of answers for all respondents is given in Appendix A along with their corresponding mobility score. The results are then simplified into five broader groups;

Table 3 – Overview of mobility aids used
Mobility Aids Used - Overview:
Respondents
Percentage
Mobility Score
Wellbeing
Those without aids
60
20%
0-1
47.6
Those who use mobility aids some of the time
69
23%
2-3
47.7
Those who use sticks most/all of the time
105
35%
4-5
44.9
Those who use frames most/all of the time
33
11%
6-7
46.2
Those who use chairs most/all of the time
37
12%
8-9
41.9

This shows that there is a wide range of mobility aids used by the respondents to the questionnaire, covering all mobility scores. Overall the results are similar to previous years with broadly an even split between bands.

The wellbeing scores for those using chairs all or most of the time is lower than the average score, suggesting that those who use chairs all or most of the time have a lower wellbeing than typical.The general decrease in wellbeing at the highest mobility scores is in contrast to the results in the 2017 survey which showed mobility score 9 similar to mobility scores 6 to 8. This figure shows the wellbeing distribution by mobility score:




Change in Mobility
Up to around 20% of patients may experience significant changes in mobility over a five year period, which is a similar result to last year. These results also show that the quicker a persons change in mobility the lower their wellbeing is likely to be.

With respondents completing both surveys over 4 or 5 years this allows an analysis of change over time without having to rely on peoples memory for the types of mobility aids they were using five years ago. 
Some have gradual changes over a number of years, whereas others have step changes such that all of the change occurs between two adjacent years. These results show there is a greater proportion of people with a low change in mobility over a four or five year period, and that correspondingly there a fewer with medium or high changes in mobility.

Symptoms

The 2013 survey asked people to identify how much each of 13 different HSP symptoms affected them. For this survey the list has expanded to 36 symptoms, and respondents were asked to identify how each affects them, selecting from a list of answers, which have been grouped together to aid the analysis.

Table 4 – Answers and Symptom Grouping
Answer
Grouping
Do not have
Do not have
Occasional symptom
Minor symptom
Minor symptom
Minor symptom
Frequent symptom
Moderate symptom
Regular symptom
Moderate symptom
Most of the time
Major symptom
All of the time
Major symptom

Symptom Impact
Table 5 lists all the symptoms in the questionnaire, ordered by how people are affected by them, with the first symptom being that which affects the highest number of people the most.

Table 5 – 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%
Pins and needles
9%
19%
34%
37%
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%
Legs swell up
10%
11%
22%
57%
Stiffness/spasticity in arms/upper body
10%
10%
24%
56%
HSP affecting speech
10%
8%
18%
65%
Difficulty swallowing
6%
11%
19%
63%
HSP affecting vision
9%
6%
18%
68%
HSP affecting hearing
5%
4%
14%
77%
Ichthyosis (widespread persistent thick, dry, "fish-scale" skin)
3%
6%
14%
77%
Changes in perceptions of smell/taste
3%
6%
12%
79%
Epilepsy or seizures
1%
1%
6%
92%













































The first key observation is that there are people who are affected all or most of the time by every one of the symptoms listed. The first five symptoms (loss of balance, muscles being stiff and difficulty running, walking, using stairs) all relate to mobility and affect almost everybody. Of all of the other symptoms there are people who do not have these symptoms.

Symptom with the Greatest Effect
Respondents were also asked to describe which of the symptoms gave the greatest effect or had the biggest impact. 283 respondents answered this question. This was a free text answer. Some respondents indicated one or two symptoms, whereas a small number of others listed many. This table shows how many symptoms with greatest effect were listed by respondents:

Table 6 – Number of Greatest Effect Symptoms
Number of symptoms
Respondents
1
98
2
94
3
44
4
27
5
8
More than 5
7

Overall, there are 617 symptoms identified as having the greatest effect or impact on respondents. The 16 symptoms which are reported by at least 10 people are shown in the following table:

Table 7
 – 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
Depression
24
HSP affecting learning or memory
24
My muscles are weak
21
Clonus (jumping feet or other muscle spasms)
19
Affected by bowel problems
16
Numbness
16
HSP affecting sexual function
14
HSP affecting speech
12
Leg/foot pain
11


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. Finally, muscles feeling stiff, bladder problems and back/hip pain affect approximately 40 respondents the most.

Symptom with Greatest Effect on Wellbeing
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 those answers where at least 30 respondents had given their wellbeing score.

Three of the symptoms show significant effects and a further three symptoms show effects which may be significant.

Symptoms which may be significant have been selected where the wellbeing score consistently decreases with increasing symptom effect and where the highest and lowest wellbeing score are more than 3 points from the average.

Table 8 – Symptom Wellbeing
Symptom
Major
Moderate
Minor
Don’t have
Depression
33.2
41.7
48.6
52.7
Stress
39.0
45.4
48.2
52.5
HSP affecting learning or memory
39.5
43.7
43.8
49.2
Poor co-ordination
42.3
45.3
46.0
50.5
Regular falls
42.6
44.1
46.9
50.7
Back/hip pain
42.6
47.0
47.4
49.3









This table shows that depression and stress have the greatest effect on wellbeing. For both 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. For cognitive effects, those that have these all or most of the time have a significantly lower wellbeing score and those that don’t have these have a higher wellbeing score, but the effects may not be significant.

For poor co-ordination, regular falls and back/hip pain there is a large range of wellbeing between those that have these as a major symptom and those that do not have, however the results may not be significant.

Change of symptoms with mobility
The data allows an examination of the change in symptoms with change in mobility, both in terms of the number of symptoms and the severity of symptoms. For this analysis the focus has been put on those symptoms which have the greatest effect. These comprise:
·         The 16 symptoms identified as the greatest effect, affecting at least 10 respondents
·         The 6 symptoms with the greatest effect on wellbeing
·         The 15 symptoms which affect at least 75% of respondents in any way
Altogether this is 21 of the 36 symptoms identified.

Table 9 – Symptom Change with Mobility
Mobility Score
Number of Symptoms
Severity of Symptoms
0-1
41% of respondents have 5-6 symptoms
36% of respondents have 7-9 symptoms
17% of respondents have 10-13 symptoms
6% of respondents have at least 14 symptoms
Respondents have
48% of symptoms with a minor effect
27% of symptoms with a moderate effect
25% of symptoms with a major effect
2-3
38% of respondents have 5-6 symptoms
41% of respondents have 7-9 symptoms
13% of respondents have 10-13 symptoms
8% of respondents have at least 14 symptoms
Respondents have
32% of symptoms with a minor effect
31% of symptoms with a moderate effect
37% of symptoms with a major effect
4-5
50% of respondents have 5-6 symptoms
34% of respondents have 7-9 symptoms
13% of respondents have 10-13 symptoms
3% of respondents have at least 14 symptoms
Respondents have
28% of symptoms with a minor effect
30% of symptoms with a moderate effect
42% of symptoms with a major effect
6-7
46% of respondents have 5-6 symptoms
35% of respondents have 7-9 symptoms
15% of respondents have 10-13 symptoms
4% of respondents have at least 14 symptoms
Respondents have
17% of symptoms with a minor effect
25% of symptoms with a moderate effect
58% of symptoms with a major effect
8-9
50% of respondents have 5-6 symptoms
32% of respondents have 7-9 symptoms
14% of respondents have 10-13 symptoms
4% of respondents have at least 14 symptoms
Respondents have
18% of symptoms with a minor effect
24% of symptoms with a moderate effect
58% of symptoms with a major effect

Of the 21 highest impact symptoms considered, all respondents had at least 5 and no respondents had more than 18. Across the mobility bands the number of symptoms tends to remain broadly the same.

With reduced mobility the effect of symptoms increases. In the early stages there are more symptoms with a minor effect, whereas further along the HSP journey there are more symptoms with a major effect. The progressive use of mobility aids seems to coincide with an increase in the severity of symptoms.

This finding is different from that identified in the 2013 survey. In the 2013 survey the analysis showed that there was an increase in the number of symptoms with increased use of mobility aids. The 2013 analysis showed that those who walk unaided tended to have no major symptoms, whereas this survey indicates that 25% of symptoms are major.

There are two likely potential reasons for this change. Firstly, the list of symptoms has increased from 13 up to 36, which perhaps indicates that the 2013 survey was not capturing the full picture of peoples symptoms. Secondly, the symptom list now includes general items like “I have difficulty walking” or “my muscles are stiff” which are perhaps likely to be noticed by someone in the earlier stages of HSP.

Comparison of Symptoms with Greatest Effect
It is interesting to observed that fatigue is the factor which is singled out by respondents as having the greatest effect, however depression is the symptom which the greatest influence on wellbeing. 

This figure shows the distribution in wellbeing for the 21 symptoms with the highest impact:




Longitudinal Change in Symptoms
13 respondents completed the symptoms questions of both this survey and the 2013 survey. The 2013 survey examined 13 symptoms and this analysis identifies changes in those 13 symptoms. 

These respondents fall into two groups. The first group, comprising 4 respondents report as being affected by the same symptoms in 2013 as 2018. On the whole these people report that some of the symptoms have gotten worse over the five year period whereas others remain the same. Interestingly, some symptoms are better for some people.

The second group, comprising 9 respondents, are those who have identified at least one new symptom over the five year period. Of the symptoms which were identified in 2013, some have stayed the same and some have gotten worse. Like the first group, some symptoms have got better for some people.

This analysis presents a different picture to that presented in 2013. In 2013 it appeared that the number of symptoms would increase with decrease in mobility, and that symptoms would similarly increase in their severity. Viewing changes in symptoms for one person over a five year period this shows either that some symptoms are intermittent or their perception of some symptoms varies from year to year.

This longitudinal analysis shows that overall the number of symptoms does tend to increase over time, but not all symptoms persist from year to year. 

Sleep

This survey sought to explore how HSP affects peoples’ sleeping patterns. To do this, respondents were asked 18 questions about sleep following a modified Global Sleep Assessment Questionnaire (GSAQ). Respondents were asked to score each question with the choice that best selected their situation. The scoring choices were:
·         Never
·         Sometimes
·         Usually
·         Always
Where a respondent indicates that that factor affects them usually or always it is indicative that they have an issue which affects their sleep quality. 290 respondents answered at least 14 of the sleep questions and are included in this analysis.

Overall Sleep Quality
The following table identifies how many of the 16 different sleep issues respondents have, i.e. where they are affected usually or always by that factor.

Table 10
 – Number of sleep factors
Number of sleep factors
Respondents
Percentage
0
57
20%
1
43
15%
2
36
12%
3
37
13%
4
29
10%
5
18
6%
6
21
7%
7
16
6%
8-10
36
9%
11-13
6
2%
14-16
2
1%


The majority of respondents are affected by at least one sleep factor. Approximately half of respondents are affected by 3 or more factors, and approximately half are affected by up to 2 factors. Grouping the factors together allows the number of sleep factors to be compared with wellbeing.

Table 11 – Number of sleep factors
Number of sleep factors
Respondents
Wellbeing
0 or 1
92 (35%)
50.4
2 or 3
73 (25%)
48.1
4 or 5
47 (16%)
43.2
6 or more
67 (23%)
39.9


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, but this effect may not be significant.

Review of Sleep Factors
Examination of the data allows the importance of the different sleep factors to be identified.

Table 12 – 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%
Did you snore loudly?
18%
Did other physical symptoms disturb you in your sleep?
16%
Did worries disturb you in your sleep?
16%
Did work or other activities prevent you from getting enough sleep?
15%
Did other factors disturb you in your sleep?
8%
Did you hold your breath, have breathing pauses, or stop breathing in your sleep?
8%
Did you have nightmares, or did you scream, walk, punch, or kick in your sleep?
8%
Did medications disturb you in your sleep?
7%

This table shows that 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.

93% of respondents usually or always sleep in a bed, and 1% of respondents usually or always sleep in a chair. The majority of the remainder of respondents indicate that they sometimes sleep in beds and sometime sleep in chairs.

A small number of respondents indicate that they never sleep in a bed and also never sleep in a chair, and conversely one person indicated that they always sleep in a bed and they also always sleep in a chair. None of these respondents provide any further details, aside from one respondent preferring to sleep on the floor.

For the question “Do other factors disturb you in your sleep?” a range of different answers were given, some of which matched the other questions whereas others included disturbance from a range of factors including light, noise, temperature, pets, children, animals and other people.

Variation in Sleep Factors with Mobility
There is no significant variation in sleep factors with mobility score, indicating that sleep factors are generally independent of mobility aids used.

Variation in Sleep Factors with Wellbeing
The sleep factors have been examined to identify if any have an effect on wellbeing. When reviewing this data it is not clear if a sleep factor affects wellbeing, or if wellbeing affects sleep, or if both sleep and wellbeing are affected by another factor.

Those whose sleep is disturbed by feeling sad/anxious, by medications or by worries have a wellbeing which is significantly below average. Those whose sleep is not disturbed by feeling sad or anxious have a better wellbeing, but not significantly. This factor is similar to depression being the overall HSP factor with the greatest effect on wellbeing.

Activities of Daily Living

Respondents selected the response which closely matched their situation for the six activities of daily living, as set out using the Katz Index of Independence in Activities of Daily Living (ADL). For each activity a respondent scores one point if they can undertake that activity independently and no points if they cannot.

Overall, a high score of six shows that the person is independent, and a low score of zero shows that the person is very dependent. Over half of respondents score six points and are fully independent, and that over one third score five points. The most common factor affecting people is their continence, their control of bladder or bowel.

Of the 84 of respondents who score 5 points, 74 are affected by the continence factor, with bathing (5 respondents) and dressing (four respondents) being the other two main factors.

Of the 16 of respondents who score 4 points, the most common combination is continence and bathing, with 9 respondents. Four respondents are affected by continence and dressing factors, and two are affected by bathing and dressing factors.

There are no clear patterns between the ADL factors and the use of mobility aids. There are people with 1 point,2 points and 3 points right through the range of mobility aid use. Similarly there are people affected by continence, bathing, dressing and toileting factors throughout the range of mobility aid use.

Variation in ADL with Wellbeing
Most of those who answered the ADL questions also answered the wellbeing questions. The results suggest that having one or two factors is not significantly different from having no factors. Where a respondent has 3 or more factors their wellbeing appears to be significantly lower, although this is a small sample size.

Looking at the factors individually, those who are affected by continence or transferring factors have a wellbeing which is not significantly different from average. Those who are affected by the bathing factor have a lower wellbeing, but perhaps not significantly. Those affected by dressing and toileting factors have a significantly lower wellbeing, although this is noted as being a small sample. 

Support Group Membership

Around 60% of respondents are members of a support group and 40% are not. The most common type of support groups identified were Facebook groups. Three key benefits people get from group membership are; getting to know other people with HSP, getting knowledge about HSP, and sharing stories. There is no difference in wellbeing between those that are members of support groups and those that are not.

The most common reasons given for not being in a support group were there not being a group near where respondents lived, not being aware of any support groups and not feeling the need to be a member of a support group.

There is no difference in wellbeing between those that are members of support groups and those that are not members of support groups.

Symptom Tracking

Half of respondents track their symptoms and half do not.

Almost all of those who do track symptoms described how they track those symptoms. The most common methods were; records produced by medical professionals, reviewing how things have changed from time to time and keeping a record/diary/log.

There is no difference in wellbeing between those that track their symptoms and those that do not.

Wellbeing Conclusions

Throughout this study the different factors which have been shown to have the greatest link with wellbeing have been identified. The tables below draw together the factors which have the strongest link 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.

Positive Effect
The factors below have been identified as having a wellbeing which is better than average.

Table 13 – Positive Wellbeing Factors
Factor
Respondents
Wellbeing
Not suffering from depression (symptoms)
79
52.7
Not suffering from stress (symptoms)
49
52.5
Not suffering from regular falls (symptoms)
34
50.7
Not suffering from poor-coordination (symptoms)
62
50.5
Being affected by up to 1 sleep factor (sleep)
100
50.4
Not having difficulty falling/staying asleep (sleep)
150
49.4
Not suffering from back/hip pain (symptoms)
44
49.3
Not affected by learning/memory issues (symptoms)
139
49.2

Negative Effect
The factors below have been identified as having a wellbeing which is significantly lower than average. Results with a small sample size (less than 20) have been put in brackets.

Table 14 – Negative Wellbeing Factors
Factor
Respondents
Wellbeing
Suffering from depression all or most of the time (symptoms)
43
33.2
Needing help using the toilet (activities of daily living)
10
(36.8)
Suffering from 3 or more ADL factors (activities of daily living)
10
(37.3)
Being sad/anxious interfering with sleep always/usually (sleep)
74
37.3
Needing help getting dressed (activities of daily living)
22
37.9
Medications affecting sleep always/usually (sleep)
18
(38.4)
Suffering from stress all or most of the time (symptoms)
68
39.0
Being affected by learning/memory issues all/most of the time (symptoms)
34
39.5
Having a rapid change in mobility over 5 year (mobility)
30
39.6
Worries affecting sleep always/usually (sleep)
63
39.8
Having six or more sleep factors always/usually (sleep)
70
39.9
Having SPG11 (diagnosis)
10
(40.4)

Four of these symptoms are related to mood; depression and stress overall, and sadness/anxiety and worries affecting sleep. Those who do not have depression or stress overall have a significantly higher wellbeing.

The Importance of Wellbeing
Many of the factors in these tables are not connected with mobility or other factors which are directly related to HSP. These factors may not form part of routine discussions with medical professionals which frequently focus on mobility, pain, continence and the other direct factors.

It is important to remember that it is possible to take action to improve some of these other factors. It is not necessary to accept, for example, that people are depressed, or cannot sleep well. Help or treatment is available for some factors, and getting such help/treatment may result in an improvement in wellbeing.

People should consult with a doctor or other medical professional to advise on the best course of action for any such action. Potential areas where changes could be made include:
·         Decreasing: Depression, stress, anxiety, worries
·         Lifestyle or other changes to improve sleep
·         Reductions in pain
·         Strength/balance training to reduce falls (or the impact of falls)
·         Activity/exercise programme to slow down reductions in mobility

Wellbeing is not solely about health and examining your HSP progress and symptoms. Improvements in wellbeing can be made in other areas, including friendships, hobbies, learning new things, being kind and being mindful.

Acknowledgements

First and foremost I need to thank all of the respondents who took time to respond to this survey, without these excellent answers this analysis would not be possible.

Special thanks are due to the Brazilian HSP group, ASPEH (https://www.aspehbrasil.org/) who took my Google translations one step further and hosted their own copy of the questionnaire in Portuguese.

Most of the recruitment has been through:
  • HSP support groups, who I thank for letting their members know about my surveys,
  • Many HSP groups on FaceBook, whose admins I thank for letting me post about this.



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