Tuesday, 23 June 2020

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!

Tuesday, 9 June 2020

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 - 

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:

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.

Monday, 1 June 2020

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)

Thursday, 21 May 2020

New shoes

Once again I've got through another pair of shoes, so time to log the details here!

Actually, I've got through two pairs - my usual shoes and my biking shoes.

Usual shoes - this time I went for Hi-Tec Quadra Classic (low rise hiking boots). I got these at the end of November 2018, so they have done 18 months (1 year 6 months) before being too holey to wear.


The wear follows the same pattern - my left shoe is much more worn that my right shoe. The Hi-Tec's have performed better than my previous sketchers as the sole has remained attached to the shoe rather than detaching like the Sketchers had.

This time I considered getting another pair of Hi-Tec as they have worn much better than the Sketchers, but given that the time they lasted is about the same I have gone instead with Karrimor Supa 5 shoes.

The Hi-Tecs have lasted about as long as the previous Sketchers, suggesting that I'm on about 18 months per pair at the moment. Regular readers may note my preference to leave my shoes a little too long before replacing them!

You can see previous new shoe comparisons (with photos of old shoes) here:
https://hspjourney.blogspot.com/2018/12/more-new-shoes.html
https://hspjourney.blogspot.com/2017/05/shoe-wear-update-data.html

On the bike shoes front, I got my first pair of bike shoes with cleats in Sept 2017, and they lasted until December 2019:

Basically the soles were starting to detach, similarly on each foot. Equal wear isnt too surprising as these only get worn for walking for short periods. Those shoes lasted 2 years and 3 months. The current cycling shoes (also with cleats) are Shimano MT300.


Thursday, 14 May 2020

An introduction to HSP

I needed a summary of HSP, and I was hunting around various support groups and social media pages, but none quite said what I wanted to get across, so I borrowed heavily from many sources and wrote my own:

What is Hereditary Spastic Paraplegia?

Hereditary Spastic Paraplegia (HSP) is a diagnosis which covers a large group of rare genetic disorders. HSP is a neurological condition, principally affecting the bodies longer muscle control nerves. There are upwards of 80 types of HSP which are generally inherited, with different types having different inheritance patterns. People with HSP can show their first symptoms at any age, although the most common ages of onset are in childhood (up to age 5) or in adulthood (age 35-50). HSP symptoms progress slowly over many years, and additional symptoms can develop as the condition progresses. HSP has several names, and is also known as Hereditary Spastic Paraparesis, Familial Spastic Paraplegia (FSP), Strümpell-Lorrain Syndrome, and some types of HSP have their own names.

Symptoms

The main symptoms are stiffness, spasticity or weakness in the lower limbs, leading to difficulties walking. There is large variation in both the speed of progression of HSP and in its severity (the amount HSP affects you). Some people may end up with mild difficulties walking. Others may be unable to walk and require a wheelchair most of the time. Those with HSP which significantly affects their mobility are likely to need different mobility aids as their symptoms change over time, and they may need various modifications at home or at work.

In addition to mobility issues many are affected by bladder problems, pain, and fatigue, and some will get numbness, tingling or other sensations. The effects of a person’s symptoms may vary from day to day more than their year-on-year progression. Life expectancy does not change for the majority.

Certain types of HSP are regarded as complex (affecting around 10% of people with HSP) and further additional symptoms may include problems with balance or coordination, learning/memory issues, problems with speech, vision, hearing or swallowing, problems with the skin, dementia, epilepsy or nerve damage. 

Diagnosis

As HSP is a rare disease (affecting around 3 in 100,000) it is not known by many medical professionals. The early symptoms of HSP are similar to other more common conditions and getting a diagnosis can be a long uncertain journey, potentially with several misdiagnoses along the way. Genetic testing is available for some types of HSP. Because there is such a wide range of potential symptoms, the speed of progression and ultimate severity are difficult to estimate for an individual.

Wellbeing 

It is difficult for people with HSP to get a clear picture how HSP will affect them over time, and plan for changes they may need to make, which can lead to feeling very uncertain about the future. Some with HSP are depressed, feel lonely, feel isolated or feel vulnerable. It is important for people with HSP to have a purpose in life and to take part in activities which they enjoy as these can help improve their wellbeing. It may be necessary to find new hobbies or interests as mobility changes over time. 

Treatment

At the moment there are no cures for HSP. Whilst there are many research teams looking into HSP around the world, all the current treatment is around symptom management. Someone with HSP may have a series of stretches and other exercises to do, they may take medication for spasticity, pain, their bladder and depression. Work on HSP is being done in many areas; to better understand how HSP works, to provide tools which can help assess HSP and its progression, to investigate new medicinal or other treatments, to explore genetic modification, and ultimately to identify if a cure can be found.

Support

There are various HSP support communities around the world which many with HSP enjoy participating with. The ability to share stories and experiences with others helps reduce feelings of isolation, and some groups also provide support in other ways. There are several support groups in Europe, mostly under the umbrella EuroHSP. There are other groups in the USA, Australia and Brazil. On-line communities include RareConnect, and there are numerous FaceBook groups and other social media groups.

Sunday, 3 May 2020

Diagnostic Gap

There have been a number of papers in recent times which talk about a diagnostic gap in HSP. I wanted to explore what this means.

Many of you will know that the first identification of a gene for HSP happened in 1998. This was on the L1CAM gene, and was given the name SPG1. Since then more genes have been associated with HSP, and these have been numbered in order of discovery with sequential numbers, with the highest number (so far) being SPG82 identified in 2019 (https://www.omim.org/entry/618770#1). However, there is also a set of genes being identified which are associated with HSP, but which are not being given SPG numbers (for example, RNF170: https://www.genomemed.org/news/2019/10/28/new-gene-for-hereditary-spastic-paraplegia). The picture gets more complicated because there is genetic overlap between HSP (when considered as a whole) and Ataxia, ALS, Alzheimer's and Parkinson's.

An interesting question is: how many different HSP genes might there be? Different researchers have different ideas. Evan Reid suggested in 2014 that there may be 100-200 genes in total (https://hspjourney.blogspot.com/2014/08/agm-hsp-research-historical-perspective.html).

The paper: Perspectives on the Genomics of HSP Beyond Mendelian Inheritance by Dana M. Bis-Brewer and Stephan Züchner, (https://www.frontiersin.org/articles/10.3389/fneur.2018.00958/full) published in 2018 describes several key features. The text below is summary extracts of the paper (I basically copied the whole paper in and retained the parts I wanted to!):

It is usually assumed that eventually nearly all HSP patients will receive a single-gene diagnosis and the diagnostic yield of multigene panels has never been higher (albeit hindered by an increasing burden of Variants of Uncertain Significance).

However, most recently discovered HSP genes are rare causes of the disease affecting few people, and recent multi-patient gene testing studies have had less than 50% success rate in identifying an HSP gene. There is concern of a persisting diagnostic gap, estimated at 30–40%, and even higher for sporadic cases. Gene therapies may soon come to HSP, but these require a specific genetic diagnosis, which emphasises the need to fill the diagnostic gap, illustrated below: 






This gap may not be fully closed by classic Mendelian approaches. There are a number of strategies for identifying further Mendelian and non-Mendelian causes of HSP:


  • Systematic reanalysis of unresolved cases can reveal new causative variants. The search should ideally be expanded beyond mutations in the protein-coding regions, but this requires whole-genome sequencing; 
  • Another contributor of genome variability that could help resolve the diagnostic gap is structural variation, including copy number variations (CNVs), translocations, and inversions. CNVs are known to play an important role in HSPs, however determining whether a CNV is benign or pathogenic remains a considerable clinical challenge.
  • Standard clinical genomic analysis focuses on typical modes of inheritance and unusual inheritance modes are often ignored. Inclusion of these can lead to successful identification of overlooked molecular diagnoses.
  • Traditional Mendelian disease analysis are focused on specific alleles. These locus-specific studies disregard a more comprehensive genetic model for human disease in which variants of varying effect size as well as environmental influences contribute to disease.
  • Contrary to general expectations for HSP families, asymptomatic carriers are not infrequent.
  • Sex-dependent penetrance is suspected in some HSPs based on the excess of affected males.
  • Given the high clinical variability observed across HSP patients, genetic modification of the primary allele has been anticipated.
  • Exceptions to the fundamental “one gene, one phenotype” may occur. Here the primary allele is sufficient to cause disease, but a secondary allele is a “modifier” that controls aspects like disease severity or progression.
  • The current classification systems suggest that HSPs are a distinct and isolated disorder, when in fact HSPs exist on a spectrum between inherited ataxias and Charcot-Marie-Tooth disease.
  • These approaches increasingly require larger datasets which contradicts, of course, the low prevalence of rare disease. Raw genetic data aggregation may be the next frontier for HSP gene discovery.
  • Gene therapy may soon be applied to specific HSP genes. These novel therapeutic approaches include gene replacement, antisense oligonucleotides (ASO), and soon gene editing. Most require a specific genetic diagnosis. 

Friday, 24 April 2020

Updated advice from Association of British Neurologists

OK, So I didnt expect Coronavirus when I was thinking about what I might be blogging about so much in 2020, but I guess with hindsight it was obvious.

In other news - the UK HSP Support Group successfully held its first virtual trustee meeting at the weekend. Coronavirus has also caused me to take my focus away from publicising the results of my 2019 survey (https://hspjourney.blogspot.com/2020/02/2019-survey-results.html)

The latest advice from the Association of British Neurologists can be found on their website: https://www.theabn.org/page/COVID-19 - clicking the patient advice link doesnt yet have anything on HSP, and readers are referred to the doctor/carer advice: https://cdn.ymaws.com/www.theabn.org/resource/collection/65C334C7-30FA-45DB-93AA-74B3A3A20293/ABN_Neurology_COVID-19_Guidance_v6_9.4.20_FP.pdf

I have summarised what I consider to be the key points from this advice below, and have discussed this briefly with two UK HSP specialists. My own views/comments are italicised, and text from the ABN guidance is not italicised. Because HSP is such a broad set of conditions it is difficult to draw conclusions which apply to everyone with HSP.

Conclusions

This is where I try to draw together the advice below into something short:
  • Mild HSP by itself does not put you in a vulnerable group or a high risk group
  • HSP is judged to have a Low or Moderate risk associated with Covid-19
  • Risk is increased with age, weight, frailty and disability
  • Risk is increased with symptoms (HSP or other) affecting lungs, heart, kidneys etc
  • Social distancing is recommended
Those with significant HSP are more likely to be considered moderate risk. Those with complex HSP where symptoms include issues swallowing (due to risk of aspiration pneumonia) are more likely to be considered moderate risk. Those with mild HSP (complex or non-complex) are likely to remain as low risk. 

Significance can take into account loss of mobility. Use of one walking stick is unlikely to be significant. Very restricted mobility (e.g. predominant use of wheelchair or in some cases use of two walking sticks) is likely to be significant as this can lead to increased risk on heart/lung function from being sedentary

Reminder: Non-complex HSP tends to affect only legs, bladder and/or back, often with fatigue and/or pain - this includes SPG4, SPG3A, SPG5 and SPG31. Complex or complicated HSP may also include symptoms affecting balance/co-ordination, learning/memory, swallowing, speech, vision, hearing. This includes SPG11, SPG15, SPG7 and SPG35. Note that some types of HSP can be either complex or non-complex for some people.

Guidance Introduction

  • Although some neurological conditions or treatments increase the risk of complicated COVID-19, most patients in these groups will overcome the infection.
  • COVID-19 is a disease caused by a new coronavirus that affects the lungs and airways. It is a new virus, so nobody has immunity to it. The entire population is therefore at risk of catching it.
  • It is estimated that as many as 80% of people who catch the virus may experience relatively mild or no symptoms but are able to pass on the infection to others.
  • People aged over 70, with long-term conditions or a weakened immune system are at risk of developing complications of the infection.
  • Social distancing reduces the risk of catching COVID-19, but self-isolation is the most effective means of avoiding infection.

Risk Assessment - highest risk group

Six clinical high risk groups have been identified by NHS England: 
  1. Solid organ transplant recipients 
  2. People with specific cancers and people who have had bone marrow or stem cell transplants in the last 6 months, or who are still taking immunosuppressive drugs 
  3. People with severe respiratory conditions including all cystic fibrosis, severe asthma and severe COPD.  
  4. People with rare diseases and inborn errors of metabolism that significantly increase the risk of infections (such as SCID, homozygous sickle cell). 
  5. People on immunosuppressive therapies sufficient to significantly increase risk of infection.
  6. Pregnant women with significant heart disease, congenital or acquired 
Readers will note that HSP by itself does not have severe respiratory symptoms or give rise to inborn errors of metabolism. Therefore, HSP does not feature in this highest risk group list.

Risk Assessment - vulnerable people

People who are not in this highest risk group are vulnerable if they are:
  • aged 70 or older (regardless of medical conditions) 
  • aged under 70 and with: chronic respiratory diseases, chronic heart disease, chronic kidney disease or chronic liver disease
  • aged under 70 and with chronic neurological conditions, such as Parkinson’s disease 
  • aged under 70 and with problems with your spleen
  • aged under 70 and with a weakened immune system as the result of conditions such as HIV and AIDS, or medicines such as steroid tablets or chemotherapy 
  • aged under 70 and being seriously overweight (a BMI of 40 or above) 
  • aged under 70 and who are pregnant
This advice is slightly different from that in earlier versions. Motor Neuron disease was on previous lists and it is noted as being incorrectly placed on the list. Seriously overweight has been added to the list. HSP is a less severe type of motor neuron disease, indicating that HSP by itself does not automatically put you in the vulnerable category. In this list chronic is taken to be long-term.

Patients with conditions that do not affect their swallowing or breathing muscles and in whom the immune system is working normally are not considered to be at increased risk from COVID-19. 

Milder or moderate forms of many of the commoner neurological disorders, such as Parkinson’s disease, multiple sclerosis, epilepsy are not currently considered to confer increased risk, so long as breathing and swallowing muscles are functioning well.

Most of the conditions or treatments that increase susceptibility to COVID-19 suppress the immune system. Additionally, since COVID-19 is a disease of the lungs and airways, any condition that has affected swallowing or breathing might increase the severity of COVID-19 infection.

There is a multi-stage process currently being undertaken in the UK to identify people in the high risk grouping which involves looking at hospital and GP records, both with automatic trawls and hospital specialist/GP manual input.    

A list of medication which suppresses the immune system is given in the document. The medication usually prescribed for HSP (spasticity, bladder, pain, depression) do not feature in this table.


Risk Assessment - specific for neurological conditions

The risk for neurological conditions has been subdivided into three levels; low, moderate, and high.
  • HSP is identified as having a Moderate or Low risk. 

HSP by itself does not render a patient susceptible to infection, however disability or the presence of co-morbidities increase the risk from COVID-19.

Most patients in the high-risk groups are not expected to suffer severe complications of the virus.

Social distancing is recommended for all people with any neurological condition, their carers and family. Shielding (self-isolation) is only recommended for people in the high risk category.

People with a neurological condition with low or moderate risk might be considered high risk if they have additional risk associated with other conditions affecting the lungs, heart, kidneys etc. 

Frailty is a risk factor for a poor response to treatment for COVID-19. People with neurological conditions resulting in frailty, or who are frail as a result of age or other conditions are at increased risk from COVID-19. 

Risk Assessment - comparison with other neurological conditions

Looking at how other conditions similar to HSP are rated:
  • Multiple Sclerosis is rated moderate or high
  • X-linked Muscular Dystrophy is rated high
  • Motor Neuron Disease is rated high
  • Cerebral Palsy is rated low
  • Movement disorders e.g. Parkinsons are rated low, moderate or high 
  • Ataxia is rated low, moderate or high


Wednesday, 8 April 2020

Lockdown exercise advice from HSPRF

The Australian group HSPRF have put this up on Facebook this morning, with some useful advice on exercises you could do indoors.
https://www.facebook.com/HSPResearchFoundation/posts/3158022867565219

EXERCISING WITH SOCIAL RESTRICTIONS

Many people with HSP have routines of regular exercise and movement, such as at the gym or at the physio, the pool or the park. If the current restrictions on moving around, gatherings of people, social distancing or shutdowns have disrupted your regular exercise routine, all is not lost, although it may take some imagination and creativity to establish a good alternative or two.
Whilst it can be difficult to reproduce some exercises that employ weights or equipment, using one’s own body weight and the forces of nature, namely gravity, can be employed to good effect to get a workout.

Push-Ups

Push-ups can be done in a whole range of ways:
  1. from a standing push-up (or push off) leaning against a wall, to a
  2. more demanding push-up using a railing, table or bench for example, to
  3. push-ups on the floor keeping the belly or knees or just the feet on the floor depending on your capability https://www.youtube.com/watch?v=Pkj8LLRsoDw to
  4. even more demanding yet, alternately raising one arm and the diagonally opposite leg in the “up” position of the push-up https://www.youtube.com/watch?v=w2vtX1mD0TA. This improves core strength and stability. Here is an easier version that also works on the core https://www.youtube.com/watch?v=uofd3HJWrWg.

Sitting and Standing

Sitting on a firm chair or bench that is high enough to have your knees and hips at roughly the same height, bring your arms up in front of you and slowly come up to a standing position, avoiding using your hands or arms to push off or to help with momentum if possible. In a slow and controlled manner, return to the sitting position. This return movement of controlled sitting from standing is at least as important in getting benefit from the exercise as the initial movement of standing from sitting. 3 sets of 10 repetitions per set is good, however just do what you can to start with while maintaining posture and control – then build up over time. This movement is equivalent to a 1/2 squat. YouTube video https://www.youtube.com/watch?v=5yxfzyzEzBY
Do not try this from a slumpy couch. If you are unable to stand from sitting on a firm chair, you could try the same movement from a stool or other form of higher seat. Or you could do a partial squat from standing, with good control and slow movement on both the way down and the way back to fully upright. This might start as a 1/8 squat and progress to a 1/4 squat as you improve. YouTube video https://www.youtube.com/watch?v=01pCqfW9v4c

Duration stretching

Duration stretching can provide enormous benefit to mobility and quality of life. Six high load daily exercises of 15 minutes cumulative duration each make up the program. They can be done in front of TV, so if you watch 90 minutes of TV a day, or there is a movie you have been wanting to see, now you can kill two birds with one stone. Here is the link to the duration stretching program: https://hspersunite.org.au/daily-duration-stretching-pays-…/

The Shower or Bath ‘Dance'

Either standing (Shower Dance) or sitting on the floor (Bath Dance) the idea is to do the exact same movements (dry and clothed in your living room) that you would do washing yourself in the shower or bath and drying yourself afterwards… You can even use the towel! We achieve a good range of motion and more exercise than you might think when we shower and bathe. You can do it to music, hum along or entertain yourself with your own singing. Just do what you would normally do and are capable of when actually showering or bathing.

Using exercise equipment

Many people have some exercise equipment at home – exercise/gym balls, maybe some hand weights (those packets of rice or flour or cans of fruit can double as weights) an exercise bike or treadmill, or elastic resistance bands.

Outdoor exercise

For many people, it is still okay to leave your home to get some exercise. Where possible, it is important to take advantage of this, not just to exercise, but to boost spirits and help maintain a positive outlook.

Tuesday, 31 March 2020

A positive from coronavirus

I have just put a post on LinkedIn - a positive from coronavirus - new flexible working practices may help those with disabilities back into the workplace - https://www.linkedin.com/pulse/positive-from-coronavirus-adam-lawrence/

If you're not on LinkedIn, the post is:

I have observed in recent weeks the rapid increase in the proportion of people continuing to work in their usual jobs, but at home. There are many types of people who, during the coronavirus crisis, are being obliged by the government to work at home and avoid unnecessary travel, and employers are doing all they can to make this happen. Many of these people are in roles that would not normally have been considered for home working. Boundaries are being pushed, employees are being trusted to work in new ways and new locations. Technology is being exploited in innovative ways to enable this impressive change.

Look at the perspective a few months before this crisis. There are a fair number of people with both disabilities and important skills that are in demand in the workplace. They have been unable to get work because there were perceptions and attitudes that the role could not successfully be fulfilled in a different way or in a different location.

I think attitudes around working at home are changing. Once the COVID-19 crisis has passed and people head back towards a more normal society, will employers remember this perspective change? There are many roles which can be successfully filled by people who do not come into the workplace, who use technology, and who manage their own time in their own places to effectively deliver work.

Having a disability can be an isolating experience. When people are unable to find purpose in their lives or contribute to society, this can negatively impact their wellbeing and quality of life. Employment is one way of getting a purpose and making contributions to society.

I hope that people with useful skills, currently unable to work because of their disability or situation, embrace the perspective change and have the confidence to seek reemployment. Such candidates could ask potential employers: “was this role successfully undertaken by a home-worker during the COVID-19 crisis?” More things are possible. More can be achieved. There will be many opportunities as society fully engages with this new more inclusive, more effective world.

Saturday, 21 March 2020

HSP and Coronavirus / COVID-19

Mon 23rd March

Update from the Association of British Neurologists - Version 2, 22nd March:

"Patients with conditions that do not affect their swallowing or breathing muscles and in whom the immune system is working normally are not considered to be at increased risk from COVID-19."

"Experts ..... have attempted to estimate the risk associated with COVID-19 from each neurological condition......  It is difficult to accurately assess risk for every condition. The risk has been subdivided into three levels; low, moderate, and high."

"We recommend social distancing for all people with any neurological condition, their carers and family. We recommend self-isolation only for people in the high risk category."

"People with a neurological condition with low or moderate risk might be considered high risk if they have additional risk associated with other conditions affecting the lungs, heart, kidneys etc."

Table G in the advice puts HSP in the Low/Medium risk category.

View the full advice here: https://www.theabn.org/news/495261/ABN-Guidance-on-COVID-19-for-people-with-neurological-conditions.htm

Sat 21st March

OK, there's no getting away from it, Covid-19 is a big issue. Some readers will know that I am also chair of the UK HSP Support Group. I wrote to our members this week to advise them that we have postponed or cancelled group meetings for the next few months. We made this decision based on two factors: the UK Government advice around social distancing and minimising non-essential travel, and that those with underlying health conditions are at a higher risk of developing severe problems if they become infected.

I have spotted several people asking questions about HSP and Covid-19, so this post sets out the various information I have found and puts my perspective on it. Readers must remember that I am NOT a medical professional.

The UK advice is here: https://www.gov.uk/government/publications/covid-19-guidance-on-social-distancing-and-for-vulnerable-people/guidance-on-social-distancing-for-everyone-in-the-uk-and-protecting-older-people-and-vulnerable-adults

Those in the higher risk groups (relevant to HSP) are:
  • Those aged 70 or older (regardless of medical conditions)
  • Those under 70 with an underlying health condition (ie anyone instructed to get a flu jab as an adult each year on medical grounds)
  • Those with chronic neurological conditions, such as Parkinson’s disease, motor neurone disease, multiple sclerosis (MS), a learning disability or cerebral palsy

HSP is a neurological condition. What is not said is how you would determine if your HSP is chronic or not. It is my view that if you are able to carry on your life like any other member of society then your HSP is not chronic. If you spend most of your time in a wheelchair then your HSP is chronic. There is a scale between these two extremes, and whilst it is (to some degree) a choice if you consider your HSP to be chronic, my view is:
  • Constant/near-constant use of frames/chairs - more likely to be chronic
  • Frequently affected by pain from HSP - more likely to be chronic
  • Frequently affected by fatigue from HSP - more likely to be chronic
  • HSP affecting cognition/speech/swallowing (etc.) - more likely to be chronic
  • Feet/legs swelling up or sores/skin issues from HSP - more likely to be chronic
  • Use of catheter - more likely to be chronic
  • Significantly affected by stress/depression - more likely to be chronic
  • Regular falls/significant balance problems - more likely to be chronic

You should note that this is just my view. If you have just one of these then you may not consider your HSP to be chronic, but if you have more than one then I think it probably is. It was quite a challenge to decide which of the HSP symptoms to include in this list.

The risk of severe problems if infected increases when you have multiple conditions. Covid-19 is a respiratory virus, so if you live with HSP and Asthma or another respiratory condition then you are more likely to be in the higher risk group. Cardiovascular disease was shown in China data to be the underlying condition resulting in the most deaths (link below). Guidance from NHS indicates that factors which make you more likely to suffer from cardiovascular problems are; inactivity, being overweight, high blood pressure and high cholesterol levels (https://www.nhs.uk/conditions/cardiovascular-disease/). Unfortunately loss of mobility with HSP tends to result in higher levels of inactivity, which increases the risk of cardiovascular issues.

Given all these factors it is my opinion that those with HSP should start by considering themselves at higher risk of having severe problems if they are infected with Covid-19. People at the beginning of their HSP journey or who are not affected significantly may be able to justify that their risk is the same as the rest of the population.  

People should follow their governments advice about social distancing, minimising non-essential travel, working at home, staying indoors, avoiding big groups. Those who:
  • are affected by HSP severely, or 
  • have HSP and at least one other health condition, or
  • are over 70 and have HSP irrespective of severity
should seriously consider if they need to self-isolate for a while.

If you are not sure about this, then it might be a good time to book a call in with your GP/Doctor/Neurologist/HSP specialist to identify the best course of action. This link gives the UK advice on self-isolation: https://www.nhs.uk/conditions/coronavirus-covid-19/self-isolation-advice/.

I realise that people with HSP are often affected by depression, low mood, loneliness, isolation (etc.) It is important to keep in touch with people during this time, so I encourage people to communicate with friends and family. Use whatever method seems right, e-mail/social media/letter/phone or perhaps something more unusual. Avoid face-to-face contact.

Also, whether you are self-isolating or social distancing don't forget to follow your stretching routines and keep as active as possible. 

If you are not a member of an HSP support group then this might be a useful thing to consider. There are 'real' groups in Europe (http://www.eurohsp.eu/), USA (https://sp-foundation.org/), Australia (https://hspersunite.org.au/) and other places. There are virtual groups on Facebook (https://www.facebook.com/search/groups/?q=hereditary%20spastic%20paraplegia&epa=SERP_TAB), Rare Connect (https://www.rareconnect.org/en/community/hereditary-spastic-paraplegia), Patients Like Me (https://www.patientslikeme.com/conditions/hereditary-spastic-paraplegia) and other places.

As far as information about Covid-19 goes, I like this page from Information is Beautiful: https://informationisbeautiful.net/visualizations/covid-19-coronavirus-infographic-datapack/ This presents both data from China and Italy, it confirms that it affects older people more than younger people, and that those with underlying health conditions make up the bulk of those who have died.