Symptoms Update - fall, bike, bed, tired!

This is a quick update on symptoms. Whilst the post title may suggest a single story, these are in fact separate things.

Last year I took part in the Plymouth HSP falls study. Over this time (and in fact before this time) I didnt have any falls. There have been a few moments when it has been close, but I have been able to prevent a fall. The other week I had my first fall, which marks the start of a new symptom to be tracked. I was coming down the stairs at home, wearing socks. About half way down one of my feet either missed or slipped off the step which caused me to fall down. I landed on my bottom, and aside from one toe bumping into the banister/balusters during the landing there was no damage done. I've noted before about using the banister to get up and down stairs, which I do pretty much all of the time (except when carrying). I know that I use the banister less going downstairs, but I think this fall was from going too fast rather than anything else.

Readers may note that some while ago I switched from using normal pedals on my bike to using cleats. As noted this has prevented my feet from sliding all over the pedals, which is a good thing. Recently I've been trying out different muscle groups whilst cycling, and when my muscles are feeling a bit tight from my normal cycling I switch for a short while to putting the power on when i'm pulling my feet up rather than pushing them down. This helps rest those other muscles, and a few minutes later I'll be back to normal.

When in bed these days I'm finding it much more comfortable to sleep on my side with my legs bent a little at the hips and knees. I have mostly slept either like this or laying flat on my back. When laying on my back I'm feeling the tension in my leg muscles, which is not conducive to going to sleep.

Overall, I'm spotting that I'm needing to sit down quicker. I had observed during the Christmas season the need to sit down later in the evening, but I am now beginning to spot that symptom earlier in the say as well. Once I've sat for a short while I'm then able to get back up and carry on. This is most likely the spasticity of my muscles, but as this happens more when I am tired, it may also be a bit of fatigue creeping in.

Taking all this into account, I kind of realise that I'm unlikely to be able to manage spending the day walking 20-25km in the Lake District any more, which is a bit of a shame. I am though, still going out for various longer bike rides at the weekends, which is a good way to test how tired i'm getting. I notice that it is my legs which get tired well before the rest of my body gets tired, and fitbit tells me that my heat rate very rarely gets into the peak zone. I've recently worked out how to get heart rate from fitbit into strava, so there's a new dataset to look at as well.

A little bit of family history

I was chatting with my mum over the Christmas holidays and we had a look at the family tree to see if there was any other evidence of HSP.

We know that the HSP comes from my mums mum - she was diagnosed. My mums mum was the youngest of 10 children in her family, being some 20 years younger than her eldest sibling. Also, my mum was the youngest of three children, and she didn't get to meet either of her mums parents.

When my mum remembers her aunts and uncles on that side of the family she is sure that there were two uncles with HSP, all living in the same village as her and her mum. It is interesting to note that these uncles were builders, and they were able to work for the family builders, with some allowances made for difficulties getting up and down ladders. Of the children of these uncles, HSP doesn't seem to have come down to the next generation. There are also four aunts and uncles who definitely didn't have HSP, and three that my mum is not sure about.

So, since there are several siblings with HSP, it must have arrived with one of my great-grandparents from that side of the family, but at the moment we don't know who. If there are people in the UK with HSP who trace their family trees back to the late 1800's then get in touch - we may be able to work this out, and could be related!

My two great-grandparents are both from Cambridgeshire:
George Goodchild - 1 mar 1871 - 15 jun 1946
Anne Harding - 17 jun 1872 - 28 jul 1940

Living with the enemy

At the HSP Support Group AGM in 2017 Robin Pajmans recommended the book "Living with the Enemy", by Ray Owen. I got this from my library to read. I found that there were a load of useful things in this. This post is a review of the book with a few points that I found useful from each chapter.

Overall the book covers several approaches for coping with the stress of a chronic condition or long term condition. It uses Cognitive Behavioural Therapy (CBT), Mindfulness and acceptance, and Acceptance Commitment Therapy (ACT).

The first chapter "knowing the enemy" is about understanding what people are dealing with in the case of a chronic condition. Whatever coping strategy is adopted, it cannot depend on finding a cure or a solution which sends all the symptoms away. With a chronic or long term condition these are not possible. It is better to avoid being in a fight with your condition.

The second chapter "getting stuck in the struggle" describes that having a long term condition (LTC) can have a huge impact on people, physically, practically and psychologically. When in a stressful situation our natural response is either to fight or flight - both of these can be difficult with an LTC.  If you are the type of person who urges to control, but your LTC is uncontrollable, it can set into unhelpful thinking patterns. The urge not to feel bad is strong, and many take the choice to miss out on things to do this - but the consequence of this is a more narrow life, missing out on things you enjoy. If you supress unwanted thoughts they will often reoccur. This chapter introduced me to the "hot cross bun model" which differentiates out what is a thought, a feeling, a behaviour and a physical symptom - and identifies which of these are in your control, and which are not.

Chapter 3, "troubling thoughts" identifies that having troubling thoughts can cause additional problems on top of an LTC. Some of those troubling thoughts are likely to be incorrect or at least distorted. Those who get caught up in such thoughts often stop noticing what is real. The chapter introduces setting up 'rules of thumb' to assess situations by and having a period of "worry time" each day to help resolve troubling thoughts.

Chapter 4, "unwanted feelings," moves on to starting to accept the presence of unwanted thoughts. If you can accept some of these thoughts being in your mind, even if you dont like them, then you can avoid making them the centre of attention and free up your mind to do something more useful!

Chapter 5, "living in the present", notes that the mind tends to wander during the day, sometimes looking at the past, at the future or other places. Whilst the mind wanders of its own accord, if you get lost in unwanted thoughts this isn't a happy place to be and by not paying attention to the present you miss out on what is happening in the here and now. The chapter introduces present moment awareness and mindfulness as techniques to help you focus on the present moment.

Chapter 6, "who am I now? a sense of self" separates out you as a person from the LTC that you have. It is the condition that is the burden, not you. The chapter sets out the concept of accepting the present and mourning the past. If you get stuck in a set story about yourself, the changing nature of an LTC means that this has potential to end up focusing on your limitations rather than your abilities.

Chapter 7, "living with purpose" sets out a method for working out your personal goals and purpose, but in the framework of LTCs being able to get in the way of some of your important goals. Knowing your values can help set your direction and give your life a better sense of purpose/fulfilment. It is important to note that goals are not the same as values. Once you know your values you can make sure that you not continually neglecting any of these.

Chapter 8, "taking action", has the central premise that you need action in order to make a change. Base your actions on your values, choose goals that will matter to you personally, and make sure that they are smart (specific, measurable, achievable, relevant, time-bound). Note that you cannot change the behaviour of other people (but you can influence it).

Chapter 9 "putting it together" gives example action statements to make as a commitment: "I will do [action] in service of [value] and am willing to experience [unwanted thought/feeling] if that is what it takes".

If you wanted to buy a copy of this, it is available from Amazon, and of course other bookshops and libraries are available! https://www.amazon.co.uk/Living-Enemy-chronic-mindfulness-acceptance/dp/0415521203

Regular readers of this blog will note that I've covered similar things like this before:
CBT: https://hspjourney.blogspot.co.uk/2014/04/stress-and-mood-management.html
Values: https://hspjourney.blogspot.co.uk/2016/01/depression-and-assessing-yourself.html
It also reminded me of Stephen Covey's Seven Habits of Highly Effective People, in which there si much more on values, prioritising tasks and focusing on what is important.

2017 Survey Results

Rare disease day is here again, and I'm very pleased to be able to publish the results of my fifth survey. The theme for rare disease day this year is research, so that is quite appropriate for this post. This also happens to be my 200th blog post!

This survey was launched in the autumn of 2017, and once again I have had a fantastic response from people around the world completing the questions and contributing to my analysis of HSP. In this post is a short version of the results. The full version can be found by clicking the link 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/open?id=1d5H11BsMHSQplf0foiJrE6hQrXedUC6w

There were 222 respondents who completed the survey, predominantly from the USA and the UK, but also from Europe, South Africa and India. About a third of respondents had completed at least one of my previous surveys. Seven people have completed all five surveys. Of those who know their type of HSP, SPG4 and SPG 7 were most common.

Mobility Analysis

I have developed an “HSP mobility score” which then allows me to cross-reference mobility against the other questions in the questionnaire. The definition of the HSP mobility score is;

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

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

Overview of mobility aids used

Mobility Aids Used - Overview:	Respondents	Percentage	Mobility Score
Those without aids	37	17%	0-1
Those who use mobility aids some of the time	46	21%	2-3
Those who use sticks most/all of the time	75	34%	4-5
Those who use frames most/all of the time	28	13%	6-7
Those who use chairs most/all of the time	36	16%	8-9

This shows that there is a significant variation in the mobility of the respondents to the questionnaire, with respondents covering all mobility scores. Overall the proportion of respondents falling into these groupings is similar to previous years with broadly an even split between these bands.

Pain

My 2013 survey showed that just under 80% of people with HSP suffered from back pain, being significant in over a third of people. My 2014 survey showed that around a third of all medication being taken for HSP and HSP symptoms is for pain.

I wanted to explore pain with HSP and from HSP symptoms to be able to quantify peoples’ pain some more. Aside from two papers from 2016, described below, there seems to be little published research quantifying what appears to be a relatively common symptom. I wanted to understand:

• How many people get pain from HSP
• What methods do people use to control/manage their pain
• How people describe the pain
• Where the pain affects them
• How intense the pain is

To do this I asked a range of questions. For methods of pain management I found a general list of methods which people use, which I listed out. I allowed an “other” option, which added some options used by a few people. To evaluate how people describe their pain I wanted to use a standard pain measurement scale, and selected the Short-Form McGill Pain Questionnaire (SF-MPQ-2) which has been used in a number of diverse studies examining chronic pain and acute pain. The questionnaire assesses major symptoms of both neuropathic and nonneuropathic pain. Anecdotally I heard that different people with HSP get their pain in different locations, and so I expanded this part of the questionnaire in order to examine pain location. Finally, I added questions to allow a brief look at change in pain levels with medication.

I found 2 papers which cover pain in HSP. A Norwegian paper “Health survey of adults with hereditary spastic paraparesis compared to population study controls”, published in Orphanet Journal of Rare Diseases in 2016, written by Krister W. Fjermestad, Øivind J. Kanavin, Eva E. Næss, Lise B. Hoxmark and Grete Hummelvoll reported a survey of 108 adults with HSP against controls. Those with HSP had more frequent musculoskeletal pain and more frequent pain in lower body pain sites, whereas controls had more frequent pain in upper body pain sites. Two thirds of those with HSP had chronic pain of more than 3 months duration in the last year, with most frequent pain sites being feet, knees, lower back and hips.

A Brazilian paper “Non-motor symptoms in patients with hereditary spastic paraplegia caused by SPG4 mutations”, published in European Journal of Neurology in 2016, written by K. R. Servelhere, I. Faber1, J. A. M. Saute, M. Moscovich, A. D'Abreu, L. B. Jardim, H. A. G. Teive, I. Lopes-Cendes and M. C. Franca Jr, reported a study of 30 people with HSP and 30 controls who used the Brief Pain Inventory. As reported in the abstract the average score for those with HSP was 3.4 compared with 1.0 for the controls. The scoring for the Brief Pain Inventory ranges between 0 and 10.

Pain from HSP

211 respondents answered the pain questions. Of these 170 (81%) said that they get pain from HSP whereas 41 (19%) do not.

Pain From HSP?

Mobility Score	Respondents	Yes	No	Percentage Yes
0-1	36	27	9	75%
2-3	45	37	8	82%
4-5	68	61	7	90%
6-7	28	19	9	68%
8-9	34	26	8	76%
Overall	211	170	41	81%

The proportion of people who get pain from HSP is similar across the mobility bands. Those who use walking sticks all or most of the time get pain most often from HSP, and those who use frames all or most of the time get the pain least often from HSP.

Methods of pain control

The 170 people who get pain from HSP indicated the approaches they use to control pain, with all 466 choices shown. 13 respondents (8%) do not use any pain control. 157 people (92%) use at least one method of pain control, as shown in the following table, with the percentage showing the proportion of all 170 respondents who use this method of pain control;

Methods of pain control

Pain Control Method	Respondents	Percentage
Prescription medicines	103	61%
Physical therapy (e.g. exercise)	96	56%
Manipulation and massage	65	38%
Over-the-counter medicines	59	35%
Heat and cold therapy	49	29%
Relaxation techniques	29	17%
TENS machine	19	11%
Meditation	15	9%
Visual imagery	6	4%
Supplements	6	4%
Acupuncture	5	3%
Biofeedback	3	2%
Cognitive Behavioural Therapy (CBT)	3	2%
Prayer	3	2%
Other	5	3%
Answers	466

This shows that most people use medication, either prescription or over the counter, or physical therapy, either exercise, manipulation or massage to relieve pain. Heat and cold therapy, relaxation techniques and TENS machines are other methods used by at least 10% of respondents. The average number of methods used to treat pain is 3, showing that most people use more than one method to control their pain.

There are no common trends between the choice of medication and use of mobility aids, with all of the most common methods of pain control being used across the full range of mobility scores.

Describing HSP pain

Respondents were asked to complete the Short-form McGill Pain Questionnaire (SF-MPQ-2). The questionnaire comprises a list of 22 different descriptions of pain, for example “stabbing pain”, and ask people to score their intensity of each item on a numeric scale from 0, “none” to 10, “worst possible”. The full list of 22 items is described later in this section.

The total score is the summation of the 22 items, which can range between 0 and 220. I have also divided the total score by 22 to bring it back to a scale from 0 to 10. The overall average score is 76.4, which converts back to 3.5 on the 0 to 10 scale. Although the scale is different, the average value (3.5) is similar to the 3.4 reported by the Brazilian paper using BFI scoring, and although the sample size is small, those who do not have pain from HSP score similarly (0.9) to the controls in the Brazilian study (1.0).

Overall SF-MPQ-2 Score

Mobility Score	Respondents	Average Score	Average (0-10)
0-1	23	69.2	3.1
2-3	28	77.2	3.5
4-5	42	80.6	3.7
6-7	17	84.8	3.9
8-9	13	63.4	2.9
Pain = Yes	116	79.9	3.6
Pain = No	7	19.0	0.9
Overall	123	76.4	3.5

This table shows that the pain score is highest for those using frames all or most of the time (6-7) and those using sticks all or most of the time (4-5). Pain is lowest for those using wheelchairs all or most of the time (8-9) and those who do not yet use mobility aids (0-1). The following figure shows the full spread of results.

There are large differences between the 22 items, as shown in the table below, which indicates how many of the 116 people indicated more pain than none (a score of zero), and the average score (out of 10) for that factor for those people:

Description of pain

Pain description	Respondents	Percentage	Average Score
Tiring-exhausting	108	93%	6.9
Aching pain	108	93%	5.7
Cramping pain	105	91%	5.8
Tingling or ‘pins and needles'	102	88%	5.0
Numbness	99	85%	4.8
Shooting pain	84	72%	3.9
Heavy pain	83	72%	4.3
Tender	83	72%	3.5
Throbbing pain	80	69%	3.7
Sharp pain	78	67%	3.8
Stabbing pain	75	65%	3.5
Gnawing pain	72	62%	3.7
Hot-burning pain	67	58%	3.0
Fearful	66	57%	3.2
Electric-shock pain	61	53%	2.8
Punishing-cruel	60	52%	3.1
Cold-freezing pain	59	51%	2.6
Sickening	59	51%	2.4
Itching	58	50%	2.4
Piercing	52	45%	2.4
Pain caused by light touch	54	47%	2.1
Splitting pain	52	45%	2.0

             

The description of the pain noted by the most respondents is “tiring-exhausting”, scoring an average of 6.9 out of 10 for 108 respondents (93%). “aching pain” is also noted by 108 respondents, with an average score of 5.7.

Pain Intensity

The last part of the SF-MPQ-2 is to rate the overall pain intensity. Of the 116 people who have scored their pain descriptions 114 provided an overall pain intensity, selecting the closest match for pain from HSP and HSP symptoms, from the options in the following table;

Table 13 – Overall Pain Intensity

Intensity	Respondents	Average Score	Average (0-10)
No Pain	4	(67.3)	(3.1)
Mild	15	27.9	1.3
Discomforting	41	61.0	2.8
Distressing	34	91.6	4.2
Horrible	16	130.2	5.9
Excruciating	4	168.5	7.7
Overall	110	79.9	3.6

The most common descriptors of pain intensity are “discomforting” and “distressing”. This table shows that the average SF-MPQ-2 score increases with the descriptors of overall pain intensity except for four respondents who scored their pain descriptions and then selected “no pain” in the overall intensity. This could suggest that their pain is not due to HSP or HSP symptoms. Their average scores are shown in brackets, and this data is excluded from the overall average.

Pain Location

To seek further information on the location of pain I used the overall pain intensity descriptions to ask about the intensity of pain is specific parts of the body. The most common locations for pain are legs, feet, back and hips, as shown in the following table.

Overall Pain Intensity

Pain Location	Respondents	No Pain	Mild	Discomforting	Distressing	Horrible	Excruciating
Legs	116	3%	11%	26%	30%	24%	6%
Feet	115	6%	22%	27%	23%	16%	7%
Back	116	12%	18%	34%	16%	15%	6%
Hips	116	16%	16%	27%	18%	16%	6%
Neck	116	40%	22%	22%	10%	4%	2%
Hands	115	44%	26%	20%	4%	4%	1%
Arms	116	52%	19%	17%	7%	5%	0%
Head	115	62%	20%	8%	8%	3%	0%
Stomach	116	63%	14%	14%	5%	3%	2%
Chest	116	83%	6%	8%	2%	2%	0%

This shows that over 95% of people who have pain with HSP have some pain in their legs, and over 90% have pain in their feet. For legs and feet the common descriptors for pain intensity are discomforting and distressing, accounting for more than half of respondents.

Over 80% of people who have pain with HSP have some pain in their back and hips. The common description for pain intensity is are discomforting. These four areas of the body are similar to those reported in the Norwegian study.

Pain is found least in the head, stomach and chest, with over 60% without pain in the head and chest, and over 80% without pain in the chest.

Walking

Perhaps the most common symptom of HSP is impairment of walking. There are plenty of studies which evaluate walking speed and distance, evaluate gait patterns and muscle problems. The majority of the papers I have seen evaluate walking in a clinical environment. There is plenty of anecdotal evidence about how some environments are easier to walk in than others, and other anecdotal evidence about how walking requires concentration such that normal conversation is not possible. I wanted to explore perceptions around real walking environments and real distractions which people encounter, to examine if there are any trends.

A comparison of walking against mobility bands doesn’t take fatigue into account, which my 2016 survey showed affects 90%. To take this into account I also used “How far can you walk?” from the Spastic Paraplegia Rating Scale (SPRS). I listed factors which could affect walking and asked respondents to “Describe if any of the following factors affect the quality of your walking when considered on their own.” Respondents selected from pick-lists how much and how often these affected them.

Maximum Distance

The table below shows the distribution of how far people can walk, distributed by their use of mobility aids. 186 people responded to this question.

Table 16 – Maximum Walking Distance

Mobility Score	As far as I want to	Exhausted beyond 500m	Up to 500m	Up to 10m	Unable to walk
0	2	0	0	0	0
1	15	7	4	1	0
2	4	10	4	1	0
3	2	10	4	2	1
4	1	14	6	1	0
5	4	13	7	16	2
6	1	2	1	3	1
7	0	5	3	6	2
8	0	2	2	7	7
9	0	0	0	2	11
Total	29	63	31	39	24
Percentage	16%	34%	17%	21%	13%

The table shows some things as expected – for example that people who can walk as far as they want to do not use wheelchairs all or most of the time, and people who are unable to walk are generally using wheelchairs all or most of the time. Generally, the more frequent the use of mobility aids the more walking distance is limited. There are a few surprising answers, for example one person is unable to walk but only uses mobility aids for some of the time.

One third of respondents indicate that they get exhausted after a distance of more than 500m. The other answers score relatively evenly, with about one sixth of respondents in each category.

Walking Factor Assessment

For each factor respondents scored how much and how often it affects them. Two analyses are presented. 

For the first analysis, the table below shows the proportion affected by each factor split by maximum walking distance. The analysis only uses the walking impact factor, and includes those who scored the factor other than “not at all”. The “answers” column indicates the total number of people who scored the factor. 

Factors Affecting Walking

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

The factors which affect people the most are those with the highest percentages in each column and the highest number of total respondents. Those factors which affect people the lease are those with the fewest answers and with the lowest percentages.

For those who can walk as far as they want, the factors which affect walking the most are:

• Being in a rush
• Tiredness/fatigue
• Going over uneven ground
• Carrying something

These factors affect around 90% of people.

For those who get exhausted after more than 500m, the factors which affect walking the most are:

• Tiredness/fatigue
• Where stairs/steps are involved
• Going down slopes
• Going up slopes
• Going over uneven ground
• Carrying something

These factors affect almost 100% of people.

For those who get can walk up to 500m, the factors which affect walking the most are:

• Tiredness/fatigue
• Being in a rush
• Going down slopes
• Going over uneven ground
• Where steps/stairs are involved

These factors affect all people.

For those who get can walk up to 10m, the factors which affect walking the most are:

• Going down slopes
• Going up slopes
• Where steps/stairs are involved
• Needing to go to the toilet

These factors affect all people.

For the second analysis, to examine this in more detail I scored each factor according to how much and how often it impacts those affected. 

Impact Score for Factors Affecting Walking

Factor Group	Factor	As far as I want to	Exhausted beyond 500m	Up to 500m	Up to 10m
Surface	Down slopes	8.80	9.37	8.56	10.60
Surface	Up Slopes	5.67	8.26	6.99	12.74
Surface	Round tight bends/corners	5.04	7.27	7.70	7.60
Surface	Over smooth ground	6.42	6.54	5.57	8.37
Surface	Over uneven ground	7.62	9.98	9.91	13.18
Surface	Where steps/stairs are involved	8.22	11.53	9.76	12.43
Activity	Talking/chatting	4.68	7.80	8.35	8.02
Activity	Carrying something	5.99	9.98	9.59	14.85
Activity	Using the phone	4.58	9.08	9.86	10.20
Activity	Concentrating	6.23	7.90	8.35	8.81
Activity	Managing children!	4.33	9.38	7.48	9.18
Factor	Being in a rush	7.44	9.69	9.32	11.92
Factor	Need to go to the toilet	6.48	8.79	11.41	11.42
Factor	Stressed	6.20	7.85	5.60	8.58
Factor	Upset/emotional	6.29	6.52	5.04	7.91
Factor	Tired/fatigued	8.77	10.80	9.49	12.42
Factor	Extra short term illness	7.48	4.63	5.52	7.43
Factor	Have had alcohol	5.18	5.39	6.22	8.40
Factor	Have had caffeine	4.71	5.81	5.19	7.07
Environment	It is a hot day	6.44	5.91	6.19	7.11
Environment	It is a cold day	9.75	7.68	8.47	9.17
All	Average all data	6.5	8.1	7.8	10.0

This allows the relative importance of each factor within each group to be examined. As a persons maximum distance decreases each factor tends to score higher, demonstrating a greater impact for that factor.

For those who can walk as far as they want, the factors range between 4 and 10, with an average of 6.5. The highest scoring factors are:

• It being a cold day
• Going down slopes
• Tiredness/fatigue
• Where stairs/steps are involved
• Going over uneven ground

For those who get exhausted after more than 500m, the factors range between 5 and 12, with an average of 8.1. The highest scoring factors are:

• Where stairs/steps are involved
• Tiredness/fatigue
• Carrying something
• Going over uneven ground
• Being in a rush

For those who can walk up to 500m, the factors similarly range between 5 and 11, with an average of 7.8. The highest scoring factors are:

• Need to go to the toilet
• Going over uneven ground
• Using the phone
• Where stairs/steps are involved
• Carrying something

For those who can walk up to 10m, the factors range between 7 and 15, with an average of 10. The highest scoring factors are:

• Carrying something
• Going over uneven ground
• Going up slopes
• Where stairs/steps are involved
• Tiredness/fatigue

I have combined the results from all groups of respondents and sorted these by factor, as shown in the following table. The table shows how much and how often it affects people and the overall factor. Also shown is the number of respondents affected and the proportion of all those scoring, sorted by overall factor.

Impact Score for Factors Affecting Walking

Factor	Respondents	How Much?	How Often?	Overall Factor	Affecting
Where steps/stairs are involved	146	3.4	3.2	10.9	96%
Tired/fatigued	144	3.4	3.1	10.6	97%
Over uneven ground	143	3.3	3.1	10.2	95%
Carrying something	143	3.3	3.1	10.2	95%
Down slopes	146	3.1	3.0	9.4	94%
Being in a rush	139	3.5	2.8	9.7	95%
Need to go to the toilet	138	3.1	3.1	9.5	91%
Using the phone	90	3.0	2.9	8.6	60%
Up Slopes	141	3.0	2.9	8.5	92%
It is a cold day	132	3.2	2.6	8.5	89%
Managing children!	103	3.0	2.8	8.3	74%
Concentrating	102	2.7	3.0	8.1	69%
Talking/chatting	101	2.6	2.9	7.4	67%
Stressed	129	2.9	2.6	7.3	88%
Round tight bends/corners	125	2.5	2.9	7.2	82%
Over smooth ground	114	2.3	3.0	6.8	75%
Upset/emotional	125	2.7	2.4	6.5	86%
It is a hot day	100	2.7	2.4	6.4	70%
Have had alcohol	100	2.9	2.1	6.1	74%
Have had caffeine	54	2.4	2.5	5.9	40%
Extra short term illness	101	2.9	2.0	5.8	74%

Taking a broad view, the factors which affect people walking the most seem to be:

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

In practical day-to-day terms, the combination of more than one factor can turn a straightforward walk, at whatever ability, into something more challenging. The data shows that if people are affected by fatigue or tiredness, or they are in a rush, then walking is often more difficult. These factors are mentioned in combination with the widest number of factors indicating that they are key factors. 

Occupation and Disability

Anecdotally, there are many stories about people having problems at work due to their HSP. I wanted to explore this issue some more to see if I could spot any trends. I set out a series of questions to obtain information about peoples occupation and employment.

Disability

219 people answered the question “Do you consider yourself to be disabled?”. 184 (84%) said they did and 35 (15%) said they did not. This table shows the variation with mobility score.

Table 26 – Do you consider yourself to be disabled?

Mobility Score	Disabled	Not disabled	Total	Proportion disabled.
0	0	3	3	0%
1	14	19	33	42%
2	16	5	21	76%
3	22	2	24	92%
4	21	3	24	88%
5	49	1	50	98%
6	10	0	10	100%
7	17	1	18	94%
8	21	1	22	95%
9	14	0	14	100%
Overall	184	35	219	84%

With the highest and lowest mobility scores there are no surprises. Those whose mobility is not affected do not consider themselves disabled, and those in wheelchairs all the time do consider themselves disabled. Between this, however, there are people in all mobility bands who consider themselves disabled, and there are people in most mobility bands who do not consider themselves disabled.

Current Occupation/Employment Situation

The following table shows the distribution of occupation/employment with age. 217 respondents answered this question, selecting the answer which best suited from pick-lists.

Current Occupation/Employment Situation

Situation	Age 0-24	Age 25-49	Age 50-74	Age 75+	Overall
Work: full time, paid	1	37	16	0	54 (25%)
Work: part time, paid	0	20	10	0	33 (14%)
Work: voluntary sector	1	7	7	1	16 (7%)
Student	10	0	1	0	11 (5%)
Carer	0	2	3	0	5 (2%)
I don’t work	2	23	35	0	60 (28%)
Retired	0	0	38	3	41 (19%)
Overall	14	89	110	4	217

This shows that:

• the majority (71%) of respondents aged up to 24 are students,
• the majority (64%) of those aged between 25 and 49 are working,
• the majority (66%) of those aged between 50 and 74 are retired (35%) or not working (32%),
• the majority (75%) of those of at least 75 are retired,

Overall, 53% of people are working or studying and 28% of people are not working, with the remainder retired. Of those who are not retired, there are approximately twice as many people working/studying as not.

195 of these people also indicated how content they are with their situation, as shown in the following table, with percentages showing the proportion that are content:

Content with Occupation/Employment Situation, by age

Situation	Age 0-24	Age 25-49	Age 50-74	Age 75+	Overall
Work: full time, paid	100%	86%	75%	-	83%
Work: part time, paid	-	75%	90%	-	80%
Work: voluntary sector	100%	43%	43%	0%	44%
Student	100%	-	100%	-	100%
Carer	-	50%	33%	-	40%
I don’t work	50%	23%	41%	-	34%
Retired	-	-	76%	100%	77%
Overall	89%	64%	63%	67%	65%

Trends by age:

The data shows those who are up to 24 are content, except when they are not working. Those who are 25-49 are most content when working and least content when not working. Those aged 50-74 are similar to those aged 25-49 although people are more content working part time rather than full time. Those who are retired are generally content.

Trends by situation:

Students appear to be content with their situation. Those in work, either full time or part time are generally content, closely followed by those who are retired. Those who don’t work, work in voluntary sector or are carers are least content with their situation.

Reasons for discontent:

Respondents were able to give reasons why they were not content with their current situation. 54 respondents provided an answer. From this there are several emerging themes:

• HSP issues affecting doing the job, including mobility, fatigue, strength and balance (10 people)
• Feeling unable to work at all (10 people)
• Being made redundant or retired early from the job (8 people)
• Unhappy in current work/job (6 people)
• Wanting to work less, but unable to do so (4 people)
• Being unable to find employment (4 people)
• Wanting to work more, but unable to do so (3 people)
• General frustrations about the current situation (9 people)

The themes affecting the greatest number of people are either when HSP affects the ability to do their job, or when they feel unable to work at all.

Change in Employment

202 respondents answered about changing jobs or stopping working as a result of HSP. About two thirds of people have had to. In detail, 127 (63%) had, and 75 (37%) hadn’t. 197 of these also answered questions about job type. This table shows proportion of people who’ve had to change jobs, by mobility aids.

Table 31 – Change in Employment, by use of mobility aids

Situation	0-1	2-3	4-5	6-7	8-9	Overall
Work: full time, paid	20%	25%	33%	67%	0%	28%
Work: part time, paid	38%	57%	55%	100%	67%	53%
Work: voluntary sector	100%	75%	80%	100%	67%	79%
Student	100%	-	-	-	33%	50%
Carer	0%	100%	100%	-	100%	80%
I don’t work	86%	100%	75%	100%	88%	88%
Retired	0%	100%	69%	86%	88%	76%
Overall	40%	62%	61%	91%	71%	62%

This shows that the people who have had to change jobs the most are those who use walking frames all or most of the time, with more than 9 in 10 having to change jobs, followed by those who use wheelchairs all or most of the time at 7 in 10.

Factors causing change

Respondents were asked the main factor which caused them to change jobs or stop working. 98 respondents completed an answer giving at least one factor. The main factors are:

• HSP offering physical limitations to undertaking work (67 respondents)
• Fatigue (23 respondents)
• Pain (17 respondents)
• Employer risk assessment/inability to alter workplace (12 respondents)
• Cognitive issues (7 respondents)
• Toilet issues (2 people)

I have put each response into as many categories as needed, noting that many respondents listed several factors, for example “Fatigue, effort navigating the building” and “Constant back pain, difficult in walking, lack of energy”.

This shows that the physical aspects of HSP are a key factor in around two thirds of respondents needing to stop work or change jobs. Fatigue similarly affects around a quarter of respondents, and pain affects around a fifth.

There were 12 respondents whose responses I have gathered under employer issues. These fell into two main groups, one where the respondent ceased to meet the requirements of their job. These were described as “being high risk”, “being discharged” or “failing a medical”. The other group was more about employers’ attitudes or failing to adapt the workplace, described as; “attitude of boss”, “they couldn’t adapt my work post”, and “they didn’t even want me driving into the parking lot”.

Consequences of Change

I asked respondents about the consequences of the change. 67 people responded in a way that I was able to judge the feeling behind the consequence described. There was a range of positive, negative and mixed consequences. Negative consequences account for about three quarters of responses, with the positive accounting for about one sixth.

By far the biggest negative consequence was financial, with about a third of respondents saying they had less money. Several (about 1 in 7) people said that they retired before they were ready to, and a similar number said they were more alone. Others commented that they were bored, frustrated or depressed and other that they were not making as much of a contribution or being a bigger burden. A few people said that they liked their current job less than their previous job.

Positive consequences included several describing themselves in a “better situation”, with others specifying less pain, less fatigue, fewer falls, and others saying they felt less of a burden on others.

A few respondents described having to start their lives over again, which I have taken to be a mix of positive and negative elements, whilst others balanced positive and negative factors in their answers, for example: “lost independence, now i do not work i have less stress, less worry, less spasms, less tension headaches, less fatigue and i can now attend physio appointments”, “I'm a lot more isolated now but also very relieved, as I was very stressed trying to work with the disability. Now I can rest and manage the pain and sensory input so my life is a lot better for that.” and “Money has changed, but I 'm glad I have more spare time”.

Support from Employer

I asked people if their employer had been supportive of their HSP situation. 146 people answered. Around 5% of respondents indicated that they had tried to hide their condition from their employer or not tell their employer about their condition. On the whole around two thirds of people said that their employer was positive about their HSP. Around a quarter said their employer was negative about their HSP, with the remainder having a mixed opinion.

Positive responses range from “My immediate boss has been considerate and has and continues to allow me to deal with doctors and physical therapists when needed” and “Very supportive. Encourages me to take breaks and not have to work the long shifts I push myself to do” through to “fairly” and “not too bad”. Negative responses range from “Terrible.  I felt discriminated” and “I was told to quit or get fired because I missed too much work.” through to “Not very, out of sight out of mind”. 

I looked at if there was any relationship between employer support and contentedness with current situation:

Content with work?

Employer attitude:	Content: yes	Content: no
Positive	77%	23%
Mixed	55%	45%
Negative	30%	70%

This table generally shows that if employers are positive to HSP then people are more likely to be content with their situation, and if employers are negative about HSP then people are less likely to be content with their situation.

Change in the Future

I asked respondents if they felt they would have to change jobs or stop working early in the future as a result of HSP. 144 respondents who answered this question had also answered about previous job changes.

Roughly two thirds of people think they will have to change jobs or stop working early as a result of HSP, and roughly one third do not. I was interested to find out if peoples previous job changes were a factor in this, as shown:

Change jobs in the future?

Future Change:	Previous change: yes	Previous Change: no	Total
Yes	64 (44%)	35 (24%)	99 (69%)
No	19 (13%)	26 (18%)	45 (31%)
Overall	83 (58%)	61 (42%)	144 (100%)

This table shows that 64 (44%) of people have changed jobs as a result of HSP and feel that they will have to either change jobs again or stop working early as a result of HSP. At the opposite end of the scale there are 26 (18%) of people who haven’t changed jobs as a result of HSP and don’t expect to have to change jobs or stop work early as a result of HSP.

Wellbeing

The results of my 2013 and 2015 surveys and other papers show that depression is a relatively common symptom for those with HSP. There are a range of personality types with differing outlooks on life. I wanted to investigate this aspect, but decided that there were too many factors to be able to isolate these in my questionnaire. Personality type questionnaires also contain a relatively high number of questions. Therefore, I elected to simply look at wellbeing.

I found 1 paper which covers wellbeing in HSP. A Norwegian paper “Health survey of adults with hereditary spastic paraparesis compared to population study controls”, published in Orphanet Journal of Rare Diseases in 2016, written by Krister W. Fjermestad, Øivind J. Kanavin, Eva E. Næss, Lise B. Hoxmark and Grete Hummelvoll reported a survey of 108 adults with HSP against controls. Those with HSP reported lower life satisfaction and lower mental wellbeing.

I selected the 14 item Warwick-Edinburgh Mental Well-being scale (WEMWBS) because this assesses wellbeing in 14 questions, and there is a big dataset describing various population norms from Scotland and England. The scale is “covering subjective wellbeing and psychological functioning, in which all items are worded positively and address aspects of positive mental health”. In addition, I have compared questions from my questionnaire against those population norms.

Overall Wellbeing

In order to assess the wellbeing of people with HSP I asked respondents to complete the Warwick-Edinburgh Mental Well-being scale. 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, although things like a poor health status and being out of work bringing the average down. Most people score 41 and 59. 

172 respondents answered this question, with the average wellbeing score of 45.9. The scores ranged between 19 and 68. The average is lower than UK population norms, which is in line with the results of the Norwegian study. However, the average is still within the “average” definition. A Scotland dataset includes an analysis of self-perceived health status. The average result from this survey is similar to those with a self-perceived health status of “poor”

For a more detailed analysis, this table shows the results, split by mobility band.

Table 35 – Wellbeing Results

Mobility Score	Respondents	Minimum Score	Average Score	Maximum Score
0-1	29	20	47.7	68
2-3	37	23	44.5	61
4-5	59	19	43.7	66
6-7	21	28	48.8	67
8-9	26	33	48.6	66
Overall	172	19	45.9	68

This table shows that the average wellbeing score is similar across all mobility scores, and scores are within 3 points of the average indicating use of mobility aids is not a significant factor in differentiating wellbeing. Wellbeing is slightly lower for those using mobility aids some of the time (mobility score 2-3) or for those using sticks most or all of the time (mobility score 4-5). The full set of responses is shown:

The group with the higher number with a very low score is those with a mobility score of 4-5 – i.e. those who use walking sticks all or most of the time. The group with the fewest people with an above average score is those with a mobility score of 2-3 – i.e. those who are beginning to use mobility aids for the first time or some of the time suggesting that the transition through the initial uses of mobility aids to using them most of the time affects wellbeing.

Wellbeing Factors

I thought it interesting to identify if any of the factors assessed in this survey gave markedly different wellbeing scores. The following factors have been examined:

Do you get pain from HSP?

Pain from HSP?	Average WEMWBS
Yes	44.7
No	51.7
Overall average	45.9

This shows that people who do not get pain from HSP have a better wellbeing than those who get pain from HSP. There is more than 5 points between the “yes” and “no” scores indicating a likely effect.

How far can you walk?

Employment	Average WEMWBS
As far as I want to	51.2
Exhausted beyond 500m	43.2
Up to 500m	43.9
Up to 10m	45.1
Unable to walk	50.7
Overall average	45.9

Those who can walk as far as they want have a better wellbeing, and those who are unable to walk are likely to have a better wellbeing. The other categories are within 3 points of the average and unlikely to be key differentiating factors.

Do you consider yourself disabled?

Consider disabled?	Average WEMWBS
Yes	45.2
No	48.6
Overall average	45.9

These results are similar, and within 3 points of the average score showing that considering yourself disabled or not is not a major factor in wellbeing.

Employment/Occupation Category

Employment	Average WEMWBS
I work full-time	47.4
I work part-time	44.5
I do not work	44.6
I am a student	47.5
I am retired	47.5
I am a carer	41.0
Overall average	45.9

These results are generally similar and generally within 3 points of the average. Those who are carers with HSP appear to have a lower wellbeing score than other categories.

Content with employment/occupation situation?

Consider disabled?	Average WEMWBS
Yes	48.7
No	40.4
Overall average	45.9

This shows that people who are content with the employment/occupation situation tend to have a better wellbeing than those who are not. There is more than 5 points between the “yes” and “no” scores indicating a likely effect.

Employer Supportive of HSP situation?

Employer Attitude?	Average WEMWBS
Positive	47.1
Mixed	46.8
Negative	41.1
Overall average	45.9

This shows that people whose employers are not supportive of HSP situations tend to have a lower wellbeing. There is more than 5 points between the “positive” and “negative” scores indicating a likely effect.

What Type of HSP?

Type of HSP?	Average WEMWBS
SPG4	47.5
SPG7	41.3
Overall average	45.9

This shows that people with SPG4 are similar to the average score. Those with SPG7 may have lower wellbeing than others with HSP.