Review of 2019

Another year has passed, and again I take the opportunity to reflect on the years activities.

Knowledge

The latter part of the year has seen quite a bit of new research published. I started the year with an overview of published research from 2018 and there have been several posts on other investigations throughout the year. I also gained lots of knowledge from my involvement with EuroHSP and by attending the Spatax meeting. This year there seems to have been more papers which are starting to look at some of the wider context that those with HSP find themselves in.

Symptoms

This has been another year without much change. I haven't had any HSP appointments this year. I've another new shoes post to write in the new year. Through the year I have noted subtle changes in my bladder, sleep, using stairs and driving. I think that I am a bit slower and am finding things a bit more difficult, however I have also walked up Scafell Pike, ridden 100km on a bike and stood up throughout a gig, so I must remember that this progress is not all negative, there is still plenty which I can do! Overall, it has been a busy year with lots going on for me outside the HSP world.  

This Blog

I'm really pleased with the continuing growth in readership of this blog. My audience remains broadly the same (predominantly UK, USA, Canada, Australia, and then European countries) although this year I have picked up Brazil and India. The most popular posts continue to be the questions for and results of my survey, as well as my general posts on research and particular HSP symptoms. 

I'm really pleased that blog posts continue to be linked to and used within other HSP and RareDisease publications. Thank you to all my readers, including those that give comments appreciating what I have to say. 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. 

Survey

The now annual pattern of my surveys is well established. My 2019 survey has a similar number of responses to my 2018 and 2017 surveys. Following collaboration with the Brazilian HSP group last year, my new feature this year has been to offer the survey up in several languages so people can answer the questions in their own language. This is yielding more results than last years translation sheets, which is good. Some languages are more popular than others, which seems to be driven by the support groups in those countries letting their members know about the survey. If you're reading this and want to take part in this survey, then (providing it is still 2019) please do! Results will be out on 29th Feb after the majority of analysis during January.

Community Contribution

HSP Community activities for 2019 included:

• Expanding my survey into more languages
• Taking part in a Disability & Inclusion panel discussion at work
• Attending the SPATAX meeting and a EuroHSP meeting
• 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.
• Starting next year, working more with PARCC

The main element of my community contribution comes from being chair of the HSP group. There are still 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.

Recent News Stories

There have been several things which have caught my eye in recent weeks - here is an overview:

Strength Training for Depression.

This story caught my attention as depression is a common symptom with HSP. It is well researched that exercise helps with depression, but most studies have tested aerobic exercise. This study tested if strength training would have a similar benefit. The study looked at older people who had been diagnosed with a major depressive disorder, and showed that strength training does have benefits. I wonder if strength training might be more appropriate than aerobic training for some with HSP.   https://www.karger.com/Article/Abstract/503750

More Challenge Needed!

An Italian paper reports examining quality of life against daily activities for 35 people with HSP. The daily life of many with HSP presents a low challenge. People who work (i.e. undertaking productive tasks) are more active than those who dont, but are less happy, however when the work is seen to be relevant or complex the experience is better. Persons with motor disabilities would benefit from more challenging opportunities for action in daily life, in order to attain well-being through active skill mobilisation. https://hspersunite.org.au/more-challenge-needed-in-daily-life/

Cannabis Based Medicinal Products Guidelines

NICE in the UK have published their guidelines for cannabis based medicinal products. A lack of evidence that such products give benefits for spasticity mean that it can only be prescribed as part of a research trial. I will write a full blog post about this next year: https://www.nice.org.uk/guidance/ng144

Development of Artificial Nerves and Neurons

This story reports the development of artificial neurons which replicate the functions of real neurons, effectively offering an alternative pathway for nerve signals to use. The article mentions that there is potential for this to be used in diseases with neuronal degeneration, they give the example Alzheimer's, and I wonder if there is potential for this with HSP.  https://neurosciencenews.com/artificial-neurons-15279/

This story is similar, and reports an artificial nerve. Tests on this nerve showed that they were able to activate muscles in a cockroach. Similarly, I wonder of the scope for using this type of approach to regain function in muscles affected by HSP.  https://www.sciencemag.org/news/2018/05/new-artificial-nerves-could-transform-prosthetics

Rehabilitation Perspectives

This is an interesting paper - it presents the contrast between different perspectives when looking at rehabilitation. From a medical perspective a disability is a malfunctioning body part, and therefore there is a biological problem to be fixed. On the other hand, social models of disability put forward that this is a construct of unequal practices and access, and is a social oppression.  There is an interesting discussion around how impairments are often seen as inferior, which puts a negative perspective on disability. It suggests that both perspectives together could be a useful basis for setting out a rehabilitation programme. https://www.jhrehab.org/2019/11/14/making-the-case-for-critical-disability-studies-with-rehabilitation-sciences/

Link between cholesterol processing and HSP.

This recent report identifies that many of the genes affected by HSP are involved in the processing of cholesterol and/or other fats in cells - lipids. There is an imbalance in lipids in both HSP and MND, and further work is required to determine if the imbalance is the cause of HSP or a consequence of HSP. I can see that this has potential to be an important peice of work. 2 links given, one to the news:  https://www.bbc.co.uk/news/health-50821327 and another to the full article:
https://academic.oup.com/brain/advance-article/doi/10.1093/brain/awz382/5679762?searchresult=1

Wheelchair Skills Training

I liked the look of this wheelchair skills training: https://www.freedomwheelchairskills.co.uk/