Review of 2018

Once more it comes near to the end of the year, and it is time for me to reflect on the years activities.

Knowledge

My key learning has been around activities with the HSP Support Group - I've been looking at safeguarding, which is still a work in progress. There have been a few interesting articles around exercise and HSP (or strictly neuromuscular conditions), and there was a load of really interesting analyses from my survey results - particularly the pain and walking side of things, and I liked the wellbeing analysis as well.

Symptoms

I had the results of my MRI scan - all OK (blog post on this in early 2019!). I am definitely becoming more distance limited, and am finding the need to sit more often, and it looks like my shoe wear rate is still increasing. There hasnt been much noticeable change in other symptoms, although perhaps my bladder is being to re-annoy me (blog post on this in early 2019!). I may also be getting more tired, but there are so many other things going on at the moment that this is difficult to point at HSP.  

This Blog

I'm really pleased with the continuing growth in readership of this blog! My audience remains broadly the same (predominantly US, UK, Canada, Australia, and then European countries. The most popular posts continue to be the results of my survey and my general posts on research and particular HSP symptoms. 

I now have a second year of Google Analytics tracking, which helps inform my blog statistics page. I'm really pleased that blog posts are being linked to and used within other HSP and RareDisease publications. Thank you to all my readers.

I continue to get comments from people appreciating what I have to say, which I'm very pleased to receive. Feedback like this gives me another reason why this is a worthwhile thing to do. Thank you to anyone that makes a comment or connects up with me in some other way. A couple of my favourite comments about the blog from this years survey are:

"Newly diagnosed so found blog in general search trying to gain more info. Your blog seemed to mirror my symptoms over the past few years. I finally have an answer to my pain and problems."

"Appreciate all the effort you put in to the surveys and sharing your personal experiences"

Survey

The now annual pattern of my surveys is well established. My 2018 survey has a similar number of responses to my 2017 survey, perhaps with a few more participants (although at the time of writing I've not discounted duplicates and empty answers). My new feature this year has been to offer translation sheets for the survey in a number of other languages so that I can get a wider spread of answers, and a quick glance at the answers shows that some have answered in languages other than English, so this has worked well. If you're reading this and want to take part then (providing it is still 2018) please do! Results will be out on 28th Feb after the majority of analysis during January.

Similarly, its good to get feedback on this. Three of my favourite comments from this year are:
"Some of the questions make me think about what I could be doing better."
"I enjoyed filling out survey to help others"

"Excellent survey. It’s interesting to compare my symptoms to others. "

Community Contribution

HSP Community activities for 2018 included:

• Being re-elected chairman of the UK Support Group!
• Continuing talking with people from other HSP groups
• Expanding my survey into more languages
• Analysing and reporting the results of my survey to share with the HSP world
• Coordinating the UK HSP Support Group response to consultations
• Continuing this blog and posting/discussing HSP things on twitter.

The main element of my community contribution comes from being chair of the HSP group. There are many issues, and I'm working with the rest of the trustees to ensure that the group continues to support its members in the most appropriate way. In 2019 I'll be working with the PARCC research project. More on that during 2019.

HSP in the news

Last year I mentioned Lily Rice, and this year she continues to be an inspiration, promoting and competing in WCMX. She also became Radio 1 Teen Hero during the year.  https://www.westerntelegraph.co.uk/news/16994523.lily-rice-named-bbc-radio-1-teen-hero/  and https://www.westerntelegraph.co.uk/news/16152411.manorbier-teenager-claims-silver-at-californias-world-wheelchair-motocross-championships/ 

Over in Australia there seems to be quite a bit of news on their programme to develop a treatment for HSP. They are currently in the process of preparing for a clinical trial (https://hspersunite.org.au/hsp-clinical-trial-program-update-december-2018/) following the pre-clinical trials (https://hspersunite.org.au/hsp-clinical-trial-program-update-june-2018/). I'm keeping an eye out for the results of those pre-clinical trials. Readers will note that the drug being trialled is Noscapine and it will be trialled on patients with SPG4, 

More new shoes

Its time for some new shoes again!

I have got through another two pairs of shoes, the Sketchers which I bought in April 2017 have now run their course, with a new pair bought in December 2018. This means that my sketchers lasted 1 year and 7 months, indicating that my rate of wear has gone up in the last year, with the previous pair lasting 2 years and 2 months.

Although my left shoe has a higher rate of wear than my left shoe, the interesting observation is that the wear on both shoes looks similar, perhaps indicating that the progression on my right leg is catching up with the left leg. Here are a couple of pictures - noting that my left shoe is on the right of both pictures:

You can compare these with the post I put in my May 2017 with my previous shoes: https://hspjourney.blogspot.com/2017/05/shoe-wear-update-data.html

This time I have chosen to replace them with some hi-tec waking shoes, so we'll see how the rate of wear on those goes.

In the other post I noted having bought replacement trainers as well as replacement shoes. Shortly after this I also bought clip-in shoes for my bike, so the new trainers from that time have had much less use, and the clip-in shoes are also bearing up well.

My other replacement pair of shoes this time is some shoes for work. I had decided that these were not looking good enough for wear in the office, so I have a new pair of work shoes. There has been shoe shuffling, so the pair now in my office are my other older pair, with the new shoes staying at home - this is principally because I cant get the inner sole out of the new shoes and I'll wear those without insoles. My other pair of shoes takes the insoles nicely, and those have moved to the office with the insoles.

You can see the wear pattern of the old shoes in these pictures. The left shoe is slightly more worn on the tip than the right shoe, but the wear on the right shoe is more round the corner. Whilst at work I go on less fun walking, so the majority of the wear is from indoor use - although I do try to get outside for half an hour at lunchtime most days. I do not recall how long I have had these shoes. Note again that my left shoe is on the right of both pictures.

HSP in the news

There have been several HSP stories in the news recently which I thought worth sharing.

The benefits of stretching

This story was on the Australian HSP site this week: https://hspersunite.org.au/daily-duration-stretching-pays-off/

In summary, nine patients with HSP took part in a study where they were given a stretching routine to follow. The stretches were high load long duration stretches for specific muscles. Each person was reviewed and given their own routine. Stretches were undertaken six times a day for 15 minutes a time - so 90 minutes a day stretching, completed for more than one year. The muscles were selected based on how short they had become.

The outcome was assessed in terms fof range of movement for the muscle and ambulatiopn speed. 40% had higher walking speeds, half had better range of movements. two thirds were in a higher ambulation category.

This shows that regular stretches really do help HSP. The study also included patients with hemiparesis, and the abstract doesnt describe if the proportion of people with improvements was the same across both conditions.

Dancing with HSP

This website was posted on the Spatax website: https://www.clickanddance.com/videos - it gives a sequence of videos which you can follow to improve. Two sets of videos are given, one for those who can stand un-aided, and antoher set for those who need the help of a chair. Dancing is a physical activity, so good for health, and the use of different muscles can help. You may even enjoy it!

Original post: https://spatax.wordpress.com/2018/11/14/danse-to-improve-your-well-being-even-with-ataxia-or-spasticity/

Incontinence Pants

This story caught my eye on the BBC news feed. (https://www.bbc.co.uk/news/business-46220556) Its not directly related to HSP, but I thought worth a mention. This company makes incontinence pants for ladies that look like pants! https://www.giggleknickers.co.uk/. Their story comes from mild incontinence following surgery, but the same factor is true of HSP, and these pants are machine washable.

Vibration sensitvity / numbness

I had seen loss of vibration sensitivity in the legs as one of the potential HSP symptoms. At work I deal in noise and vibration, and so this had caught my attention.

Wearing my "work" hat I was determining that generally we dont experience vibration that often, and I know that humans are quite sensitive to vibration. I was trying to think of a situation when you would know that it is vibrating whilst at the same time noticing that you weren't feeling it in your legs. I couldn't think of a situation like this!

However, earlier in the year I went to the HSP clinic and they tested my vibration sensitivity, and it was actually very easy. A tuning fork was set vibrating, and the handle was then placed on my leg - could I feel it? And so, my vibration sensitivity is retained for now (at least).

I wondered where the origins of this came from. A quick bit of internet searching suggests that the use of a tuning fork to detect vibration sensitivity is usually credited to Heinrich Rumpf, who published findings in 1889.

Various extracts from articles:

The use of the tuning fork to clinically test vibration sense is generally credited to Heinrich Rumpf, professor and director of the poliklinik in the University of Marburg, who published his findings in 1889. A controversy surrounded its significance. Tomson, Treitel, Rydel, and Seiffer argued that it was a discrete sensation, sometimes impaired when touch and pressure sensation were preserved, in tabes and polyneuritis. Egger in Dejerine’s clinic, and Schwaner, thought that the receptors lay in the periosteum but the sensation (pallaesthesiae) was conducted by bone. However, vibration sense was not generally accepted as a valid clinical test for another 10 years.
https://jnnp.bmj.com/content/65/5/728

 Although vibratory sense had been described by Cardano and Ingrassia in the 16th century and tests had been developed by Rinne and Rumpf by the 19th century, it was not until 1903 that Rydel and Seiffer found that vibratory sense and proprioceptive sense were closely related and that both senses were carried in the posterior columns of the spinal cord. By 1955, the sensory examination included tests for light-touch, superficial pain, temperature, position sense, vibration, muscle (deep pain), and two-point discrimination.
https://www.thieme-connect.com/products/ejournals/abstract/10.1055/s-2002-36762

I couldn't find an easy article describing when vibration sensitivity became part of the standard neurological test, but it would appear to be somewhere between about 1900 and 1955.

My next path is to look at early mentions of vibration with HSP, and see when that was first mentioned. Finding copies of old medical journals on-line for free is a challenge! This paper from 1963 notes that loss of vibration sense is one of the more frequent "additional" symptoms of HSP - it's about two thirds of the way down the left hand column on page 518.  here https://jnnp.bmj.com/content/jnnp/26/6/516.full.pdf. Assuming that the text on P518 is referring to the referenced articles, it suggests that it was spotted either in the early 1900's or in the 1950's. Further investigation is required.

I note that this paper also has references for a load of the early HSP papers!

Taking control of your healthcare system

As I have been going through my HSP journey it has become apparent that I have to be in charge of my healthcare. I perceive that if I didn't do this things just wouldn't happen.

Whilst there may be a degree of local circumstances in my case, i don't think that I am untypical. It is also my preference to know what is going on and to be involved with sorting it out.

Here in the UK it is the Doctor (General Practitioner, or GP) who is able to give access to other parts of the health system, so it is useful for them to know you. In my situation the GP has many doctors, and I rarely see the same one on consecutive appointments. All other parts of the health service write to your GP with their findings, and the GP then makes the relevant booking/prescription/etc.

I have found that I have needed to chase up appointments, and make sure that things are in progress. As HSP is a rare disease, i often have to give a summary of what is going on and why we are trying to do things.

Summary of ley appointments:

Doctor appointment in 2014:

• Referral to HSP clinic - OK although some confusion over clinic code
• Referral to low mood clinic - easy
• Eliminated some bowel problem causes, started path to see others.

From 1st HSP appointment:

• Bladder medication - prescription from GP - easy!
• Referral to neuro-physiotherapist - easy. Stayed on their books.
• Referral to orthotics - easy.
• Bowels discussion - needed to chase to gey appointments, took a long time.
• Ultrasound scan of bladder - discussion, no action.

From 2nd HSP appointment:

• Booked directly with clinic as returning patient. Some hassle
• MRI scan - had to send clinic letter to scan team to show need. Lots of hassle. Also had to chase getting results to HSP clinic.
• Bladder ultrasound - discussed not needed, but appointment given.

My advice is:

• Know what appointments you are trying to get and make sure that you get confirmation of things.
• Assume that no-one talks to each other and that no-one knows what HSP is or how it affects you. You are the expert!
• Realise that a slow acting condition can be a good thing in terms of having to wait for appointments!
• Keep copies of your letters from clinics/appointments so you can refer back to them.
• Note down questions as they come to you, then you only need to find your notes at appointment time!

Various awareness days

Here is a calendar of various awareness days and weeks throughout the year.

February
It is Rare Disease Day on the last day of February (https://www.rarediseaseday.org/) - this is relevant because HSP is a rare disease!

March
The 1st March is International Wheelchair Day (https://internationalwheelchairday.wordpress.com/) - this is relevant as many with HSP need a wheelchair to help their mobility.

March 16th 2019 was Disabled Access Day, which is scheduled to return in 2021 (https://www.disabledaccessday.com/home/). The day is about encouraging people to try something new. This is relevant as many with HSP have difficulties getting into places and undertaking activities.

May
During May is Mental Health Awareness Week (https://www.mentalhealth.org.uk/campaigns/mental-health-awareness-week) - this is relevant because many with HSP have difficulties with depression, stress or isolation

June
In the middle of June is Carers Week (https://www.carersweek.org/) - this is relevant because many people with HSP need care, and some with HSP are providing care for others.

Near the end of June it is Small Charity Week (https://smallcharityweek.com/) - this is relevant because the charities that support HSP are often small, dealing with a rare disease.

September
On 21st September it was Footdrop Awareness Day (https://www.nationalfootdropsociety.com/) - this is relevant because footdrop is a common symptom of HSP

At the end of September it was National Inclusion Week (https://www.inclusiveemployers.co.uk/national-inclusion-week/about) - this is relevant because many with HSP have difficulties at work trying to balance their HSP with their jobs.

October
The 10th October was World Mental Health Day (https://www.mentalhealth.org.uk/campaigns/world-mental-health-day) - this is relevant because many with HSP have difficulties with depression, stress or isolation

December
Coming up on 3rd December is the International Day of Persons with Disabilities (http://www.un.org/en/events/disabilitiesday/) - This is relevant because many with HSP consider themselves to be disabled.

I'll update this page as I become aware of other dates in the year.

(I started this post to note inclusion week, mental health day and persons with disabilities day. Then I realised there were other things I'd noted throughout 2018)

2018 Survey Questions (for translation)

The table below is all the questions from my 2018 survey. Its here to allow me to get an easy google translate of all of them. Use the translate tool on this blog to convert this list to the language of your choice. Answer in the questionnaire: http://hspjourney.blogspot.com/2018/09/2018-survey-open.html

Question	English
	1) Personal Details
	In this survey I ask for answers to two questions, your name and your country. I dont mind if you use your real name or a pseudonym of your choice, just enough to allow me to track people who may come back and answer any subsequent surveys. I am also collecting e-mail addresses, should you wish to be contacted about the results of this survey or participation in future surveys. All other questions are optional, so answer if you want or dont if you dont want to. After this page there are 7 more pages: 2) Mobility, 3) HSP Symptoms, 4) Sleep, 5) Activities of Daily Living 6) Support and Tracking, 7) Wellbeing, 8) This questionnaire and my blog By completing this survey your results will be used in the analysis to paint a picture of certain aspects of HSP. All analysis is anonymised. Your information will not be shared with any other person/company. The survey data is backed up using on-line file storage systems.
1	What is your name?
2	What is your City/Town/State/Province/County?
3	What country do you live in?
4	What is your e-mail address (for contact about these surveys)?
5	I only wanted to be e-mailed about these things:
5	The results this questionnaire
5	Future questionnaires
5	Not at all
6	Do you have HSP?
6	I have HSP - genetic test
6	I have HSP - other diagnosis
6	I am answering on behalf of someone else with HSP
6	I do not know if I have HSP
6	I do not have HSP
7	If known, what type of HSP do you have? (e.g. SPG4)
8	Were you diagnosed with any other conditions before you were diagnosed with HSP?
8	Yes
8	No
9	If yes, describe or list your other diagnoses.
10	Is there HSP in your family?
10	Other members of my family have HSP
10	Other members of my family do not have HSP
10	Some members of my family may have HSP
10	I do not know if other members of my family have HSP
11	Do you have any other long term health conditions?
11	Yes
11	No
12	If yes, please describe those conditions.
	2) Mobility
	These questions allow me to understand your level of mobility and to correlate your answers here with the other questions.
13	How does HSP affect your mobility at the moment? How did it affect your mobility in 2013?
13	HSP does not affect my mobility
13	I walk without aids, but there are some effects
13	I use walking sticks/poles/crutches some of the time
13	I use walking sticks/poles/crutches most of the time
13	I use walking sticks/poles/crutches all of the time
13	I use FES
13	I use orthotics/AFO
13	I use a rollator/walking frame some of the time
13	I use a rollator/walking frame most of the time
13	I use a rollator/walking frame all of the time
13	I use a wheelchair/mobility scooter some of the time
13	I use a wheelchair/mobility scooter most of the time
13	I use a wheelchair/mobility scooter all of the time
13	At the moment
13	In 2013
14	How has your mobility changed over the last 5 years?
15	How far can you walk?
15	I can walk as far as I want to
15	My spasticity means I get exhausted after a distance beyond 500m
15	I can walk up to 500m
15	I can walk up to 10m
15	I am unable to walk
16	Do you consider yourself to be disabled?
16	Yes
16	No
	3) HSP Symptoms
	This section explores which HSP symptoms you have. Most of the symptoms listed here are HSP symptoms, with some from 'pure' HSP and some from 'complex' HSP. Some of the symptoms listed are more general neurological symptoms.
17	Please select how you are affected by the following mobility symptoms.
17	Difficulty walking
17	Difficulty running
17	Difficulty using stairs
17	My muscles are stiff
17	My muscles are weak
17	Loss of balance
17	Regular falls
17	Get more stiff when it is cold
17	Get more stiff when it is hot
17	Do not have
17	Occasional symptom
17	Minor symptom
17	Frequent symptom
17	Regular symptom
17	Most of the time
17	All of the time
18	Please select how you are affected by the following muscular symptoms.
18	Stiffness/spasticity in arms/upper body
18	Poor co-ordination
18	Back/hip pain
18	Leg/foot pain
18	Clonus (jumping feet or other muscle spasms)
18	Epilepsy or seizures
18	Pes cavus (arched/high feet)
18	Hammer toes (toes curl under)
18	Affected by bladder problems
18	Affected by bowel problems
18	Difficulty swallowing
18	Legs swell up
18	Feet swell up
18	Do not have
18	Occasional symptom
18	Minor symptom
18	Frequent symptom
18	Regular symptom
18	Most of the time
18	All of the time
19	Please select how you are affected by the following other symptoms.
19	Fatigue
19	Stress
19	Depression
19	Numbness
19	Pins and needles
19	Ichthyosis (widespread persistent thick, dry, "fish-scale" skin)
19	Loss of vibration sensitivity in legs
19	HSP affecting learning or memory
19	HSP affecting hearing
19	HSP affecting vision
19	HSP affecting speech
19	HSP affecting sexual function
19	Fail to remember dreams
19	Changes in perceptions of smell/taste
19	Do not have
19	Occasional symptom
19	Minor symptom
19	Frequent symptom
19	Regular symptom
19	Most of the time
19	All of the time
20	Of these symptoms, describe which ones have the greatest overall effects/impacts for you.
21	Any other comments on these symptoms, or describe other symptoms you have.
	4) Sleep
	This page explores your sleep quality.
22	Please think about your sleep over the last four weeks and select the choice which best describes your situation.
22	Did you have difficulty falling asleep, staying asleep, or feeling poorly rested in the morning?
22	Did you fall asleep unintentionally or have to fight to stay awake during the day?
22	Did sleep difficulties or daytime sleepiness interfere with your daily activities?
22	Did work or other activities prevent you from getting enough sleep?
22	Did you snore loudly?
22	Did you hold your breath, have breathing pauses, or stop breathing in your sleep?
22	Did you have restless or "crawling" feelings in your legs at night that went away if you moved your legs?
22	Did you have repeated rhythmic leg jerks or leg twitches during your sleep?
22	Did you have nightmares, or did you scream, walk, punch, or kick in your sleep?
22	Did pain disturb you in your sleep?
22	Did other physical symptoms disturb you in your sleep?
22	Did worries disturb you in your sleep?
22	Did medications disturb you in your sleep?
22	Did needing the toilet disturb you in your sleep?
22	Did other factors disturb you in your sleep?
22	Did you feel sad or anxious?
22	Did you sleep in a bed at night?
22	Did you sleep in a chair at night?
22	never
22	sometimes
22	usually
22	always
22	Please describe any other factors which disturbed you in your sleep?
23	Any other comments on sleep
	5) Activities of Daily Living
	This page explores activities of daily living
24	Please select the answer which best describes your bathing.
24	Bathes self completely or needs help in bathing only a single part of the body such as the back, genital area or disabled extremity.
24	Needs help with bathing more than one part of the body, getting in or out of the tub or shower. Requires total bathing.
25	Please select the answer which best describes you getting dressed
25	Gets clothes from closets and drawers and puts on clothes and outer garments complete with fasteners. May have help tying shoes.
25	Needs help with dressing self or needs to be completely dressed.
26	Please select the answer which best describes your toileting
26	Goes to toilet, gets on and off, arranges clothes, cleans genital area without help.
26	Needs help transferring to the toilet, cleaning self or uses bedpan or commode.
27	Please select the answer which best describes your transferring
27	Moves in and out of bed or chair unassisted. Mechanical transferring aides are acceptable.
27	Needs help in moving from bed to chair or requires a complete transfer.
28	Please select the answer which best describes your continence
28	Exercises complete self control over urination and defecation
28	Is partially or totally incontinent of bowel or bladder
29	Please select the answer which best describes your eating
29	Gets food from plate into mouth without help. Preparation of food may be done by another person.
29	Needs partial or total help with feeding or requires parenteral feeding.
30	Any other comments on these activities of daily living?
	6) Support and Tracking
	This page examines if you are a member of a support group and how you track your progress.
31	Are you a member of a support group?
31	Yes
31	No
32	If yes, describe which support group you are a member of.
33	If yes, what do you think the key benefits of membership are?
34	If no, which of these best describe why?
34	i dont feel the need to be a member of one
34	I am not aware of any support groups
34	There are no groups near where I live
34	I feel it would be too much effort to be a member
34	I dont think the group would give me the support I need
34	Another reason
35	Do you track the progress of your symptoms?
35	Yes
35	No
36	If yes, describe how you track this
	7) Wellbeing
	This page investigates wellbeing.
37	Please select the option that best describes your feelings and thoughts over the last 2 weeks
37	I’ve been feeling optimistic about the future
37	I’ve been feeling useful
37	I’ve been feeling relaxed
37	I’ve been feeling interested in other people
37	I’ve had energy to spare
37	I’ve been dealing with problems well
37	I’ve been thinking clearly
37	I’ve been feeling good about myself
37	I’ve been feeling close to other people
37	I’ve been feeling confident
37	I’ve been able to make up my own mind about things
37	I’ve been feeling loved
37	I’ve been interested in new things
37	I’ve been feeling cheerful
37	None of the time
37	Rarely
37	Some of the time
37	Often
37	All of the time
	8) My Survey and Blog
	This final section aims to capture your awareness of my surveys and blog.
38	Did you take part in one or more of my previous surveys?
38	Yes, completed at least one
38	I started to fill out some answers
38	Didn't participate - I wasn't aware of these
38	Didn't participate - didn't want to answer
39	If you wish, please give me feedback about this survey, previous survey, or on my analyses.
40	Have you read my blog http://hspjourney.blogspot.co.uk?
40	I read regularly
40	I read occasionally
40	I have not read
41	If you wish, please give me feedback about my blog - perhaps things you like/dislike/would like to see etc.
42	If you read my blog, how did you become aware of it?

Annual survey - The background, the reasons and the limits!

Yesterday evening I used google translate to translate the "my survey is now open" message into various languages and post this on a multitude of different HSP groups on Facebook. There are a number of common questions which come up, and this blog post explores answers for these. Most of these questions have come up in previous years on the English groups, so the answers are still relevant.

One beauty of Blogger is the translate tool, allowing people from all over the world to read these posts.

Why do you want this data?

Back when I first had my diagnosis I was exploring websites giving the different HSP symptoms. There was little information about what they were and how common they were, and there was also little information about how quickly HSP progresses. Eventually I decided to find out more information by asking other people with HSP, and that formed the basis for my first survey in 2013. I set up an on-line questionnaire, and asked people to complete via support groups and facebook groups. There was a lot of interest in the results of this, and this set the precedent for the other surveys. Each year I look at a small number of topics and ask questions. I write up the results, and that gives others with HSP the same information to compare their own situation against.

I dont speak English....

This question comes up each year - can I see the questionnaire in another language? It is a tricky question. the constraints are:

1. Access to a survey website which meets the requirements
2. Making sure that translated questions have the same meaning as my English questions
3. Time spent setting up, managing and analysing the results.

On the first, all of the free on-line survey hosting sites are limited to single language and a maximum of 100 responses. I easily get more responses than that, but I dont wish to spend my own money on a monthly subscription for a survey platform which I use once a year. The current solution is that I have a log-in to my employers survey platform, with the agreement that I can use it for one survey - it is the single language option. Some platforms do have multi-language options, but they are even more expensive.

On the second, when I have been looking at which standard tools I want to use to ask questions about certain HSP symptoms I spot that some of them have been translated into other languages, and been demonstrated to give the same results. By keeping to one language I avoid having to add another of interpretation. The other language list this year is: Spanish, French, Italian, Portuguese, German, Dutch, Swedish, Danish, Greek and Japanese. (Some of those groups are listed here: http://hspjourney.blogspot.com/2017/03/community-pages-2017.html)

On the third point, its very personal. I'm fitting in the survey and write up around my family and my job.  I think that running 11 surveys instead of one would at least double the amount of time that it takes me to do this.

Conclusion - I would be happy to run surveys in more than one language if someone else would pay to host these - a single sign in with a multi-language option would be best. I would also need to talk with others fluent in both English and those languages to make sure that the questions mean the same things.

Late Sept Update - since writing this I've now Google translated the questions into these other languages (and Norwegian) as a trial. I realise that I haven't had the translated meanings checked. I'll see what this does for my number of respondents and the analysis in the new year.

*** Please drop me a line if you are interested in helping! ***

Why dont you ask about....

I select a small number of topics each year. Some of these I will repeat on a 5 year rotation, others will come and go as needed. Summary:

Mobility, symptoms, misdiagnosis - 2013

Medication, exercise, diet, relaxation - 2014

Home modifications, depression, quality of life - 2015

Fatigue, bladder, bowel, HSP information - 2016

Pain, walking, occupation, wellbeing - 2017

Mobility, symptoms, misdiagnosis, sleep, daily living - 2018 

Possible future topics: Travel, grief, dating/relationships, life space logging, getting support, diagnostic tools used. 

I'm likely to repeat the 2014 medication questions in my 2019 survey.

Why dont you ask about my condition?

Some of the facebook groups are for HSP and similar topics. I get asked why I dont include questions for other conditions. The main reasons are; firstly the same as why I havent added additional languages - the extra time and effort involved, and secondly that I dont have a good understanding of what the key issues are for people with these other conditions. There would be a learning curve to understand this.

*** I am happy to discuss with anyone else my question selection process and my data analysis/interpretation processes, so that they could set up a similar survey for any other condition. Please drop me a line if you want to do this ***

Is my data safe?

The process I use is - use a survey platform to host the questions. I download the data to my PC and do all the analysis in Excel. I send emails using the BCC field for addressing. I included this data statement at the start of this years survey: 

"By completing this survey your results will be used in the analysis to paint a picture of certain aspects of HSP. All analysis is anonymised. Your information will not be shared with any other person/company. The survey data is backed up using on-line file storage systems."

2018 Survey Open

After the success of my previous surveys, and feedback from readers and others, I'm continuing the pattern with another survey this year.

My focus for this survey is understanding:

• HSP Symptoms
• Sleep
• Activities of Daily Living
• Support group membership and Progress tracking
• Misdiagnosis

There are a range of questions for each topic. I have designed my own questions for symptoms, support group membership and misdiagnosis. Sleep is assessed using the Global Sleep Assessment Questionnaire (with a couple of additional questions added). Activities of Daily Living are assessed using the Katz index.

Last year I had good opportunity to explore which HSP factors affected Wellbeing, and these questions are repeated from last year, using the Warwick-Edinburgh Mental Well-being Scale (WEMWBS) for assessing positive mental health.

The list of HSP symptoms is expanded from my first survey in 2013, and I will examine change in symptoms between 2013 and now, where I have data. I will also examine change in mobility over the period I have been running surveys for those who have completed more than one survey.

Following the previous pattern, I will collect results until early 2019, then analyse these to publish the results here on rare disease day, 28th Feb 2019.

Also like before, all questions are optional (apart from your name and country). If you have taken part in any of my surveys before, I'd appreciate you using the same name to allow tracking.

Therefore, I would appreciate any readers with HSP to complete this years survey:
https://www.surveymonkey.co.uk/r/HSP2018AL

If you dont speak English then the following links will take you to a PDF which has the questions in English and other languages to help you answer. I will use Google translate to get your answers back to English for the analysis
https://drive.google.com/open?id=1yQhHgn-Q8hXxhwJrx19av6cWO1olG1tP (French)
https://drive.google.com/open?id=1MLAIichys6Xc3ueGJ9VGwcdkfOzjWs7U (Portuguese)

https://drive.google.com/file/d/1h0io177kouGr1PTvwTOKm7qJiO6mPhLc (Italian)
https://drive.google.com/open?id=1O7uIE-n3aLiBlY5f9lzd3kCcHYCCdq6f (Dutch)
https://drive.google.com/open?id=1VjHdjUoNZb3S34AIImBGF_C4wbpWItbh (Spanish)
https://drive.google.com/open?id=1-SAnAHoUObvZ6M1qrEkQCB0nB9sQUnWQ (Swedish)
https://drive.google.com/open?id=1ildph84q9ZxVDKDbI3TsonxAZjqG0UxO (Danish)
https://drive.google.com/open?id=17NCcijYAmLZubl86EaS7IJRsPR83JQIn (German)
https://drive.google.com/open?id=1tKimZpFMP-RM8XbvUyZe-zGugUgOPBbv (Japanese)
https://drive.google.com/open?id=1hAf0U3Wv7kEHSx6FFMKr6OtVazSGVGTD (Greek)
https://drive.google.com/open?id=1pR8dRWArU-wvYuzWKwqzSy4C3at4BDBk (Norwegian)

** 27th November Update **

Hi. I’m collecting answers to my HSP survey for about another month. I’ve over 200 responses so far. This is an initial look at two questions.

For symptoms, fatigue is one symptom which about one third of people feel affects them the most. Others are:

Fatigue	34%
Mobility	27%
Stiffness/weakness	19%
Balance	17%
Pain	17%
Bladder/Bowel issues	15%
Sexual function	6%
Cognitive issues	6%

Looking at sleep – over half of people usually or always have difficulty falling asleep, staying asleep, or feeling poorly rested in the morning. The biggest factor is needing the toilet where nearly half of people are affected by this usually or always. Other factors are:

Need the toilet	44%
Repeated jerks or twitches	30%
Feeling restless	27%
Disturbed by pain	27%

Over a quarter fall asleep unintentionally or have to fight to stay awake, and over a quarter have sleepiness interfering with daytime activities.
 

I’m very pleased to see answers other than in English, it is expanding my audience. I would be pleased to see more data, so if you have a spare 20 minutes, please give your answers.

** 5th October Update **

I have over 150 responses so far, the most ever at this time! Thanks to everyone who has completed so far. People are taking about 20 minutes to complete this survey.

Initial headlines:

·       About 80% of people regard themselves as disabled.

·       About a third of people were mis-diagnosed prior to their HSP diagnosis.

·       About a third of people have another long term health condition alongside their HSP

·       About 60% of people are a member of a support group

·       Symptoms affecting the most people:

o   Difficulty running/walking – 90%

o   Difficulty with stairs and/or balance – 85%

o   Feeling more stiff when cold and/or fatigue – 80%

o   Pain in lower body, bladder problems, stress, clonus – 60%

·       There is a big overlap in muscles feeling stiff and muscles feeling weak, each around 85%

I’d be really pleased to hear from more, so if you have HSP and a spare 20 minutes, please take part. If you dont speak English there are translation sheets for the questions into French, Portuguese, Italian, Dutch, Spanish, Swedish, Danish, German, Japanese, Greek and Norwegian. 

** 27th Sept Update **

Thanks again to those continuing to answer. I have now made those translation sheets for the other languages of the Facebook groups I have posted survey links to
https://drive.google.com/file/d/1h0io177kouGr1PTvwTOKm7qJiO6mPhLc (Italian)
https://drive.google.com/open?id=1O7uIE-n3aLiBlY5f9lzd3kCcHYCCdq6f (Dutch)
https://drive.google.com/open?id=1VjHdjUoNZb3S34AIImBGF_C4wbpWItbh (Spanish)
https://drive.google.com/open?id=1-SAnAHoUObvZ6M1qrEkQCB0nB9sQUnWQ (Swedish)
https://drive.google.com/open?id=1ildph84q9ZxVDKDbI3TsonxAZjqG0UxO (Danish)
https://drive.google.com/open?id=17NCcijYAmLZubl86EaS7IJRsPR83JQIn (German)
https://drive.google.com/open?id=1tKimZpFMP-RM8XbvUyZe-zGugUgOPBbv (Japanese)
https://drive.google.com/open?id=1hAf0U3Wv7kEHSx6FFMKr6OtVazSGVGTD (Greek)
https://drive.google.com/open?id=1pR8dRWArU-wvYuzWKwqzSy4C3at4BDBk (Norwegian)

** 22nd Sept Update **

Many thanks to all the people who have answered the questionnaire to date.

There have been questions about if I can ask the questions in a different language. I have posted a page which contains all the questions, and you can use the Google Translate tool to convert from English into your language of choice:
http://hspjourney.blogspot.com/2018/09/2018-survey-questions-for-translation.html

The highest demand has been for French and Portuguese, so I have made a PDF which places the English next to the French or Portuguese so that you can print/have on screen and use this as a tool to answer questions.
https://drive.google.com/open?id=1yQhHgn-Q8hXxhwJrx19av6cWO1olG1tP (French)
https://drive.google.com/open?id=1MLAIichys6Xc3ueGJ9VGwcdkfOzjWs7U (Portuguese)