SP Foundation 2022 Conference

The USA HSP group, the SP Foundation, held their annual conference a couple of weeks after the UK HSP Group. They have put their conference up on their YouTube channel. There are some interesting presentations made. I've not watched all of these in full, but have dropped in and scan watched to get a feel of these.

John Fink gives advice on how to exercise with HSP. https://www.youtube.com/watch?v=lnWNVQoAEoI - he gives approaches how how to dance with HSP to promote exercise, muscle movement and brain training. He includes a summary of treatments for HSP symptoms.

Chelsea Burton talks about symptomatic management of spasticity. https://www.youtube.com/watch?v=ffYBcmzK24A - She covers in detail what happens to muscles with spasticity, both positive and negative. She describes different physical therapy options - starting with stretching, and covering orthotics, ultrasound, FES, strength training and other options. She also gives details of various spasticity medications.

Angie and Jeremy McCord give the meeting some exercises to do! https://www.youtube.com/watch?v=EkB9pztTYyc Some of these use exercise bands.

Darius Ebrahimi-Fakhari talks about lessons learnt in childhood HSP. https://www.youtube.com/watch?v=kcUtKubq9io - he describes their cohort of children with HSP, and talks about approaches for drug discovery, looking to develop in cell testing for drug repurposing. 

Hande Ozindler talks about developing treatments for upper motor neuron diseases. https://www.youtube.com/watch?v=5naax-hv__E - She talks about how her lab are researching treatment options for upper motor neuron degradation. 

Peter Baas gives an overview of HSP, and then talks about options for investigating treatments for SPG4.  https://www.youtube.com/watch?v=5llJfLCf8gs#

There is a panel discussion taking questions from the audience: https://www.youtube.com/watch?v=p7XrluYZwUU. John Fink then answers some more questions! https://www.youtube.com/watch?v=KGyjiCUqjSc

Successful 2022 support group AGM

I'm pleased to report that last weekend the UK HSP Support Group successfully held its AGM. We took the decision to hold this virtually to make it accessible to as many people as possible.

I think that it went very well, and we had lots of different speakers popping in to say a few words, which was really good and stopped people getting bored of hearing the same voice speaking all the time.

You can watch the AGM here: https://www.youtube.com/watch?v=iJkWzmnx9eY

The highlights of the AGM are:

• What is HSP? (01:11)
• Cardiff dissertations (07:40)
• Estelle runs London Marathon (18:27)
• 1000 Mile Challenge report (19:37)
• Potato Pants festival (21:23)
• Raising awareness award (38:42)
• Fundraising award (41:24)
• Summer presentations (48:14)

The discussion after was also good, and the group is going to investigate compiling a list of the different HSP specialists that people see, as this will help people seek out where they can see a specialist at the beginning of their HSP journey or at times later down the line when they start to experience symptom changes more rapidly.

Athletes with HSP in Commonwealth Games

Regular readers will note that I have identified athletes with HSP at the Paralympics. I thought to try and do the same for the Commonwealth Games. The organisation of the Commonwealth Games is different from the Olympics, and the Commonwealth Games website doesnt appear to have a searchable area to find athletes from the current games. So, I've had to resort to Google!

My first port of call was to look at the athletes I identified for the Olympics and see if any were taking part in the Commonwealth Games. Out of the eight athletes, only the UK and Canada have teams in the Commonwealth Games (Ukraine, Spain, Brazil and USA are not in the Commonwealth). Neither Ian Marsden or Austin Smeenk appear to be competing - Canoe/Kayak/Rowing are not in the Commonwealth Games, and Austin is not listed in the Canada team list.

My wider search for athletes with HSP on Google found three. Kieran Jones from Wales competes in both Basketball and Shot-put. You can hear an interview with Kieran here: https://anchor.fm/adventureswithaggie/episodes/AWA-X-Kieran-Jones-Shot-Put-and-Javelin--5x-British-Champion-e18ot3j - He is not shown on the Team Wales basketball team, and his focus on Twitter (https://twitter.com/Kieranjones____) now appears to be on shot put for the Paris 2024 Paralympics.

I already knew about Lily Rice, competing for Team Wales in the swimming (https://hspjourney.blogspot.com/2022/05/lily-rice-commonwealth-games.html) and recent tweets from Lily indicate she is on the path for the Games.

I also found Síomha Nic Bradaigh, competing for team Northern Ireland, also in the swimming. Various information here:

https://www.swimireland.ie/news/team-announcement-2022-commonwealth-games

https://belfastmedia.com/siomha-nic-bradaigh-commonwealth-games

Both Lily and Síomha appear to be swimming backstroke in the S8 category, so they may be competing against each other.

I will keep my eyes out and see if I can find any more athletes with HSP in the Commonwealth Games.

Symptoms update - weight gain

Just a short post for today. In the last few months I have been cycling a lot less than previously. There have been a lot of things going on, and it has been easier to spend my time focussing on those things than going off cycling.

The downside of not cycling is that my weight has increased. Also, according to my sports watch, my cardio fitness has decreased. I am considering if weight gain and decreased cardio fitness is an inevitable part of reduced mobility. 

Changes: The immediate change is to make more time for cycling to decrease my weight and increase my cardio fitness. In the longer term I'm going to need to consider if I need to make some dietary changes to influence my weight with the foresight that my mobility is on a downward trend. And, whilst it is appropriate for me to get back out on my bike more whilst I still can, I'm going to need to think about what exercises I can do in the future when riding my bike becomes more difficult.

Reminder - the benefits I get from cycling are: regular exercise, fresh air and thinking space. In a commuting to work sense I also get cost, time and environmental benefits compared with driving, as well as the avoidance of parking restrictions at/near my office.

Lily Rice - Commonwealth Games

There was some exciting news the other day. Many with HSP will know of Lily Rice, who has been amazing people with her wheelchair back-flips and her medals in WCMX.

Lily is competing for Wales in the Commonwealth Games in the summer, in the swimming.

Regular readers will have spotted that I looked for athletes with HSP for the summer and winter Olympics, and Lily becomes the first athlete for my Commonwealth Games list!

You can see more info here:

https://www.swimwales.org/cy/news/fantastic-four-para-swimmers-selected-for-commonwealth-games

https://www.bbc.co.uk/sport/av/wales/61601015

https://www.instagram.com/p/CeEM-gxIGuc/

https://www.bbc.co.uk/sport/wales/61602780

Good luck Lily!!!!!

Accepting the future

There's quite a bit going on in my life at the moment, with me needing to think about moving house in the not too distant future. This is quite an interesting topic and its making me realise that I have to take my future mobility into account in that decision making process.

Regular readers will know that my legs are a bit stiff, that I get through shoes at a reasonable rate, and that I'm needing to use the bannister to go up and down stairs. Running is something that I cannot do for more than a few paces, and I'm starting to need to help myself up when standing up. I consider that whilst my HSP affects me, it does not do so significantly at this time.

I know that I'm going to end up needing to use a wheelchair in the future, and I've known this for quite a long time. However, I've always put this sufficiently far forward in the future that it hasn't needed to influence decisions I've made. In recent months I've felt like this future isn't really so far in the future, and perhaps I ought to be thinking about that future in a more practical way.

I'm not one for moving house regularly, so I would expect that whichever house I end up living in is likely to need to be able to deal with me using walking sticks and potentially either a walking frame and/or a wheelchair depending on what I need and what I find most useful. I know that stairs are going to become progressively more difficult, so I know that this will have to be a factor in any decision. Equally, I am likely to need to change my bike for another mode of transport, that may be an electric trike of some kind, or a mobility scooter, and I'm going to need space to be able to store this and transfer on/off.

So, I like to plan and I like to know whats coming up. I am hopeful that my daily stretches keep my mobility with me for as long as possible, but I also know that change lies ahead. The uncertainty on my progression is not so good for my mental health. I worry that I might be worrying too much, my progression will stay slowly progressing and am building unnecessary factors into this house moving thought process. Whilst at the same time I worry that my HSP will progress faster than it is at the moment, and I'll move into a house that is suboptimal for me in a only few years time.

There are, of course, a load of other non-HSP factors that I need to take into account - children, working from home, energy efficiency, proximity to school, budget and so on. Readers should not be surprised to know that I've got a spreadsheet to track all this......

Stuff That Works

I've been spotting Stuff That Works pop up on my social media feeds recently and decided to investigate. This website is aiming to crowd source peoples information on treatments, and claims to be able to show greater insights with more data.

I have entered my details into this website. I am one of 174 contributors at the moment, which means that we're at the "most tried treatments" stage. Right now this doesnt seem to show anything particularly surprising, with baclofen and physiotherapy being the two most common treatments shown.

Looking further down the list comes magnesium, stretching, tizanidine, exercise, gabapentin, botox and functional electrical stimulation, but each of these have fewer than 10 reports. The interesting thing is that when you click on each of these treatments most people report that they are insignificant (there are a few exceptions to this). I also note that these treatments are for the spasticity side of things and not so much for other symptoms.

Once 500 contributors is reached there becomes another level of analysis shown, which should show the effectiveness of the treatments. With more contributors they claim to be able to influence the direction of the research, which I am interested to find out more about. 

If you wish to go in and add details for yourself you can do so here: https://www.stuffthatworks.health/hereditary-spastic-paraplegia  

There is an interesting map showing contributors, but it is not clear how the map is formed. The main block for HSP overlaps at the edge of Multiple Sclerosis, although my indicator is halfway between MS and Parkinson's. Other nearby conditions include ALS, stiff person syndrome and cerebellar ataxia. If you zoom out other large conditions fairly near include restless leg syndrome and peripheral neuropathy. I quite enjoy looking around the map!

I think the drawback to this system is that it asks you to identify one treatment (or combination of treatments) which improves your condition, which means that you have to think about how to answer this! It is interesting to note that "no significant change" is shown in a neutral face when HSP is a degenerative condition, and no significant change may be an important result. At least you can go back in and edit your answers as often as you like!

Whilst they claim to be the first crowd sourced site, I note that there are already other two other similar long established sites:

Rare Connect https://www.rareconnect.org/en/community/hereditary-spastic-paraplegia with 1154 members

Patients Like Me https://www.patientslikeme.com/conditions/hereditary-spastic-paraplegia with 233 members

Symptoms Update - Fatigue and Bladder

 A few things I've been spotting over recent weeks.

Fatigue

I am finding that I've been feeling tired more frequently. I noted this back in November (https://hspjourney.blogspot.com/2021/11/new-shoes-symptoms-update.html). Since then I've generally maintained the reduced caffeine, and I've been trying to stick with the more consistent bedtime. I'm finding screen time a challenge, and need more will-power to avoid this! It is also worth noting that I'm finding that at work the pressures of the projects I'm working on have felt high for a few months, and I know this isn't helping. Furthermore, I also realise that I'm not keeping myself as active as I have been previously. There doesn't seem to be an easy solution here, and I'm still not sure how much of my fatigue is HSP, and how much is me being busy all the time.

Bladder

Back in November I noted an alternative bladder medication, the switch from neditol (tolterodine) to oxybutynin. Overall both medications work, and I find that I am not desperately needing to go all the time. The messaging is different though. With the neditol I found that once I needed to go I needed to go to the toilet quickly, whereas with oxybutynin the need to go message builds up over a longer time, giving me more time to plan. I dont think that I am going to the toilet any more or fewer times per day, but I think it has have improved my need to go during the night, with there being less urgency there.

One other observation, and this is something which I've noted before the medication change-over. I think that flow pressure of my urine is decreasing - sometimes the flow out is just a dribble, and at other times the flow is steady, but less 'powerful' than it was in the past. This may, of course, be something to do with getting older rather than a result of HSP.

Spasticity

I think I'm spotting that moving is becoming a bit more difficult. I'm having to make more use of my upper body, especially when standing up, and I think that my speed and distance are both continuing to decrease slowly. I think that the main change is that when I'm chatting with people about my future, I'd placed my eventual need for a wheelchair to be sufficiently far in the future to be out of consequence. I had generally put the wheelchair at '5 to 10' years in the future, whereas now I feel that this timeframe is a bit too optimistic. I need to accept that this future me is getting closer, and I perhaps ought to be describing this as '5 or so' years away. I'll see how this feels and report back! I also know that the increasing need for wheelchair goes in parallel with increased need for changes to the way I cycle.

A quick and easy guide to sex and disability

When I was reading the various comments made by people who responded to my survey, a few people were clear that they were having less sex than they used to. One person indicated that they miss sex quite a lot, and another reported having hardly any since their diagnosis. A comment was made by one that their partner no longer desires them. This got me thinking a bit more, and I'm minded to ask more questions about sex and relationships in a future survey.

I also note that the topic of sex and disability doesn't feature much in the media, and so I bought a copy of the book:  A Quick and Easy Guide to Sex and Disability, by A Andrews. I wanted to understand more, and in the usual way, share this understanding with you readers!

The book is a cartoon book, and in the first few pages you find out that the author is a paraplegic cartoonist, that has sex! They write the book with the aim of ditching the question "how can disabled bodies have sex" and replace it with "how can disabled folk have more enjoyable sex". They note that society does not really discuss sex and disability, and present three myths: 'disabled people aren't attractive', 'disabled people don't care about things like sex', and 'disabled people cant perform sex.' - identifying that the things people say can impact on wellbeing.

They start by covering communication - firstly communicating with yourself, and then goes on to give ideas about how you may want to try communicating, noting that this can be hard to do. The remainder of the book goes into some of the more practical points which can occur when having sex with a disability. The conclusion is that sex is whatever you want it to be, and the possibilities are endless.

I like the information presented here, and it is presented in a very friendly, matter-of-fact way, allowing you to see many perspectives and help you form your own view. The cartoons in the book present a wide range of disabilities, and also a wide range of people - capturing the diversity of the human race in a very nice way!

Sticky McStickStick

I have got a few new books recently with a view to improve my knowledge of various aspects. One of these was Sticky McStickStick by Micheal Rosen.

Michael was admitted to hospital with coronavirus, and had to learn to walk again. I got this book to see if it might be a useful way to help younger children learn about using mobility aids, which are a frequent companion for many with HSP.

Michaels journey is in the opposite direction to those with HSP. My key takeaway points are that at the beginning of his recovery they tried various different mobility aids, and he found the wheelchair to be quite enjoyable.  Michael journeys through using wheelchairs, walking frames and finally to a walking stick in his recovery, and the transition helps him, and I like the overall point about changing to a more suitable aid as the situation changes. 

The story also describes the different work that physiotherapists do to help people, and shows the range of exercises that they can suggest to help. Micheal names his walking stick Sticky McStickStick, which he describes as developing an attachment to. One aspect is that Michael spends a while using the stick, and there are various considerations made to make sure that he is safe and able to go about his life safely whilst using the stick. At the end of the story Sticky McStickStick isnt used any more, and sits about his house as a reminder how it helped him at the time. This is an interesting parallel for many with HSP, where many people have mobility aids and other equipment at home which they no longer use.

Overall the story touches on many important issues for those with HSP:

• Mobility aids can help increase the number of things you can do and the distance you can go,
• It is important to transition from one aid to another as things change,
• People can worry about transitioning from one aid to another, 
• It is important to make sure that it is safe and appropriate to use new aids at home
• People can develop attachment to aids, which can further make transition between aids difficult
• Advice from professionals can help you determine if your aids are appropriate and guide you in activities to help 

I'm pleased to see a range of mobility aids pictured in a book in a positive friendly way.