10 years of blogging!

It was on 23rd June 2010 that I wrote my first blog post here. I didnt know at that time what I was really planning to do. I had decided that two posts a month would be a good level of posting, and 261 posts later I think that was the right decision to make (there have been a few months where I have posted more than twice!).

Interestingly, FaceBook told me earlier that 23rd June was also the date when I put mu my first poster at the Spatax meeting in 2016, so it looks like this may be a memorable date for me!

If you'd like to go back to where it all began - follow this link: https://hspjourney.blogspot.com/2010/06/my-history.html

If you'd like to see about my 2010 Spatax poster - follow this link: https://hspjourney.blogspot.com/2016/06/international-meeting-on-spastic.html

What with Coronavirus taking a fair proportion of my time (in one way or another) since March, I've not really had a chance to make lots of noise about my survey results - I'm planning to do that a bit more after the AGM for the UK Support Group (4th July - https://hspgroup.org/) and before I launch my 2020 survey in September.

My 2019 survey results are here: https://hspjourney.blogspot.com/2020/02/2019-survey-results.html

The key points on medication is that there is no single point source of information for this, and many drugs that people are taking dont have published results showing benefits for those with HSP. Regular travel, music and exercise were shown to suggest better wellbeing, and being lonely/isolated and needing to take many medicines suggested lower wellbeing.

I'd like to thank everyone who has taken the time to read my blog posts over the years - regularly, occasionally or just by chance! I get lovely feedback, and I am pleased to know that what I say helps others on their journey with this Rare Disease!

HSP pain

This post is another one where I explore a symptom of HSP - this time pain.

My 2013 survey showed just under 80% of people with HSP had back pain, with similar results in my 2018 survey. I looked in more detail at pain in my 2017 survey, where around 80% of people get some pain with their HSP. Of these, over 95% have some pain in their legs, over 90% have pain in their feet, and over 80% of have some pain in their back and hips.

There are a few published papers on this:

Out of 109 people with HSP in The Netherlands with pure HSP in 2020, 72% reported leg and/or back pain: Experienced complaints, activity limitations and loss of motor capacities in patients with pure hereditary spastic paraplegia: a web-based survey in the Netherlands - 

https://ojrd.biomedcentral.com/articles/10.1186/s13023-020-1338-4

Depression and pain were significantly increased in a study of 118 SPG4 patients in Germany in 2020: Non-motor Symptoms Are Relevant and Possibly Treatable in Hereditary Spastic Paraplegia Type 4 (SPG4) - https://pubmed.ncbi.nlm.nih.gov/31646384/

A study of 108 people with HSP in Norway in 2016 reports that those with HSP more frequently report musculoskeletal pain compared with controls: Health survey of adults with hereditary spastic paraparesis compared to population study controls - https://ojrd.biomedcentral.com/articles/10.1186/s13023-016-0469-0

34 patients with SPG4 in Brazil reported higher pain, fatigue and depression than controls in 2016: Non-motor Symptoms in Patients With Hereditary Spastic Paraplegia Caused by SPG4 Mutations - https://pubmed.ncbi.nlm.nih.gov/26806216/.

My 2017 survey showed 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 were 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.

Furthermore, people who do not get pain from HSP have a better wellbeing than those who get pain from HSP.

My 2019 survey looked at medication. Respondents taking medicine for pain form the second largest group of around one fifth of all the medication being taken (the biggest group being medication for spasticity and spasms). 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. Further analysis of that group was not undertaken.

The second group includes some medications that are used to treat effects of HSP, and these include:

Pregabalin/ Lyrica, Duloxetine, Pramipexole, Oxycodone, Nabilone and Nabiximols. Whilst there are no papers reporting these drugs for use in HSP all but Nabilone are indicated as potential treatments on some HSP websites.

Links to my surveys mentioned:

2013: http://hspjourney.blogspot.co.uk/2014/02/hsp-survey-results.html
2017: http://hspjourney.blogspot.com/2018/02/2017-survey-results.html
2018: https://hspjourney.blogspot.com/2019/02/2018-survey-results.html
2019: http://hspjourney.blogspot.com/2020/02/2019-survey-results.html

Until recently pain as an HSP symptom was routinely experienced by people with HSP but there were no published papers identifying this as a symptom to the wider healthcare community. 

Fortunately this issue is beginning to be addressed - the 2020 paper from Germany concludes: "We recommend that clinicians regularly screen for depression, pain, and fatigue and ask for bladder, sexual, and defecation problems to recognize and treat non-motor symptoms accordingly to improve quality of life in patients with SPG4." It is my opinion that this approach would work well with other types of HSP as well.

Symptoms update - uneven legs, and feet

A bit of a symptoms update, mostly around foot care.

I had noticed some time ago that I was 'gathering' dead skin on my feet. I had been avoiding scrubbing the bottoms of my feet in the shower because it was difficult to do that, and I had more important things to get to (like a coffee!)

I decided recently that this situation had to change, and I shouldn't leave the dead skin on my feet, so I started two things - a foot care routine, and adding stretches in to my routine to make it less difficult to clean the bottom of my feet in the shower.

So, on the foot care front, I found an old foot bath in the garage which I resurrected and soak my feet for half an hour every now and again. I also bought a range of tools for dealing with my feet, the most vicious of which looks like a rasp, and there are a few files and a pumice stone. I've been scraping the dead skin off every now and again, and applying moisturiser most days. Readers should note that foot care is not my speciality, this approach just felt right for me after a quick bit of Googling! Its an ongoing project, but there is definitely less dead skin on my feet now!

On the stretch front I have added the simple stretch of sitting on the floor with my legs crossed. I cannot do this without leaning back on something, so I sit with my back against some furniture and then get my legs as far crossed as they will go. I focus on trying to get my knees to go down, hold for about 30 seconds and then swap my legs over (so the other foot is closer to my body) and repeat.

The stretches are working because it is becoming easier to be able to get to the bottom of my feet in the shower, so that is good (and hopefully the footcare means I will get less dead skin building up).

The interesting thing to note is that my gait is most affected on my left leg - that is the shoe which get the wear/damage quicker. For foot cleaning it is more difficult for me to lift my right foot. This tells me that I am getting degradation in both legs, but that degradation seems to be affecting different muscles in each leg - and why this post has "uneven legs" in the title!

The great unknown is why I was collecting the dead skin there in the first place. I suspect that a big part of this is lack of regular scrubbing of my feet. But, I also think that part of it will be due to my insoles causing my foot to be at a different angle in my shoe which will re-distribute my weight to different parts of the foot.

I think that the message I would like people to take away is that stretches can help get movement back. It is a question of working out which muscles are tight, and then finding a way of regularly stretching those muscles so that they become longer and are used more regularly. You should notice a change with a new stretch (the change may be small and it may take a few days/weeks to be noticeable)