Saturday, 21 March 2020

HSP and Coronavirus / COVID-19

Mon 23rd March

Update from the Association of British Neurologists - Version 2, 22nd March:

"Patients with conditions that do not affect their swallowing or breathing muscles and in whom the immune system is working normally are not considered to be at increased risk from COVID-19."

"Experts ..... have attempted to estimate the risk associated with COVID-19 from each neurological condition......  It is difficult to accurately assess risk for every condition. The risk has been subdivided into three levels; low, moderate, and high."

"We recommend social distancing for all people with any neurological condition, their carers and family. We recommend self-isolation only for people in the high risk category."

"People with a neurological condition with low or moderate risk might be considered high risk if they have additional risk associated with other conditions affecting the lungs, heart, kidneys etc."

Table G in the advice puts HSP in the Low/Medium risk category.

View the full advice here: https://www.theabn.org/news/495261/ABN-Guidance-on-COVID-19-for-people-with-neurological-conditions.htm

Sat 21st March

OK, there's no getting away from it, Covid-19 is a big issue. Some readers will know that I am also chair of the UK HSP Support Group. I wrote to our members this week to advise them that we have postponed or cancelled group meetings for the next few months. We made this decision based on two factors: the UK Government advice around social distancing and minimising non-essential travel, and that those with underlying health conditions are at a higher risk of developing severe problems if they become infected.

I have spotted several people asking questions about HSP and Covid-19, so this post sets out the various information I have found and puts my perspective on it. Readers must remember that I am NOT a medical professional.

The UK advice is here: https://www.gov.uk/government/publications/covid-19-guidance-on-social-distancing-and-for-vulnerable-people/guidance-on-social-distancing-for-everyone-in-the-uk-and-protecting-older-people-and-vulnerable-adults

Those in the higher risk groups (relevant to HSP) are:
  • Those aged 70 or older (regardless of medical conditions)
  • Those under 70 with an underlying health condition (ie anyone instructed to get a flu jab as an adult each year on medical grounds)
  • Those with chronic neurological conditions, such as Parkinson’s disease, motor neurone disease, multiple sclerosis (MS), a learning disability or cerebral palsy

HSP is a neurological condition. What is not said is how you would determine if your HSP is chronic or not. It is my view that if you are able to carry on your life like any other member of society then your HSP is not chronic. If you spend most of your time in a wheelchair then your HSP is chronic. There is a scale between these two extremes, and whilst it is (to some degree) a choice if you consider your HSP to be chronic, my view is:
  • Constant/near-constant use of frames/chairs - more likely to be chronic
  • Frequently affected by pain from HSP - more likely to be chronic
  • Frequently affected by fatigue from HSP - more likely to be chronic
  • HSP affecting cognition/speech/swallowing (etc.) - more likely to be chronic
  • Feet/legs swelling up or sores/skin issues from HSP - more likely to be chronic
  • Use of catheter - more likely to be chronic
  • Significantly affected by stress/depression - more likely to be chronic
  • Regular falls/significant balance problems - more likely to be chronic

You should note that this is just my view. If you have just one of these then you may not consider your HSP to be chronic, but if you have more than one then I think it probably is. It was quite a challenge to decide which of the HSP symptoms to include in this list.

The risk of severe problems if infected increases when you have multiple conditions. Covid-19 is a respiratory virus, so if you live with HSP and Asthma or another respiratory condition then you are more likely to be in the higher risk group. Cardiovascular disease was shown in China data to be the underlying condition resulting in the most deaths (link below). Guidance from NHS indicates that factors which make you more likely to suffer from cardiovascular problems are; inactivity, being overweight, high blood pressure and high cholesterol levels (https://www.nhs.uk/conditions/cardiovascular-disease/). Unfortunately loss of mobility with HSP tends to result in higher levels of inactivity, which increases the risk of cardiovascular issues.

Given all these factors it is my opinion that those with HSP should start by considering themselves at higher risk of having severe problems if they are infected with Covid-19. People at the beginning of their HSP journey or who are not affected significantly may be able to justify that their risk is the same as the rest of the population.  

People should follow their governments advice about social distancing, minimising non-essential travel, working at home, staying indoors, avoiding big groups. Those who:
  • are affected by HSP severely, or 
  • have HSP and at least one other health condition, or
  • are over 70 and have HSP irrespective of severity
should seriously consider if they need to self-isolate for a while.

If you are not sure about this, then it might be a good time to book a call in with your GP/Doctor/Neurologist/HSP specialist to identify the best course of action. This link gives the UK advice on self-isolation: https://www.nhs.uk/conditions/coronavirus-covid-19/self-isolation-advice/.

I realise that people with HSP are often affected by depression, low mood, loneliness, isolation (etc.) It is important to keep in touch with people during this time, so I encourage people to communicate with friends and family. Use whatever method seems right, e-mail/social media/letter/phone or perhaps something more unusual. Avoid face-to-face contact.

Also, whether you are self-isolating or social distancing don't forget to follow your stretching routines and keep as active as possible. 

If you are not a member of an HSP support group then this might be a useful thing to consider. There are 'real' groups in Europe (http://www.eurohsp.eu/), USA (https://sp-foundation.org/), Australia (https://hspersunite.org.au/) and other places. There are virtual groups on Facebook (https://www.facebook.com/search/groups/?q=hereditary%20spastic%20paraplegia&epa=SERP_TAB), Rare Connect (https://www.rareconnect.org/en/community/hereditary-spastic-paraplegia), Patients Like Me (https://www.patientslikeme.com/conditions/hereditary-spastic-paraplegia) and other places.

As far as information about Covid-19 goes, I like this page from Information is Beautiful: https://informationisbeautiful.net/visualizations/covid-19-coronavirus-infographic-datapack/ This presents both data from China and Italy, it confirms that it affects older people more than younger people, and that those with underlying health conditions make up the bulk of those who have died.


Sunday, 8 March 2020

Symptoms update - Shadows and Hills

I have been having a bit of a turbulent time in recent months, which has led to me being more stressed, more depressed and more tired than normal. Therefore, this symptom update should be read with a small pinch of salt as I wont know for a while if these are short-term changes as a result of my turbulence or part of the long-term HSP journey.

Shadows

I happened to be walking back home one evening in the dark. As I walked under street lights my shadow was cast down on the ground in front of me. It was interesting to see how uneven my gait has become. I know that my gait is changing over time, but it was quite a surprise to see how much it has changed.

Hills

I have managed to get out on my bike recently, and I find that my uphill speed is getting very slow - by which I mean easily 4-6 km/h. I was quite surprised to by cycling up a hill on the way to work and was overtaken by a runner on the pavement. I will have to have a look at my biking statistics to see if my average speed is coming down or not. This is not as straightforward as you may expect, as there is a mix of riding in the list - some off-road stuff which is slower anyway, and some cycling with the boys at walking speed on the way to/from school.

I think that both the shadows and hills factors occur because I am more tired than usual. After a busy day I feel that I am more tired than I used to be, and I am beginning to feel the need to sit down and have a small rest.


Saturday, 29 February 2020

2019 Survey Results

Once again it is Rare Disease Day, and I am happy to publish the results of my 2019 Survey. This post is an abbreviated version of the analysis. for the full analysis, access this PDF: https://drive.google.com/open?id=1Vrh5bd8ss0XDU_DR39kEpneo-3YZuaGm

Thanks are due to everyone who answered questions, manages HSP patient and facebook groups, and helped persuade people to take part. Several also helped by reviewing the translated questions.


Introduction

This post presents the results of my seventh survey, launched in September 2019, which repeats some of the questions asked in my 2014 survey, and offers the opportunity to look at some results longitudinally. This survey also marks development from last years multi-language question sheets, I set up the survey in different languages so people could read and answer directly in their own language. As previously, I have used Google Translate to translate questions and answers between languages for this analysis.

Respondents

There were 367 respondents who completed the survey, predominantly from the USA, UK and Brazil. Table 1 shows the distribution of respondents’ locations;

Table 1 - Location of respondents
Location
Respondents
USA or Canada
82
South America
81
UK or Ireland
79
France
40
Other Europe
55
Australia
25
Rest of world
5

There have been many more responses to this survey than my previous ones. The most notable change from previous years is a large block of responses from Europe, who completed the questions in French, Dutch, Spanish and Italian. Of the other locations, the distribution of people around the world is similar, including those from Brazil answering in Portuguese.

A brief analysis shows that 150 people who completed this survey had also completed at least one of my previous surveys, representing about 40% of respondents, the proportion is increased from last year. A small number of people, four, have completed all seven surveys. There are 22 respondents who completed both the 2014 survey and this 2019 survey.

HSP Analysis

The first questions in the questionnaire asked if people had HSP. 365 respondents answered this question, selecting from the following options:

Table 2 – HSP Diagnosis
HSP Diagnosis
Respondents
I have HSP - genetic test
199
I have HSP - other diagnosis
123
I do not know if I have HSP
7
I am answering on behalf of someone else with HSP
36

There were 201 respondents (55%) who knew which type of HSP they had, with the most common answer being type 4. This is similar to last year, SPG 7 was second and SPG 11 took third place.

5) Wellbeing

In order to assess the wellbeing of people with HSP respondents completed the Warwick-Edinburgh Mental Well-being scale (WEMWBS). The scale is scored by summing the response to each item answered on a 1 to 5 Likert scale. The minimum scale score is 14 and the maximum is 70.

The total score is higher for those with a better wellbeing. The average score for the England and Scotland population is 51-52 with most scoring between 41 and 59.

There were 336 respondents who answered this question, with the average wellbeing score of 47.4, which is slightly higher than the average score of 45.9 for people with HSP reported in both 2017 and 2018. 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”.

As there is a wide spread of locations for respondents, the wellbeing for each geographical area has been calculated to identify if there is potential for cultural differences to give rise to different wellbeing scores.

Table 6 – Regional Wellbeing
Area
Wellbeing
UK and Ireland
45.7
USA and Canada
48.0
South America
48.6
France
46.7
Other Europe
49.0
Australia
45.4

This shows that people in Australia, the UK and Ireland have a slightly lower wellbeing and those in Europe aside from France, UK and Ireland, and those in South America have a slightly higher wellbeing. All scores are within 3 points of the average suggesting that any regional differences are not significant.

Although it is a small sample size, those who do not know if they have HSP have wellbeing of 42.1, more than 5 points below average, suggesting that uncertainties around diagnosis have a negative impact on wellbeing. Having or not having other family members with HSP did not influence wellbeing.

Mobility Analysis

All 367 respondents gave answers to this question. The data allows identification of the mobility aids used the most regularly. 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 about 5%. Broadly these results are similar to those from previous years. Table 7 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 7 – Use of mobility aids
Mobility Aids Used - Summary:
Respondents
Percentage
Using sticks/poles/crutches/canes
186
51%
Using Wheelchair/Mobility Scooter
141
38%
Using Walking Frame/Rollator
77
21%
Using Orthotics/AFO/Insoles
99
27%
Using FES
17
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;
  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 orthotics, AFO and insoles. The results are simplified into five broader groups;

Table 8 – Overview of mobility aids used
Mobility Aids Used - Overview:
Respondents
Percentage
Mobility Score
Wellbeing
Those without aids
71
19%
0-1
47.9
Those who use mobility aids some of the time
88
24%
2-3
47.0
Those who use sticks most/all of the time
101
28%
4-5
49.0
Those who use frames most/all of the time
39
11%
6-7
45.4
Those who use chairs most/all of the time
68
19%
8-9
46.2
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 of numbers of people between bands.

The wellbeing scores do not vary significantly across types of walking aid being used. This result is similar to the result from 2017, but different from that from 2018 which showed that those using wheelchairs all or most of the time had a lower wellbeing. The distribution of wellbeing scores is shown for all respondents in the following figure, split by mobility score.

 
Medication

I wanted to explore the medication which people take for HSP. I wanted to find the reasons that people take medicines for, and how effective they perceive those medicines to be. I allowed people to enter whatever they wanted and enter details for up to 5 medicines, with a comment box to note any others.

People answered the following questions;
  • What is the name of the medication?
  • Which HSP symptoms do you take the medication for?
  • How do you take the medicine?
    • Choice of: By mouth, Injection, Pump, On the skin, Other
  • Do you notice benefits from the medication?
    • Choice of: High levels of benefit, Medium levels of benefit, Low levels of benefit, Not sure, No benefit noticed
  • Have the benefits changed over time?
  • Do you notice side effects from the medication?
    • Choice of: Lots of side effects, Some side effects, No side effects
  • What are the main side effects you notice from the medication?
  • Why did you start taking the medication?

Initially, a summary of all medication is given, looking at overall numbers of medication and why these are being taken. Each of the main groups of medication is then examined, and an overall summary is given at the end of the section.

Medication – Overall Summary

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

Table 12 – Number of respondents, number of medicines against mobility.
Mobility Score
No medicine
1 Medicine
2 Medicines
3 Medicines
4 Medicines
5 Medicines
6 Medicines
Total
0-1
16
26
12
0
2
1
1
58
2-3
14
28
19
9
2
5
1
78
4-5
10
35
22
8
8
2
0
85
6-7
4
9
10
5
2
5
1
36
8-9
2
16
19
15
3
6
2
63
Total
46
114
82
37
17
19
5
320
Wellbeing
50.6
48.9
46.8
44.7
44.2
44.8
37.6


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

The data shows that those who do not take medicine have a significantly better wellbeing and those who take the larger number of medicines have lower wellbeing.

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

Table 13 – Medication Groups
Symptom
Number of medicines
Number of people
Spasticity/spasms
21
327
Pain/nerve pain
33
109
Bladder
12
64
Depression/anxiety
10
17
Bowel
3
8
Sleep/fatigue
2
2
Other
19
23
Total
100
550

The table shows that there are more people taking medicine for spasticity or spasms than in the other groups, whereas there is a higher number of types of medicine used to treat pain or nerve pain.

The following table indicates how many people are taking each of the most commonly used medicines, showing medicines where at least 10 people are using them.

Table 14 – Most common medicines
Medication
No. People taking medication
Baclofen (muscle relaxant)
178
Botulinum toxin / Botox (muscle relaxant)
26
Gabapentin / Neurontin (anticonvulsant)
24
Clonazepam (muscle treatment)
23
Oxybutynin (bladder)
22
Tizanidine / Zanaflex (muscle relaxant)
21
Pregablin / Lyrica (anticonvulsant)
14
Paracetamol (pain)
13
Diazepam (muscle/anticonvulsant)
12
Amitriptyline / Elavil (pain/antidepressant/bladder)
11
Mirabegron (bladder)
11
Solifenacin (bladder)
10

This shows that baclofen is by far the most commonly used medication for HSP with around half of people taking it, and accounting for around one third of all medication taken. All of the other medications are taken by less than 10% of people, and account for less than 5% of medication taken.

Spasticity and Spasms

Almost 60% of the medication being taken is used to treat spasticity and spasms, the key features of HSP. The biggest proportion of this group of medications comprises people taking Baclofen. The full list of medications in the group is shown in the following table, with descriptions of the medications taken from the UK NHS, drugs.com and other websites.

Table 15 – Spasticity and Spasm Medication
Medication
Description
People taking medication
Baclofen
Baclofen reduces muscle tone and unwanted muscle contractions. This helps to reduce painful muscle spasm.
178
Botulinum toxin A / Botox / OnabotulinumtoxinA
Botulinum Toxin Type A is used to treat muscle spasms. It works by stopping muscle contraction in the muscle that it is injected into. This prevents muscle spasm. It also blocks pain signals, which indirectly block the development of a migraine.
26
Gabapentin / Neurontin
Gabapentin is an anti-epileptic medication, also called an anticonvulsant. It affects chemicals and nerves in the body that are involved in the cause of seizures and some types of pain. It is also used to treat restless legs syndrome (RLS).
24
Clonazepam
Clonazepam belongs to a group of medicines called benzodiazepines. It's used to control seizures or fits due to epilepsy, involuntary muscle spasms, panic disorder and sometimes restless legs syndrome.
23
Tizanidine / Zanaflex
Tizanidine reduces muscle tone. This helps to reduce painful muscle spasm. The effect of Tizanidine usually lasts for a few hours.
21
Diazepam
Diazepam is a medicine which helps to control feelings of anxiety. It makes people feel less agitated and less tense. It also acts as a sedative and an anticonvulsant. It can help to relieve muscle spasm.
12
Amitriptyline / Elavil
Amitriptyline is a tricyclic antidepressant. It is also a medication that is used in management of long term (chronic) pain, especially nerve pain. It can also be used for improving sleep. It is prescribed in low doses for managing pain and is not addictive. Amitriptyline is also used for the treatment of bedwetting in children
11
Dantrolene
Dantrolene is a muscle relaxer that is used to treat muscle spasticity (stiffness and spasms) caused by conditions such as a spinal cord injury, stroke, cerebral palsy, or multiple sclerosis.
9
Ziclague
Ziclague is intended for supporting treatment in states of muscle spasticity. It relaxes the muscles (decreases muscle tone) in a dose-dependent manner.
7
Cyclobenzaprine / Flexeril
Flexeril is a muscle relaxant. It works by blocking nerve impulses or pain sensations that are sent to your brain.
5
Dalfampridine / Ampyra / Fampridine
Ampyra is used to improve walking in patients with multiple sclerosis (MS). It produced an increase in walking speed in clinical trials. Exactly how it works is not known. It is thought to increase nerve function. It reduces fatigue.
5
L-DOPA / Levodopa
Levodopa is used to treat the stiffness, tremors, spasms, and poor muscle control of Parkinson’s disease.
4
Amantadine
Amantadine is a drug used in the treatment of fatigue in multiple sclerosis. Amantadine may reduce involuntary movements (dyskinesia) caused by other Parkinson’s drugs. Amantadine can also help to reduce stiffness you may experience in your muscles.
3
Lorazepam
Lorazepam belongs to a group of drugs called benzodiazepines. It affects chemicals in the brain that may be unbalanced in people with anxiety. Lorazepam is used to treat anxiety disorders and seizure disorders and can be used for relaxation of excessive muscle rigidity and contractions.
2
Benserazide
Benserazide is a peripheral decarboxylase inhibitor that increases the amount of levodopa crossing into the brain and its subsequent conversion to dopamine.
1
Thiocolchicoside
Thiocolchicoside (Muscoril, Myoril, Neoflax) is a muscle relaxant with anti-inflammatory and analgesic effects.
1
Tolperisone
Tolperisone (trade name Mydocalm among others) is a centrally acting skeletal muscle relaxant used for the treatment of increased muscle tone associated with neurological diseases.
1
Dronabinol
Dronabinol, also known as Marinol and Syndros, is a trade name for a specific form of tetrahydrocannabinol, sold as an appetite stimulant, antiemetic, and sleep apnea reliever. It may possess analgesic, antispasmodic, and muscle relaxant activity.
1
Nortriptyline
Nortriptyline is a tricyclic antidepressant. It affects chemicals in the brain that may be unbalanced in people with depression. Nortriptyline is used to treat symptoms of depression.
1
Mantidan
Mantidan is an antidyskinetic medicine. It is used to treat Parkinson's disease and its symptoms, including dyskinesia (sudden uncontrolled movements). By improving muscle control and reducing stiffness, this medicine allows more normal movements of the body as the disease symptoms are reduced.
1
Carbamazepine
Carbamazepine is an anticonvulsant. It works by decreasing nerve impulses that cause seizures and nerve pain, such as trigeminal neuralgia and diabetic neuropathy. Carbamazepine is also used to treat bipolar disorder.
1
Riluzole
Riluzole is used to treat amyotrophic lateral sclerosis (ALS). Riluzole helps to slow down the worsening of the disease and prolong survival.
1

Of these medicines, those with at least 20 people taking are examined in further detail. All these medications are reviewed in respect of published HSP treatment information at the end of Section 8.

Baclofen
There are 178 respondents who indicate that they take Baclofen for HSP symptoms. The symptoms, as described by respondents, fall into two main areas; spasticity for 123 respondents,  clonus for 22 respondents, stiffness for 10 respondents, and there are 14 respondents who describe another symptom which they take baclofen for. The majority (156) indicate that they take baclofen in tablet form. There were 18 people who indicate that they take it using a pump.

Three quarters of people note that they get a benefit from this medicine. One fifth indicate that benefits are high, and one third each indicate that benefits are medium or low. 15% indicate that they don’t know if they get any benefits, and the remainder (less than 10%) indicate that they do not get any benefits from Baclofen. Over half of people say that the benefits of Baclofen have not changed over time, around 10% do not know if benefits have changed and the remaining 40% saying benefits have changed, of which one quarter (10%) of people indicate that the benefits have gotten smaller over time.

Looking at side effects, half of respondents indicate that they do not get any side effects, around 45% indicate that they get some side effects, with the remaining 5% getting many or lots of side effects. The key side effects noted are sleepiness, increased fatigue or increased tiredness. Other side effects mentioned include bowel/urine issues, nausea, effects on focus and mood, and increased pain.
  
There were 46 other respondents who have taken baclofen in the past but have stopped taking it now. 32 of these described why they no longer take baclofen; 14 describe that the were not noticing any benefit from it, 13 indicated that they didn’t want the side effects, and there were 5 who thought baclofen had an adverse effect on their strength or gait.

Baclofen is one of the recognised treatments for HSP. The first paper to mention the use of Baclofen to treat HSP seems to date from 1989, in the Journal of the Medical Association of Thailand (PubMed ID: 2738487). Papers continue to be published on this into 2018, with just over 25 papers altogether.

Botulinum Toxin Type A
There are 26 respondents who indicate that they take Botox for HSP symptoms. The symptoms, as described, are predominantly for spasticity with a few people indicating they take it to treat their bladder, and three respondents describing taking it for other symptoms. All indicate that their botox is injected.

The majority of people note that they get a benefit from this medicine. Half indicate that benefits are high, one third indicate medium benefits, with the remainder indicating low benefit.

Looking at side effects, most respondents indicate that they do not get any side effects, with a small number indicating some side effects, principally around how botox affects their gait.

Botox is one of the recognised treatments for HSP. The first paper to mention the use of Botox to treat HSP seems to date from 2003, in the journal Developmental Medicine & Child Neurology (PubMed ID: 14667075). Papers continue to be published on this into 2019, with 8 papers altogether.

Gabapentin
There are 24 respondents who indicate that they take Gabapentin for HSP symptoms. The symptoms, as described by respondents, are predominantly for pain and cramp, with a few indicating that they take it for spasticity. All indicate that their gabapentin is taken orally.

Most people note that they get a benefit from this medicine. Half indicate that benefits are medium, and around one quarter each indicating high or low benefits, with the remainder not sure.

There is a mix of responses around side effects, with some indicating lots of side effects and others indicating no side effects. The side effects mentioned most often are around sleepiness, increased fatigue or increased tiredness.

There are 11 respondents who indicated that they have taken Gabapentin in the past but no longer take, with the main reasons given being around side effects.

Gabapentin is one of the recognised treatments for HSP. The first paper to use Gabapentin seems to date from 2007 in the European Journal of Neurology (Pubmed ID: 17539946). There are papers into 2019.

Clonazepam
There are 24 respondents who indicate that they take Clonazepam for HSP symptoms. The symptoms, as described by respondents, are predominantly for spasms, with a few indicating that they take it for sleep, and other symptoms. All indicate that their clonazepam is taken orally.

Most people note that they get a benefit from this medicine. One third each indicate that benefits are medium or high, with the remainder showing low benefits, not sure or no benefit.

There is a mix of responses around side effects, with some indicating lots of side effects and others indicating no side effects. The side effects mentioned most often are around sleepiness or increased fatigue, with others showing mood swings or weakness.

Clonazepam does not feature in published papers relating to HSP.

Tizanidine / Zanaflex
There are 21 respondents who indicate that they take Tizanidine for treatment of HSP symptoms, for both spasticity and spasms. All indicate that their Tizanidine is taken orally.

Most people note that they get a benefit from this medicine. Three sevenths indicate medium benefits, with another one seventh noting high benefits.  The remaining three sevenths each indicate that benefits are low, they are not sure about benefits or they get no benefit.

There is a mix of responses around side effects, with some indicating lots of side effects and others indicating no side effects. The side effects mentioned most often are around sleepiness or increased fatigue, with others showing issues with balance, pain or constipation. 12 respondents indicated that they have previously taken Tizanidine but no longer take it, either due to side effects or to no perceived benefits.

Tizanidine is reported as having benefits for spastic paresis in 1982, in the Journal of the Neurological Sciences (PubMed ID: 7035623). It is mentioned in passing in treatment of SPG7 and SPG3A, but no trials are reported.

Pain

Respondents taking medicine for pain form the second largest group of around one fifth of all the medication being taken. The pain medication being taken falls into two main groups, one group is for pain, whereas the other group is for nerve pain, pain from spasms, and other HSP symptoms.

The first group (pain) includes common pain medication, some of which is available over the counter, e.g. paracetamol and ibuprofen, and others are prescription medication like fentanyl and morphine. I have not undertaken further analysis on this group.

The second group includes some medications that are used to treat effects of HSP, and the following table shows a subset of these medications, including those taken by the highest numbers of people and those where there are reports of using it to treat HSP.

Table 16 – Pain Medication
Medication
Description
People taking medication
Pregabalin/ Lyrica
Pregabalin is an anti-epileptic drug, also called an anticonvulsant. It works by slowing down impulses in the brain that cause seizures. Pregabalin also affects chemicals in the brain that send pain signals across the nervous system. Pregabalin is also used to treat peripheral and central neuropathic pain.
14
Duloxetine
Duloxetine is an antidepressant medicine. It's used to treat depression and anxiety. It's also used to treat nerve pain, such as fibromyalgia, and can be used to treat stress urinary incontinence in women.
9
Pramipexole
Pramipexole is medication used to treat Parkinson's disease and restless legs syndrome. It may be used alone or together with levodopa. It is taken by mouth.
7
Oxycodone
Oxycodone is an opioid medication used for treatment of moderate to severe pain. It is usually taken by mouth, and is available in immediate-release and controlled-release formulations.
3
Nabilone
Nabilone is a synthetic cannabinoid with therapeutic use as an antiemetic and as an adjunct analgesic for neuropathic pain.
1
Nabiximols
Nabiximols is intended to alleviate neuropathic pain, spasticity, overactive bladder, and other symptoms of multiple sclerosis.
1

Given the sample sizes, analysis has not been undertaken for those medications but all these medications are reviewed in respect of published HSP treatment information at the end of Section 8

Bladder

Respondents taking medicine for bladder issues forms the third largest group of around 1/8 of all the medication being taken. The three medications used by the most are Oxybutynin, Mirabegron and Solifenacin. Eight other medications were mentioned, taken by 4 or fewer people. An analysis of bladder medication has not been undertaken as part of this analysis.

Depression

Respondents taking medicine for depression forms the fourth largest group of around 3% of all the medication being taken. There are ten different antidepressant medications being taken by the 17 respondents who indicate taking this type of medication. These sample sizes are too small to allow further analysis to be undertaken.

Other Symptoms

Respondents taking medicine for other symptoms forms the remainder of medication being taken, totalling 6%. This includes 8 respondents taking medication for bowel issues, 3 respondents take medication to help sleep or reduce fatigue, as 23 others who take a range of medication for a variety of reasons. Several of these are worthy of note:

·         Three respondents are taking Noscapine with the aim of stopping the progress of HSP
·         Three respondents take Omeprazole to protect their stomach/gut from other drugs they are taking.
·         One respondent takes Olanzapine to improve their cognitive function
·         One respondent takes Nuedexta to treat the pseudobulbar effect (a less common HSP symptom).
·         One respondent takes Liraglutide to help with weight loss to reduce the load on their legs.
·         One respondent takes fusidic acid to treat skin problems bought on by HSP.

Medication Review

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

Table 17 – HSP Treatment Review
Medication
People taking medication
HSP Treatment? (HSP groups)
HSP Treatment? (PubMed)
Baclofen
178
Yes – SPF
Yes, many
Botulinum toxin A / Botox
26
Yes – SPF
Yes, many
Gabapentin
24
Yes – SPF
Trial: 17539946
Clonazepam
23
Yes – SPF
No
Oxybutynin
22
Yes – Gene Review
No
Tizanidine / Zanaflex
21
Yes – SPF
Trial: 7035623
Pregabalin
14
Yes -Review
No
Diazepam
12
Yes – SPF
Patient: 2803825
Amitriptyline
11
Yes - SPF
No
Mirabegron
11
Yes – Gene Review
No
Solifenacin
10
Yes – Gene Review
No
Dantrolene
9
Yes - SPF
Review: 20862796
Duloxetine
9
Yes - PLM
No
Ziclague
7
Yes - ASPEH
No
Pramipexole
7
Yes - HSPRF
No
Cyclobenzaprine
5
Yes - Review
No
Dalfampridine / Ampyra
5
Yes - HSPRF
Trial: 25808501
L-DOPA / Levodopa
4
Yes - TWS
Patient: 16463348
Amantadine
3
No
Patient: 16463348
Oxycodone
3
Yes - SPF
No
Noscapine
3
Yes - HSPRF
Screen: 24857849
Omeprazole
3
No
No
Lorazepam
2
No
No
Benserazide
1
No
No
Thiocolchicoside
1
No
No
Tolperisone
1
No
No
Dronabinol
1
No
No
Nortriptyline
1
Yes - SPF
No
Mantidan
1
No
No
Carbamazepine
1
No
Patient: 3352913
Riluzole
1
Yes - SPF
No
Nabilone
1
No
No
Nabiximols
1
Yes - HSPRF
No
Olanzapine
1
No
Patient: 22118709
Nuedexta
1
No
No
Liraglutide
1
No
No
fusidic acid
1
No
No

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

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

Exercise and Relaxation

The next section of the survey looked at respondents exercise and relaxation routines. Overall 362 respondents answered this question, although not all respondents answered the question for all of the activities. There are at least 249 answers for each activity.

The following table shows the distribution of answers given, and respondents who undertake activities weekly or more frequently are grouped together as undertaking that activity “regularly”, and the percentage of respondents is shown.

Table 18 – Exercise and Relaxation

Multi-Per-Day
Daily
Weekly
Monthly
Occasionally
Dont
Total
Regular
Stretches
45
138
74
2
47
31
337
76%
Walking/Running
42
101
44
1
28
85
301
62%
Physiotherapy
7
25
119
14
48
93
306
49%
Music
44
46
28
12
34
103
267
44%
Cycling/Manual wheelchair
12
37
42
7
35
146
279
33%
Weights
4
22
39
6
31
167
269
24%
Other
12
8
18
7
14
105
164
23%
Swimming
5
9
45
7
65
152
283
21%
Cardiovascular gym machines
2
21
30
2
20
187
262
20%
Yoga/Pilates/Tai Chi
3
13
39
4
21
192
272
20%
Gardening
3
14
34
13
40
159
263
19%
Massage
6
8
29
16
42
170
271
16%
Sauna/Hot bath/Steam room
5
6
21
6
36
187
261
12%
Workout routine/DVD/video
2
5
7
2
15
230
261
5%
Aromatherapy
2
6
0
1
12
228
249
3%
Ball/racket sports
2
0
6
3
8
236
255
3%
Dancing
1
1
5
2
19
224
252
3%
Power plate/Circulation booster
0
4
3
0
11
236
254
3%
Acupuncture
0
1
2
7
21
221
252
1%


This shows that stretches are the most common form of exercise being undertaken with over 90% of respondents doing this, and over 75% doing this regularly. Other activities which are commonly undertaken include walking/running, physiotherapy, music and cycling/manual wheelchair with more than one third doing regularly. A more in depth analysis of these activities is given below. Additionally, weights, swimming, cardiovascular gym machines, and yoga/pilates/tai chi are regularly undertaken by more than a fifth of respondents.

Overall, 339 respondents undertake at least 1 activity regularly and 23 respondents do not undertake any of the activities regularly.  There are 4 respondents who indicated that they don’t take part in any of the activities listed at all, and didn’t indicate any other activities which they do take part in. Their mobility score ranges between 4 and 9.

Examining the set of answers for those who indicated “Other”, things that are being undertaken on a daily basis include: bowling, VR/video games, arts and crafts, painting/drawing, meditation, knitting, musical instruments, prayer and sculpting. Things that are generally being undertaken on a regular basis include: archery, pets, walking dogs, photography, reading, working, movies, fencing, mindfulness, cooking, learning languages, bird watching, skiing, driving, diving, singing and shopping,

Wellbeing
The following table shows the activities and frequencies with the five highest and three lowest wellbeing scores where at least 20 people take part.

Table 19 – Wellbeing from Exercise and Relaxation
Activity
Frequency
Wellbeing
Cardiovascular gym machines
Daily
51.0
Music
Weekly
50.8
Music
Occasionally
50.7
Gardening
Weekly
50.3
Acupuncture
Occasionally
50.2
Cycling/Manual wheelchair
Weekly
44.8
Yoga/Pilates/Tai Chi
Occasionally
44.5
Walking/Running
Occasionally
44.5



The table shows that those who use cardiovascular gym machines on a daily basis have the highest wellbeing. Those who listen/take part in music on a weekly or occasionally basis also have a wellbeing which is more than 3 points better than average. Weekly gardening and occasional acupuncture are shown to have higher wellbeing. Weekly cycling/manual wheelchair, occasional yoga/pilates/tai chi and occasional walking/running are shown to have lower wellbeing.

All other activities and frequencies fall between this range. Looking at music as a whole, those who don’t listen/take part in music have a wellbeing of 45.8, more than 5 points below those who do this at least weekly. Similarly those who go walking/running weekly have a wellbeing of 49.1, almost 5 points higher than those who do this occasionally.

The questions do not allows respondents to give reasons why they choose to do things at a certain frequency, and so conclusions about how the frequency of activities affect wellbeing cannot be drawn. My supposition is that people who regularly undertake an activity they enjoy have a higher wellbeing than those who do not, as evidenced with the cardiovascular gym machines, music and gardening activities.

It is more difficult to identify to draw conclusions about how activities affect the lower end of wellbeing scale. People may have lower wellbeing because they are not able to undertake the activities they enjoy as often as they would like to, as shown by those who walk/run weekly having a higher wellbeing than those who do this occasionally.

People described activities which they would like to be able to do, but cannot. The most common answer was running with 31 people who would like to do this, other common answers included hiking, swimming, walking, dancing and cycling. Additionally, people also described activities which they have had to stop doing as a result of their HSP. The most common answer was walking with 41 people who have stopped doing this. Other common answers include running, hiking, working, cycling and sports generally.   

Most Important Activities
Respondents were asked to identify those activities which they felt were most important for exercise, relaxing and flexibility. People identified the most important activities for exercise to be stretching, walking and aerobic activities. Music and stretches were identified as most important for relaxing. Stretches and physiotherapy were identified as most important for flexibility.

Summary of Exercise and Relaxation Results
People who do not use mobility aids at all (mobility score 0 or 1) have regular activities which are likely to include walking or running and stretches. Many respondents also do physiotherapy and music. Other activities undertaken regularly include yoga/tai-chi/pilates.

Those who use mobility aids some of the time (mobility score 2 or 3) regular activities are likely to include stretches and walking/running. Many respondents also take part in physiotherapy and music. Other activities undertaken regularly include cycling/manual wheelchair and weights.
           
When people use sticks most or all of the time (mobility score 4 or 5), regular activities are likely to include stretches and walking/running. Many respondents also take part in physiotherapy and music. Other activities undertaken regularly include cycling/manual wheelchair and weights.

Where people use walking frames most or all of the time (mobility score 6 or 7), regular activities are likely to include stretches. Many respondents also take part in walking/running and physiotherapy. Other activities undertaken regularly include cycling/manual wheelchair, weights, cardiovascular gym machines and music.

For those who use wheelchairs most or all of the time (mobility score 8 or 9) regular activities are likely to include stretches. Many respondents also take part in cycling/manual wheelchair, physiotherapy and music. Other activities undertaken regularly include walking/running.

Overall, these patterns are similar to those reported in 2014.

Life-space Tracking

Respondents described how far they move about and how often they do this. There are 326 respondents who answered this question. 

Table 28 – Life space tracking
Frequency
Leave bedroom
Go outside
Local neighbourhood
Local town
Further than town
Every day
318
231
139
69
35
5-7 days a week
5
51
54
34
18
2-4 days a week
2
31
52
50
26
Weekly
1
10
46
64
29
Monthly
0
0
11
50
77
Less often than monthly
0
2
7
34
106
I don’t do this
0
1
17
25
35

  • This shows that 98% of people with HSP leave the room they sleep in every day. There are 2% of people who leave this room less frequently, but all leave at least weekly.
  • 71% of respondents go outside every day, and 25% go outside multiple times per week. There are 3% who do this weekly and 1% who do this less often than monthly or not at all.
  • There are 43% of people who go to their local neighbourhood every day, and 33% who go multiple times per week. 19% go weekly or less often, and 5% who do not go at all.
  • When travelling beyond the local neighbourhood, 21% do this every day, 26% do this multiple times per week, 45% doing this less often and 8% not doing this at all
  • Travel further afield occurs less frequently, with 11% doing this every day, 14% doing this multiple times per week, 65% going less often and 11% not going at all.

·       Examining the results when split by mobility scores shows no clear patterns, indicating that travel decisions are not dependent on mobility. The wellbeing scores have been calculated where there are more than 20 people in a group:

Table 29 – Life space tracking - wellbeing
Frequency
Leave bedroom
Go outside
Local neighbourhood
Local town
Further than town
Every day
48.1
49.2
50.0
51.6
51.7
5-7 days a week
-
45.0
47.2
48.5
-
2-4 days a week
-
47.4
47.3
48.3
49.0
Weekly
-
-
47.0
47.3
49.9
Monthly
-
-
-
45.5
47.1
Less often than monthly
-
-
-
47.3
46.8
I don’t do this
-
-
-
46.7
48.5

The analysis shows that the highest wellbeing is found in those people who travel the furthest every day. All other situations are within 3 points of the average score, so it is interesting to note that those who can do things every day have a higher wellbeing than those who cannot, but there are no clear patterns beyond this.

Various interesting comments are made about this, with several indicating that they need help using aeroplanes. Many have adapted cars or mobility scooters and can travel independently whereas others rely on another to drive them places. Others note that they need help with transfers, and rely on this to move anywhere, whereas others are children and rely on their parents/carers to help them get around. Some people need help lifting and carrying things rather than getting around, and some indicate that they don’t get out as much as they would like because they have caring responsibilities.

Life with HSP

In recent surveys I have asked if people considered themselves disabled or not. I added to that with a set of similar questions exploring life with HSP.

Do you Consider Yourself to be Disabled?
There are 365 respondents who answered this question, as shown in the following grid. Wellbeing scores are also calculated and shown in brackets where there is a small sample size (less than 20 people), and are not shown for very small samples (less than 10 people).

Table 31 – Identify as Disabled?
Consider Self Disabled?
Yes
No
Wellbeing Yes
Wellbeing No
All
246 (67%)
119
46.4
49.7
Mobility 0-1
22 (31%)
49
48.4
47.8
Mobility 2-3
55 (63%)
33
45.3
50.0
Mobility 4-5
76 (75%)
25
48.1
52.0
Mobility 6-7
34 (87%)
5
44.5
-
Mobility 8-9
59 (89%)
7
45.6
-
British Isles
66 (86%)
11
44.6
(52.5)
North America
59 (74%)
21
47.3
49.8
South America
11 (17%)
53
(40.5)
50.3
France
32 (86%)
5
46.2
-
Europe
39 (80%)
10
50.2
(44.2)
Australia
19 (76%)
6
(45.5)
-

Two thirds of respondents consider themselves to be disabled and one third 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 used there is a more clear distinction between wellbeing, with those identifying as disabled and using mobility aids having lower wellbeing than those who do not identify as disabled but who do use mobility aids.

Identifying as disabled may have cultural differences across the world, and this has been examined in terms of wellbeing. Of note, the proportion of people identifying as disabled in South America is considerably lower than elsewhere in the world. In Europe (excluding UK, Ireland and France) those that identify as disabled have a higher wellbeing than those who do not identify as disabled, although this is a small sample size and the result should be treated with caution. 

Do you Consider Yourself to be Lonely?
This question aimed to identify one often reported aspect of having a rare disease, loneliness. 361 respondents answered this question, as shown in the following grid. Wellbeing scores are also calculated, and shown in brackets where there is a small sample size, and not shown for very small samples.

Table 32 – Identify as Lonely?
Consider Self Lonely?
Yes
No
Wellbeing Yes
Wellbeing No
All
116 (32%)
245
39.5
51.3
Mobility 0-1
16 (23%)
55
(38.2)
51.2
Mobility 2-3
29 (34%)
57
37.7
52.3
Mobility 4-5
24 (24%)
77
40.7
51.7
Mobility 6-7
16 (42%)
22
(39.6)
50.3
Mobility 8-9
16 (50%)
16
(41.2)
(49.7)
British Isles
21 (28%)
55
35.8
49.5
North America
32 (40%)
48
40.7
52.8
South America
23 (36%)
41
40.4
53.2
France
9 (25%)
27
-
48.6
Europe
12 (25%)
36
(41.1)
51.5
Australia
10 (40%)
15
(35.8)
(51.9)

This shows that one third of people with HSP consider themselves to be lonely. Loneliness tends to occur more often in those whose mobility is more restricted.

There is a significant difference in wellbeing between those who are lonely and those who are not. The averages for each are more than 5 points from the average wellbeing suggesting a significant effect.

The pattern is consistent across different areas of the world.

Do you Consider Yourself to be Isolated?
Although there is an overlap between the meanings of lonely and isolated, this question was asked to identify if the perceptions of having HSP change depending on how you describe them. 362 respondents answered this question, as shown in the following grid. Wellbeing scores are also calculated and shown in brackets where there is a small sample size and not shown for very small samples.

Table 33 – Identify as Isolated?
Consider Self Isolated?
Yes
No
Wellbeing Yes
Wellbeing No
All
107 (30%)
255
39.0
50.9
Mobility 0-1
12 (17%)
59
(33.4)
50.6
Mobility 2-3
25 (29%)
61
36.0
51.5
Mobility 4-5
24 (24%)
77
41.9
51.3
Mobility 6-7
16 (42%)
22
(39.6)
50.3
Mobility 8-9
14 (44%)
18
(41.2)
(49.9)
British Isles
21 (28%)
55
36.6
49.2
North America
34 (43%)
46
40.8
53.3
South America
17 (27%)
47
(37.8)
52.5
France
6 (17%)
30
-
48.9
Europe
8 (16%)
41
-
50.5
Australia
8 (32%)
17
-
(49.2)

This shows that one third of people with HSP consider themselves to be isolated. Isolation tends to occur more often in those whose mobility is more restricted. Although the pattern is similar to the loneliness question there is a stronger pattern than was shown with the loneliness question. Results are also consistent across different areas of the world. This suggests that there is little difference overall in terms of describing HSP has having potential to be lonely or isolating, although isolation shows a stronger pattern.

Similarly to loneliness, there is a significant difference in wellbeing between those who are isolated and those who are not. The averages for each are more than 5 points from the average wellbeing suggesting a significant effect.

There are many views and opinions around this topic. Some respondents come from a more stable background with supportive family and friends, and getting out and about socially. Others feel more lonely/isolated. These feelings can arise because people are unable or do not want to explain their situation/problems to their close friends/family, or because they don’t like the changes that HSP puts on them, including both physical changes and employment changes. Some feel invisible whereas others think they are very visible.

Do you Consider Yourself to be Vulnerable?
This question aimed to identify if people feel vulnerable with HSP or not. The background to this question arose from discussions around safeguarding, where the approach for safeguarding may need to be different for vulnerable people and those who are not vulnerable. It was not known if people considered themselves to be vulnerable or not. 361 respondents answered this question, as shown in the following grid. Wellbeing scores are also calculated and shown in brackets where there is a small sample size.

Table 34 – Identify as Vulnerable?
Consider Self Vulnerable?
Yes
No
Wellbeing Yes
Wellbeing No
All
195 (54%)
166
44.5
50.6
Mobility 0-1
38 (54%)
33
44.5
51.8
Mobility 2-3
40 (48%)
44
43.5
49.4
Mobility 4-5
56 (55%)
45
47.4
51.1
Mobility 6-7
22 (56%)
17
41.6
(50.4)
Mobility 8-9
22 (69%)
10
42.6
(50.6)
British Isles
35 (46%)
41
42.1
48.8
North America
41 (51%)
39
45.1
50.9
South America
37 (59%)
26
44.5
53.7
France
22 (59%)
15
43.5
(51.5)
Europe
28 (58%)
20
47.7
50.2
Australia
12 (48%)
13
(41.1)
(49.2)

This shows that around half of people with HSP consider themselves to be vulnerable. Vulnerability tends to be similar across mobility bands and in different parts of the world.

There is a difference in wellbeing between those who are vulnerable and those who are not. Some groups are more than 3 points from the average indicative of an effect, but not necessarily significant.

People identified that they feel vulnerable for two main groups of reasons, one group is around the physical changes of HSP not allowing people to be able to defend themselves or resist attack, the other group is around the consequences of HSP symptoms, including balance problems, incontinence and speed/ease of movement in crowded situations.

Have you suffered from abuse?
This question aimed to identify if people with HSP have suffered from abuse. The background to this question similarly arose from discussions around safeguarding. It is important to note that the question is deliberately not specific about types of abuse or timeframes when the abuse occurred. 359 respondents answered this question, as shown in the following grid. Wellbeing scores are also calculated and shown in brackets where there is a small sample size, and not shown for very small samples.

Table 35 – Suffered from abuse?
Suffered from Abuse?
Yes
No
Wellbeing Yes
Wellbeing No
All
52 (14%)
307
43.0
48.2
Mobility 0-1
3 (4%)
66
-
48.0
Mobility 2-3
13 (15%)
72
(39.2)
48.6
Mobility 4-5
12 (12%)
88
(45.9)
49.4
Mobility 6-7
11 (28%)
28
(41.5)
47.0
Mobility 8-9
7 (23%)
24
-
46.6
British Isles
10 (13%)
66
(37.8)
46.8
North America
15 (19%)
65
(43.6)
49.0
South America
11 (18%)
51
(48.8)
48.6
France
2 (5%)
35
-
46.9
Europe
1 (2%)
46
-
49.1
Australia
7 (28%)
18
-
(48.3)

This shows that around one in seven people with HSP have suffered from abuse. There is a general trend which suggests that those with restricted mobility have suffered abuse more than those who are more mobile. There appears to be a higher proportion of people who have suffered from abuse in Australia, America and the British Isles, and a lower proportion in mainland European countries.

Those who have suffered abuse tend to have a lower wellbeing than those who have not. It is interesting to note that this trend does not appear to hold in South America.

Approximately three quarters of those who suffered from abuse indicated that this was as a result of their HSP, and these come from across the range of mobility scores.

Wellbeing Conclusions

Throughout this report 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 36 – Positive Wellbeing Factors
Factor
Respondents
Wellbeing
Travel further than town daily (life-space)
35
51.7
Travel to town daily (life-space)
69
51.6
Not feeling lonely (life with HSP)
245
51.3
Daily use of cardiovascular gym machines (activities)
21
51.0
Not feeling isolated (life with HSP)
255
50.9
Weekly music (activities)
28
50.8
Occasional music (activities)
34
50.7
Not feeling vulnerable (life with HSP)
166
50.6
No medication (medication)
46
50.6

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

Table 37 – Negative Wellbeing Factors
Factor
Respondents
Wellbeing
Taking 6 medicines for HSP (medication)
5
(37.6)
Feeling isolated (life with HSP)
107
39.0
Feeling lonely (life with HSP)
116
39.5
Having SPG7 (diagnosis)
35
41.7
Diagnosis uncertain (diagnosis)
7
(42.1)
Suffered from abuse (life with HSP)
52
43.0
Taking 4 medicines for HSP (medication)
17
(44.2)

Several of these symptoms are related to mood; feeling lonely, feeling isolated, suffering from abuse and uncertainty of diagnosis. Those who do not feel lonely or isolated overall have a significantly higher wellbeing than those who do.

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 lonely or isolated. Help is available for some factors, and getting such help may result in an improvement in wellbeing.

People should consult with a doctor or other professional to advise on the best approach for any such change they wish to make. Potential areas where changes could be made include:
  • Decreasing: loneliness and/or isolation
  • Taking part in activities which are enjoyable
  • Being able to travel
  • Getting help/support/counselling for any abuse
  • Treatment/advice/exercises to slow the progression of HSP