Exoskeleton for HSP approved in Japan

 I saw this in the news the other day. 

A lower body exoskeleton has been approved in Japan for the treatment of HSP. The exoskeleton is meant to improve gait and reduce progression. The various documentation points to a clinical trial, but the results do not appear to be published. 

The trial appears to have begun in 2016 and was completed in 2018. They seem to have looked at both people with HSP and with HTLV-1-associated myelopathy (HAM). In total 42 people took part, but it doesn't say how many had HSP.

The trial had people using the exoskeleton and a hoist in 40 minute blocks, nine times within one month. The 40 minute period comprised a 5 minute warm-up, 30 minutes of gait training and 5 minutes of cool down. A control was run using the same 40 minute blocks with a hoist but not the exoskeleton. The primary outcome was a two minute walking test, and various secondary measures were also measured.

The exoskeleton reads bioelectric signals and adjusts the power of the exoskeleton in response. They call this process interactive biofeedback, and identify that this process can regenerate some neural function. 

No results are given to identify what the benefits were for people with HSP or how long those benefits lasted, but it is interesting to think that this may be an option to help people with HSP. One of the doctors who ran the test is quote in a press release saying that the exoskeleton significantly improved gait (in the primary and clinically important secondary endpoints) in HAM and hereditary spastic paraplegia. The trials demonstrated that Cybernics Treatment can regenerate neural function through interactive biofeedback (iBF).

This exoskeleton is also used to similarly treat other neurological conditions: Spinal muscular atrophy (SMA), Spherospinal muscular atrophy (SBMA), Amyotrophic lateral sclerosis (ALS), Charcot-Marie-Tooth disease, Distal myopathy, Inclusion body myositis, Congenital myopathy, Muscular dystrophy.

Here are the various links I read to write this post:

https://exoskeletonreport.com/2022/11/medical-hal-exoskeleton-approved-for-treatment-of-ham-and-hereditary-spastic-paraplegia-in-japan/

https://www.cyberdyne.jp/english/company/PressReleases_detail.html?id=12909

https://exoskeletonreport.com/product/hal-lower-limb/

https://www.cyberdyne.jp/english/services/CybernicsTreatment.html#:~:text=Cybernics%20Treatment%20is%20a%20treatment,stroke%2C%20neuromuscular%20diseases%2C%20etc.

https://dbcentre3.jmacct.med.or.jp/JMACTR/App/JMACTRE02_04/JMACTRE02_04.aspx?kbn=3&seqno=5969#OtherInformation

2022 Blog Data and Health Review

One of my tasks for the passing of the new year is to update the pages of this blog so they are up to date. I have now done this. Readers may have spotted that there are 4 pages, and this post gives an update of the changes and observations from each page. A review of my health first, followed by blog statistics and the two straightforward updates.

Health Review: http://hspjourney.blogspot.com/p/symptoms-timeline.html

Once again I've a spreadsheet which tracks various health data, which I publish headlines from on my symptom tracker page. The key observation from 2022 is that my cycling distance has shrunk considerably and at the same time my weight has increased also considerably. I think that its fair enough to say that my regular cycling was helping keep my weight in check.

During coronavirus pandemic lockdowns I was able to decrease my weight with a low point at end of 2020/start of 2021. I did this by paying more attention to the sizes of my portions. It looks like I'll need to do that again during 2023 to help target a more healthy weight.

This graph shows my cycling since I started tracking data, with the yellow colour showing my commute cycling and the blue showing my other cycling (in km on the right hand axis). The other main point to note from this graph is that my maximum speed in 2022 was generally slower than my minimum speed in 2016/early-2017 (in km/hr on the left hand axis). 

Part of my reasons for tracking was to see how average speeds were changing over time, with the minimum, maximum and average speeds staying reasonably level up until around 2020.

The data for number of steps and active minutes have decreased similarly with the cycle distance, and I have realised that the step count is more of an activity tracker than a specific step counter. I'm considering trying out an electric bike so that I can maintain my level of activity whilst my spasticity makes cycling harder work. My other thought process is to consider a rowing machine to help my upper body strength in preparation for that point when I'll need to begin using a wheelchair. 

In terms of other data being tracked, I note that my sleep has increased in the last few months, back up to where it was in 2017. There are two possibilities for this - one is around antidepressants and mood. One of the first things I noted when starting to take my antidepressants was a decrease in my resting heart rate, and my head is in a better place now than it was during 2019-2021. The other possibility is around bladder medication. 2017 was at the beginning of my bladder journey, and at that time I wasnt having to visit the toilet during the night. In 2020 I switched from one to two bladder tablets per day, and in 2021 I changed tablet type. Of these factors the mood one fits more easily into the sleep pattern.

The last pattern to note is alcohol. I drank less in 2022 than in any previous year, however the pattern is changing - on days when I have a drink I'm often drinking more, but there are fewer of these drinking days.

Blog Statistics: http://hspjourney.blogspot.com/p/blog-statis.html

On the whole this is a straightforward update. I've a spreadsheet which I update each year with the updated totals for each page, using a blend of both Blogger and Google Analytics data.

When looking at the total readership of pages, this follows the usual pattern. I up the threshold which I use for this data so that there are about 30-35 posts shown. However, the total readership of my blog in 2022 was lower than in recent years, so I've had to lower the in-year readership level to give the top twenty-ish posts for the year. 

I need to reflect on if there are any changes that I've unintentionally made in my posts, or some other change in what I've been doing to let the world know about my posts. A review of the list of posts suggests that there isnt much differences between topic posts in 2022 compared with previous years. It might come down in part to there being one fewer AGM presentation in 2022, or my depression/anxiety meaning that my posts are not so readable/not promoted as much. 2022 had fewer people arriving from other HSP/rare disease groups, so that may also be a factor. 

Index: http://hspjourney.blogspot.com/p/index.html

This was very easy, I have added all of the 2022 posts to the index in the relevant categories, so the index is up to date (well, until I post publish on this post....)

Survey Page: http://hspjourney.blogspot.com/p/my-on-line-resarch.html

Again, very easy update here. I had updated this early in 2022 with the table showing which topics were in which survey year, so the update for now was to simply add the abstract and links for my 2021 survey results.

Review of 2022

Annual Review: 2022

Its time for my annual reflection on thoughts and activities throughout 2022.

Knowledge

I feel that most of my knowledge gains this year have come from outside the HSP world - appreciating and understanding different perspectives on acceptance, disclosure and that side of things. 

I'm also using my annual survey to explore areas that don't seem to be addressed, with this year adding questions on menstrual health.

Symptoms

My key word for symptoms for 2022 is depression, although as noted in a recent post this is more to do with my life outside the HSP world than to do with my progression. 

Otherwise, the pattern of 2021 has continued - increased fatigue, needing to use arms more to stand up, needing to sit down more often, generally walking slower and with more effort. This years review with the neurologist put the start point for spasticity medication in clearer focus - when I start to find things difficult to do rather than just slower. 

This Blog

I'm also pleased with the continuing readership of this blog, with a similar audience and similar popular posts. One of the consequences of my depression is that I have had a bit less enthusiasm for writing posts this year, and its has been more challenging to keep to my personally set target of two posts a month.

However, I'm pleased that blog posts continue to be linked to and used within other HSP and RareDisease communities. Thank you to all readers, including those who make comments appreciating what I say. Such feedback reinforces the reasons for writing the blog. 

Survey

The annual pattern of my surveys is well established, and I am using the same seven languages as 2021. As in recent years I have collaborated across countries getting the questions right. Results will be out on 28th Feb after the majority of analysis during January.

Community Contribution

HSP Community activities for 2022 included:

• Representing the UK at EuroHSP
• Patient Journey for ERN: https://www.ern-rnd.eu/patient-journey-hereditary-spastic-paraplegias-hsps/
• Carrying on with the Enable disability network at work (Accessibility Challenge)
• Involvement with the ED&I group at the Institute of Acoustics
• Analysing and reporting the results of my survey to share with the HSP world

The main element of my community contribution comes from being chair of the HSP support group. Whilst we are getting various issues resolved, there are still many to go. I'm working with the rest of the trustees to ensure that the group continues to support its members in the most appropriate way - please reach out to help us.

HSP Patient Journey

One of my areas of work* in HSP has now been published. I've been working with friends at EuroHSP to help develop a patient journey for people with HSP for the European Reference Network for Neurological Diseases.

They have been running a project for a while to try and illustrate aspects of neurological diseases. I have been able to use the results of my surveys and conversations with many people who have HSP to try and get the views of those with HSP down.

You can see this here: https://www.ern-rnd.eu/patient-journey-hereditary-spastic-paraplegias-hsps/

The text at the top of this page describes the aim:

Patient Journeys  are info-graphical overviews that visualize patients’ needs in the care of their rare disease. Because Patient Journeys are designed from the patient’s perspective, they allow clinicians to effectively address the needs of rare disease patients.

Patient Journeys consider that patients’ needs may differ at different stages of the disease – e.g., initial symptoms vs. treatment. They also reflect the patients’ personal experiences, which may vary depending on the person, clinic and country.

ERN-RND considers Patient Journeys working documents that patients and clinicians can use together to identify gaps in care and adapt care pathways to better meet the needs of patients living with these conditions. Patient Journeys can therefore be seen as a first step toward systematic patient engagement in the design of care pathways.

In addition, Patient Journeys are a useful resource for patients, families, non-specialist clinicians, and the general public to understand the care needs of patients living with a rare neurological disease.

Each line on the journey covers a different aspect - the disease line gives details about HSP, the clinic line addresses different issues which might be encountered when talking with healthcare and other relevant professionals. The challenges line gives some of the challenges experienced by people with HSP at the moment. The goals line sets out some objectives which clinicians can hope to deliver in combination with people who have HSP.

There is a second sheet of the journey, which uses words to describe in a a little more detail these aspects.

I was quite pleased when talking about the graphics to make sure that we covered walking sticks, walking frames and wheelchairs. The team within the ERN were very accommodating to these ideas.

(*Note - thats work in the task-based sense. This, like my support group work is all voluntary)

Symptoms update - fatigue and walking speed

Reflecting back on posts this year, I can see that there have been quite a few more symptom update posts than in previous years. Perhaps this means that my symptoms are changing more quickly.

Two observations for this post - firstly I went away for a weekend with some friends the other week, which included walking between a few pubs one evening. It is quite clear to me that my walking pace is substantially slower than it was. As long as I keep at the pace I want to go I dont have any issues with tripping or balance, but if I try to walk faster then my feet do trip up more.

Secondly, I've been finding that I am feeling quite fatigued recently. There hasnt been much change in the number of hours that I sleep recently, but I am finding that I wake up tired, and feel tired for most of the day. I have been talking with a lot of people at work about disability this week, and I reflected that it might be my fatigue which hits me at work before any of the mobility issues.

A little more on that topic to round off this post. At work a colleague and I co-chair our companies employee network for people with disabilities and long term health conditions (alongside our usual day-jobs). We've had a week where we've been trying to raise awareness about many aspects of disability, including me doing a session wearing my Chair of the HSP Support Group hat. It has been really good to help people understand some of the many aspects that people with disabilities and health conditions have to deal with.

Nerve stimulation research update

I heard about this story on the radio one morning recently. The story reported how nine people who had lost their mobility through spinal cord injury were able to become mobile again, having used epidural electrical stimulation.

I had not heard of epidural electrical stimulation before. A quick search reveals that a small device is implanted over the protective coating of the spinal cord. The device then produces electrical currents to the lower part of the spinal cord which was not previously able to communicate with the upper part. (https://www.medicaldevice-network.com/comment/epidural-stimulation/) What I am not clear about is if this is referring to upper and lower in the context of the site of the issue with the spinal cord or if they are using upper and lower in terms of neurons. The image in the story (link below) shows a spinal cord injury high up along the spine and the device shown implanted in the lumbar spine.

We know that the upper motor neurons degrade over time, which breaks the chain of communication between the brain and the lower motor neurons. My understanding is that in HSP the degradation of the upper motor neurons occurs throughout the nerve rather than at a specific point along the nerve. This suggests that the lower part of the upper motor neurons will have had some degeneration, making external electrical stimulation more challenging. If the epidural electrical stimulation can talk with the lower motor neurons then I hope that there is a potential for use in HSP. I spot a gap here in my understanding of the spine and the upper and lower motor neurons! 

The other sides to the story I heard was that they were using an AI system with stimulating the nerves to be able to help people regain their mobility better. They also reported that general levels of nerve communication were lower when this was being used, which they were speculating was a big reduction in the feedback signals being sent back up to the brain (and not being received due to the spinal cord injury).

This latter aspect gave me thought for reflection - I know that feedback for things like bladder and bowel are affected, giving cause for the often experienced incontinence with HSP, and it also makes sense that some people lose their sensitivity to vibration in their legs. I suspect that I had been focussing most on the communication from the brain above the various forms of information that the brain receives back from the lower body.

The story which was talked about on the radio was this one, from Nature, from a team in Switzerland: https://www.nature.com/articles/s41586-022-05385-7

Potential Exoskeleton Walking Solution

I saw this article in New Scientist recently, which may have the potential to help those with HSP.

Various research into exoskeletons has shown that they can improve gait, but it is claimed that this research hasnt converted into real world benefits as all the studies have been in laboratory conditions. This study uses two prototype ankle exoskeletons to help people walk, and has used an AI system to learn about how people walk in real world situations and is able to deliver benefits in the real world.

This study used ten healthy participants in their 20s, and they report that there was a 9% increase in walking speed and a 17% reduction in energy used to walk. They found the exoskeleton relatively easy to used, and the AI is reported to be able to learn quickly. The paper reports that substantial development work would need to occur to develop this into a finished product.

It occurs to me that the spasticity from HSP makes people walk slower, less efficiently and people need to use more energy for the same walk. I wonder if this kind of product would help people in the earlier stages of HSP maintain their full independence for longer and make it easier to get out and about. 

New scientist article: https://www.newscientist.com/article/2341275-exoskeleton-boots-learn-how-you-walk-to-help-improve-your-gait/

Full paper: https://www.nature.com/articles/s41586-022-05191-1

Image of the exoskeleton from the paper:

I'm pleased to see that the full paper is open access, meaning that anyone can read about this.

Update from Australia

 There has been another interesting update from the Australian HSP research group.

As usual their updates are categorised, with research updates and living with HSP updates. On their living with HSP page they report the following:

• They acknowledge that as HSP is a chronic progressive condition, people are more at risk of mental health conditions. They highlight the Australian R U OK? campaign (https://www.ruok.org.au/), and there are similar campaigns in other parts of the world. I encourage anyone feeling low to try and seek out a conversation.
• They report a study from July identifying that mindfulness can separate pain and the suffering from pain (https://www.sciencedaily.com/releases/2022/07/220708162754.htm). Pain is a common symptom for people with HSP, and I hadnt thought that mindfulness could be a treatment to reduce the impact of pain.
• Another page goes into more detail about why stretching is useful for people with spasticity - https://hspersunite.org.au/better-understanding-spasticity/. HSP tends to make the muscles in the lower body more spastic, and the common advice for people with HSP is to undertake a series of regular stretching. This article gives some of the background behind this. If you're not sure which stretches help which muscles, or indeed which stretches are best for you speak to a physiotherapist or look at TreatHSP (https://www.treathsp.net/en/clinic/physiotherapy) or the HSP support groups.

I was also pleased to see my own post on sex and disability here! - You can go and read all articles here: https://hspersunite.org.au/news/living-with-hsp/  

On the research front they report that:

• a gene therapy is under development for SPG49, 
• neuroinflammation may be important for people with SPG11,
• new research highlights that verbal fluency, memory and executive function can be affected in people with SPG11,
• there is an overlap in some people with HSP and Multiple Sclerosis
• SPG87 is reported to have been found in both 'pure' and 'complex' forms - 'complex' HSP is most often used to represent there being additional symptoms beyond the usual lower body spasticity.  

There are other stories reported as well - you can read more here: https://hspersunite.org.au/news/research-highlights/

I know its just my perspective, but it always takes me a few minutes to remember that Australia is in the southern hemisphere when I get their Spring update in my inbox in September/October!

2022 Survey Open

I am pleased to announce that my 2022 survey is now open. 

This is my 10th survey. I am analysing changes in mobility aid use for anyone who has completed any of my previous surveys. If you have completed any of my previous surveys I ask that you please take some time to answer these questions.

The questions are available in English, Dutch, Italian, French, Spanish, German and Portuguese. These languages are the same as last year. Click on the relevant link in the table to below to access the questions in that language.

The focus for this years survey is: 

• Mobility
• Pain
• Disclosure
• Life with HSP
• Menstrual health

As previously my analysis of answers will take into account both mobility and wellbeing. I will collect answers until approximately the end of 2022, with results published on Rare Disease Day 2023 - Tuesday 28th February. Also, as before only "name" and "country" are required questions. Answer all others that you want.

If you are using your phone to complete this survey I suggest you rotate your screen to landscape to make answering the questions easier. 

Language	Link to access survey:	Responses 30 Dec
English	https://forms.gle/u3PfSNHUZzGGvBpU6	263
Dutch	https://forms.gle/Ftn53UpFxbN7KWjp6	51
French	https://forms.gle/HKAovdAscgK7Njyd7	61
German	https://forms.gle/o4achYc6ya9QWMkq8	35
Italian	https://forms.gle/JFNGfAnk7UQMGB437	15
Portuguese	https://forms.gle/gy7PBbgBt3KkkVHK8	63
Spanish	https://forms.gle/FANkK3eQp77UdxQ76	42

2nd December update

A brief analysis of the English results (as this is the biggest dataset so far) shows the following points:

• Roughly 90% of people consider themselves to be disabled.
• Most people have or would disclose their HSP to others
• Between a quarter and half of people experience more pain, spasticity, fatigue or worse mental health during their menstrual cycle.
• About three quarters of people get pain from their HSP, with the most common descriptors being cramping pain, aching pain or tiring/exhausting pain. Pain is most intensely felt in the hips, legs, feet or back.

Symptoms update - antidepressants

Just a brief update for today.

In addition to my bladder medication I am now also taking antidepressants. Really this is a combination of things happening at the same time, rather than something specific to do with HSP. Therefore this post goes further outside my HSP experiences than they usually do. I'm grouping each of the factors together in headings:

HSP

In the background of my mind I know that my HSP is progressing. My spasticity gradually increases, and my walking and cycling speeds correspondingly gradually decrease. I know that the time that I will need to use mobility aids is approaching, and I've a journey of acceptance to go through before I get there. At that point my HSP becomes much more visible, and I suspect that societies view of me will change because I'll be using mobility aids.

Relationships

I've vaguely alluded to this in a few posts, but here it is explicitly - another factor is that my wife and I separated in 2020. That has been a bit of an impact recently, and we're now moving into the zone where its time to sort out houses. One of my uncertainties in this decision is how much account should I take of my future HSP in this decision - if I assume that I would be in my next house for 10 years, then I think I'd more than likely be using a wheelchair by that point (but not necessarily full-time).

Work

What with both of the above factors, and supporting my children, there are more things which I perceive to be important outside of work than at work. This has meant that I've been less able to put my full enthusiasm into delivering my responsibilities at work.

Coronavirus

Worth mentioning that there's been a worldwide coronavirus pandemic, an energy crisis and a whole load of other negative other stories in the news. Part of me is thankful that the pandemic lockdowns hit just after we separated as most people were busy looking after themselves rather than asking me what was going on. On the other hand, if there hadn't been a pandemic then perhaps we'd have sorted the house and other things out by now.

How long on antidepressants?

I'm thinking that I'll be taking these antidepressants until after the uncertainty around the house is resolved. I've asked myself if I've noticed any difference with taking these, and the answer is that I'm not sure. The main obvious difference is that since I have started taking them my resting heartrate has dropped significantly, which is a sign that they are doing something!