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;
- No mobility
effects
- Can walk without
aids but some effects
- Orthotics/AFO/FES
and/or Sticks/Poles/Crutches/Canes some of the time
- Sticks/Poles/Crutches/Canes
and Frame/Chair some of the time
- Sticks/Poles/Crutches/Canes
most of the time
- Sticks/Poles/Crutches/Canes
all of the time
- Rollator/Walking
frame most of the time
- Rollator/Walking
frame all of the time
- Wheelchair/Mobility
scooter most of the time
- Wheelchair/Mobility
scooter all of the time
In the remainder of
this paper, whenever “sticks” are referred to as a mobility aid, this term
includes poles, crutches and canes. Whenever “frames” are referred to this
includes both walking frames and rollators. Whenever “chairs” are referred to
this includes both wheelchairs and mobility scooters. Whenever AFO is referred
to 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.
