I have now been taking detrusitol for about 4 months now, and I thought time for a brief update.
I have found that my urgency has continued to be a much smaller issue than it was, and by and large I haven't had any accidents since then.
Also, I have spotted that my bowel urgency has also decreased similarly over the same time. A look at the detrusitol/tolterodine leaflet suggests that it is only for bladder issues. However, the common (less than 1 in 10) side effects include both diarrhoea and constipation so I may have found some unintended benefits here too.
In summary, I'm pleased that this issue is much more under control than it was before.
Wednesday, 11 February 2015
Saturday, 17 January 2015
Is HSP a disability?
In my work I belong to a professional association. They have started tracking disability information to try to demonstrate that they are not discriminating, as required by UK legislation.
The question begins with a statement which gives the legislative definition of disability and then gives a pick list of choices which you can select one.
Given my current minor symptoms it is clear that I choose "I do not have a disability".
But, I wanted to find out what they were using the data for and understand the process so asked a question. The interesting answer is that disability is a personal choice, and I hadn't really grasped that concept before.
It is entirely up to me to decide the point on the scale between now and when I end up (most likely) in a wheelchair when I would call myself "disabled".
It hadn't really occurred to me, assuming my symptoms progress a lot, that I could choose never to call myself disabled. In a lot of respects this is a very powerful and positive thought to carry forward.
The question begins with a statement which gives the legislative definition of disability and then gives a pick list of choices which you can select one.
Given my current minor symptoms it is clear that I choose "I do not have a disability".
But, I wanted to find out what they were using the data for and understand the process so asked a question. The interesting answer is that disability is a personal choice, and I hadn't really grasped that concept before.
It is entirely up to me to decide the point on the scale between now and when I end up (most likely) in a wheelchair when I would call myself "disabled".
It hadn't really occurred to me, assuming my symptoms progress a lot, that I could choose never to call myself disabled. In a lot of respects this is a very powerful and positive thought to carry forward.
Thursday, 15 January 2015
Life Expectancy
Last year I put up a post which compared HSP with other types of Motor Neuron Disease, and in which I summarised HSP as:
"The primary features of HSP are spasticity and weakness in the legs, varying between individuals. There is progressive difficulty walking and symptoms worsen over time. Initial symptoms are typically difficulty with balance, stubbing the toe or stumbling. Changes begin gradually. As the disease progresses, canes, walkers and eventually wheelchairs may become needed, although some people never require assistive devices. A wide variety of symptoms are observed across cases and over time, including balance, fatigue, bladder and back pain. The majority of individuals with HSP have a normal life expectancy."
http://hspjourney.blogspot.co.uk/2014/09/the-als-ice-bucket-challenge.html
As mentioned, I had borrowed the text from other HSP sites. I also used this post as the basis for one of my columns in the UK HSP Support Group Newsletter, and I was asked by one reader about life expectancy. This is an interesting question, and one I'm slightly nervous about writing about given that this is a sensitive issue. My advice for any readers who are concerned about this should contact their HSP specialist to get their own answer.
Some clarity is obtained from the NINDS website:
http://www.ninds.nih.gov/disorders/hereditary_spastic_paraplegia/hereditary_spastic_paraplegia.htm which indicates that "The majority of individuals with uncomplicated HSP have a normal life expectancy." I realise now that I should have included this important differentiation in my post (and I have now added an update accordingly). There is an increased risk with pure HSP due to falls and from infections, principally bladder.
Given that life expectancy for uncomplicated/pure HSP is usually normal this raises the question about what is the life expectancy for complex HSP. In summary, there is not much information which I can find, and this is an overview of what I have found, with links.
In my trawl of research papers there are only two papers which talk about life expectancy, both in relation to SPG11.
http://www.ncbi.nlm.nih.gov/pubmed/23443022 - A Dutch study covering 18 patients
http://www.ncbi.nlm.nih.gov/pubmed/210which - A study from France with 7 patients
The Dutch study suggests a life expectancy of 3-4 decades after the start of walking problems. The paper from France gives life expectancy of 30-40 years, but it is not clear from the abstract if this is from onset, the same as the Dutch study, or 30-40 years total. These two studies seem to have quite small sample sizes, and I dont have the benefit of the full papers to see what the uncertainty/confidence might be.
There is also a lot of information about HSP on this Washington University page http://neuromuscular.wustl.edu/spinal/fsp.html which indicates that SPG11 has an onset usually before 20 but sometimes up to 50, which perhaps ties in with the Dutch study in that some people don't get SPG11 until older than 30-40. The Washington page doesn't give life expectancy for SPG11.
The Washington page also describes all of the different forms of HSP and other related conditions, although it is not clear where all the data comes from. There are references in most areas of the page, but I don't have access to full papers to check. This page indicates that SPG13 has a life expectancy of less than 2 decades after onset.
Looking for further information starts to become more of a challenge needing to use other search terms like prognosis, life span, death, morbidity. This following paper presents all the HSP types and indicates which are complex/complicated and which are pure/uncomplicated, and could form a start.
http://download.springer.com/static/pdf/682/art%253A10.1007%252Fs00415-014-7598-y.pdf?auth66=1420585106_72fa6be6ff74353504b77ad14ce2220d&ext=.pdf
All of the other information/comments I find are not specific and I can find various answers to the question which contradict each other. Since I dont know which to believe, if any, I'll avoid any of those links here.
Originally I was quite surprised how little information there is out there on this topic. I couldn't decide if that's just because HSP is a rare condition and this aspect hasn't yet got the attention that it needs in the limited budget research world, or if it was the case that the other issues associated with complex HSP need more attention than looking at this issue. Some more thought, and discussion with others leads me to believe that life expectancy varies significantly from person to person, and perhaps that it can be varied with the level of care that is received. Which brings me back to my original observation - if this is important to you, talk to your specialist.
"The primary features of HSP are spasticity and weakness in the legs, varying between individuals. There is progressive difficulty walking and symptoms worsen over time. Initial symptoms are typically difficulty with balance, stubbing the toe or stumbling. Changes begin gradually. As the disease progresses, canes, walkers and eventually wheelchairs may become needed, although some people never require assistive devices. A wide variety of symptoms are observed across cases and over time, including balance, fatigue, bladder and back pain. The majority of individuals with HSP have a normal life expectancy."
http://hspjourney.blogspot.co.uk/2014/09/the-als-ice-bucket-challenge.html
As mentioned, I had borrowed the text from other HSP sites. I also used this post as the basis for one of my columns in the UK HSP Support Group Newsletter, and I was asked by one reader about life expectancy. This is an interesting question, and one I'm slightly nervous about writing about given that this is a sensitive issue. My advice for any readers who are concerned about this should contact their HSP specialist to get their own answer.
Some clarity is obtained from the NINDS website:
http://www.ninds.nih.gov/disorders/hereditary_spastic_paraplegia/hereditary_spastic_paraplegia.htm which indicates that "The majority of individuals with uncomplicated HSP have a normal life expectancy." I realise now that I should have included this important differentiation in my post (and I have now added an update accordingly). There is an increased risk with pure HSP due to falls and from infections, principally bladder.
Given that life expectancy for uncomplicated/pure HSP is usually normal this raises the question about what is the life expectancy for complex HSP. In summary, there is not much information which I can find, and this is an overview of what I have found, with links.
In my trawl of research papers there are only two papers which talk about life expectancy, both in relation to SPG11.
http://www.ncbi.nlm.nih.gov/pubmed/23443022 - A Dutch study covering 18 patients
http://www.ncbi.nlm.nih.gov/pubmed/210which - A study from France with 7 patients
The Dutch study suggests a life expectancy of 3-4 decades after the start of walking problems. The paper from France gives life expectancy of 30-40 years, but it is not clear from the abstract if this is from onset, the same as the Dutch study, or 30-40 years total. These two studies seem to have quite small sample sizes, and I dont have the benefit of the full papers to see what the uncertainty/confidence might be.
There is also a lot of information about HSP on this Washington University page http://neuromuscular.wustl.edu/spinal/fsp.html which indicates that SPG11 has an onset usually before 20 but sometimes up to 50, which perhaps ties in with the Dutch study in that some people don't get SPG11 until older than 30-40. The Washington page doesn't give life expectancy for SPG11.
The Washington page also describes all of the different forms of HSP and other related conditions, although it is not clear where all the data comes from. There are references in most areas of the page, but I don't have access to full papers to check. This page indicates that SPG13 has a life expectancy of less than 2 decades after onset.
Looking for further information starts to become more of a challenge needing to use other search terms like prognosis, life span, death, morbidity. This following paper presents all the HSP types and indicates which are complex/complicated and which are pure/uncomplicated, and could form a start.
http://download.springer.com/static/pdf/682/art%253A10.1007%252Fs00415-014-7598-y.pdf?auth66=1420585106_72fa6be6ff74353504b77ad14ce2220d&ext=.pdf
All of the other information/comments I find are not specific and I can find various answers to the question which contradict each other. Since I dont know which to believe, if any, I'll avoid any of those links here.
Originally I was quite surprised how little information there is out there on this topic. I couldn't decide if that's just because HSP is a rare condition and this aspect hasn't yet got the attention that it needs in the limited budget research world, or if it was the case that the other issues associated with complex HSP need more attention than looking at this issue. Some more thought, and discussion with others leads me to believe that life expectancy varies significantly from person to person, and perhaps that it can be varied with the level of care that is received. Which brings me back to my original observation - if this is important to you, talk to your specialist.
Monday, 29 December 2014
Review of 2014
Another year has passed and its time to reflect on things and consider how different things have become over the year.
Knowledge
Aside from the knowledge of my own symptoms my main knowledge activity this year has been getting further with the analysis of the research papers. This is quite a time consuming task, although it has helped me to appreciate the diversity of research that happens and what topics tie in with HSP. The main publication was early in the year with a study reporting new HSP genes and link with other diseases.
Symptoms
This year has seen a number of firsts - My first appointment with an HSP specialist, my first medication (Detrusitol), and if it weren't for the speed of the NHS system I should have had my first neuro-physio appointment as well. I've also been on a stress/mood management course, seen a bowel specialist and I'm using banisters more when going up and down stairs. I'll have to chalk up 2014 as a year of appointments, although I know already that there will be a fair few in 2015.
This Blog
The readership of this blog continues to increase. I've had more page views in 2014 than I had in 2013. My audience remains broadly the same (predominantly US, UK, Ukraine, France, Germany, Turkey, Russia). The most popular posts are the results of my survey, the various presentations given at the UK HSP Support Group AGMs and my general posts on particular HSP symptoms.
I've had various comments made that people appreciate reading what I have to say, which I'm very pleased to receive and gives another reason why this is a worthwhile thing to do. Just recently I had a conversation with my uncle who commented that when he was first diagnosed with HSP there was not very much information around at all. The most surprising thing I find is that people I know now come up and ask me about it, which I always find quite surprising!
Survey
I was really surprised how much coverage and readership the results of my 2013 survey were when I published them on 28th Feb, and this really set the path in motion for doing this every year. I've already got a few ideas lined up for 2015 and beyond.
I'll follow the same path for the 2014 survey - analysis will start in a week or so's time so I can write up to publish on 28th Feb - Rare disease day. There are still a few more days when I'll be taking answers!
Knowledge
Aside from the knowledge of my own symptoms my main knowledge activity this year has been getting further with the analysis of the research papers. This is quite a time consuming task, although it has helped me to appreciate the diversity of research that happens and what topics tie in with HSP. The main publication was early in the year with a study reporting new HSP genes and link with other diseases.
Symptoms
This year has seen a number of firsts - My first appointment with an HSP specialist, my first medication (Detrusitol), and if it weren't for the speed of the NHS system I should have had my first neuro-physio appointment as well. I've also been on a stress/mood management course, seen a bowel specialist and I'm using banisters more when going up and down stairs. I'll have to chalk up 2014 as a year of appointments, although I know already that there will be a fair few in 2015.
This Blog
The readership of this blog continues to increase. I've had more page views in 2014 than I had in 2013. My audience remains broadly the same (predominantly US, UK, Ukraine, France, Germany, Turkey, Russia). The most popular posts are the results of my survey, the various presentations given at the UK HSP Support Group AGMs and my general posts on particular HSP symptoms.
I've had various comments made that people appreciate reading what I have to say, which I'm very pleased to receive and gives another reason why this is a worthwhile thing to do. Just recently I had a conversation with my uncle who commented that when he was first diagnosed with HSP there was not very much information around at all. The most surprising thing I find is that people I know now come up and ask me about it, which I always find quite surprising!
Survey
I was really surprised how much coverage and readership the results of my 2013 survey were when I published them on 28th Feb, and this really set the path in motion for doing this every year. I've already got a few ideas lined up for 2015 and beyond.
I'll follow the same path for the 2014 survey - analysis will start in a week or so's time so I can write up to publish on 28th Feb - Rare disease day. There are still a few more days when I'll be taking answers!
Wednesday, 10 December 2014
Power Plate/Circulation Booster
My mum got a power plate/circulation booster for her birthday. Looking around these devices seem to come in two varieties.
Firstly, there are the "vibrating plate" type where there are various motors within the plate which cause it to vibrate. One of the main brands is Power Plate. These machines help get muscle tone when exercises are done whilst on the plate. Exercises might include squats, push-ups etc. The vibration causes the muscles to activate by reflex to compensate for the movement of the plate. This is, essentially, anerobic exercise. There are many claims made about what this can do, along with many sceptical people. It seems to me that such a device is unlikely to give massive weight loss, but getting muscles to activate by reflex sounds like there could be some benefits for HSP.
One study I found included someone with HSP which concluded that spasticity decreased in patients with cerebral palsy with the use of these plates.
http://www.medicaljournals.se/jrm/content/download.php%3Fdoi%3D10.1080/16501970600680262
Another shows improvement in walking in a patient with SP.
http://www.ncbi.nlm.nih.gov/pubmed/23045444
The other type of machine is the "circulation booster". This machine applies an electrical current to the soles of your feet to cause the muscles to activate by mimicking the muscle activation signals sent by the brain. The process is called electro muscle stimulation, or EMS. Generally EMS is used to in a number of ways to develop muscle strength. It is also used for rehabilitation and/or to prevent muscle loss/atrophy. Similarly there are weight loss claims which are generally received sceptically.
This technique is similar to FES (functional electrical stimulation). The circulation booster claims to stimulate muscles and improve blood circulation. There are a fair few papers out there which detail FES with HSP, but I couldn't find any for EMS.
To get back to my original line, my mum got one of the circulation boosters. I tried this for half an hour whilst I was there over the summer. I could feel that my muscles were being activated, and by the end of half hour I could feel this in my upper leg muscles too. Of course, half an hour isn't really long enough for a proper assessment, but one for me to bear in mind in the future.
Firstly, there are the "vibrating plate" type where there are various motors within the plate which cause it to vibrate. One of the main brands is Power Plate. These machines help get muscle tone when exercises are done whilst on the plate. Exercises might include squats, push-ups etc. The vibration causes the muscles to activate by reflex to compensate for the movement of the plate. This is, essentially, anerobic exercise. There are many claims made about what this can do, along with many sceptical people. It seems to me that such a device is unlikely to give massive weight loss, but getting muscles to activate by reflex sounds like there could be some benefits for HSP.
One study I found included someone with HSP which concluded that spasticity decreased in patients with cerebral palsy with the use of these plates.
http://www.medicaljournals.se/jrm/content/download.php%3Fdoi%3D10.1080/16501970600680262
Another shows improvement in walking in a patient with SP.
http://www.ncbi.nlm.nih.gov/pubmed/23045444
The other type of machine is the "circulation booster". This machine applies an electrical current to the soles of your feet to cause the muscles to activate by mimicking the muscle activation signals sent by the brain. The process is called electro muscle stimulation, or EMS. Generally EMS is used to in a number of ways to develop muscle strength. It is also used for rehabilitation and/or to prevent muscle loss/atrophy. Similarly there are weight loss claims which are generally received sceptically.
This technique is similar to FES (functional electrical stimulation). The circulation booster claims to stimulate muscles and improve blood circulation. There are a fair few papers out there which detail FES with HSP, but I couldn't find any for EMS.
To get back to my original line, my mum got one of the circulation boosters. I tried this for half an hour whilst I was there over the summer. I could feel that my muscles were being activated, and by the end of half hour I could feel this in my upper leg muscles too. Of course, half an hour isn't really long enough for a proper assessment, but one for me to bear in mind in the future.
Friday, 28 November 2014
Update on research analysis
I've been keeping myself busy over the last few weeks. Back in 2013 I had downloaded all of the papers on the PubMed database which referenced HSP, but I hadn't really done anything with them. I've now undertaken two passes through the papers getting ready to put a more in depth analysis up on a new page.
I've limited myself to papers with abstracts - so I've got some better information on what information the paper contains, there are just over 1000 of those.
My first pass was to look through the papers and give them a "usefulness" rank. Papers which I thought would be very useful were given the rank of 10, with smaller numbers for papers which were less relevant. My "default" rank for papers on HSP which aren't obviously useful is 4, which allowed me to score lower than this for papers which mention HSP in passing rather than being specifically about HSP (or some aspect of this).
My second pass has been to read all of the abstracts again and divide the papers into categories. Evan Reid had indicated at the AGM in the summer that HSP research could be divided up into three zones, and I wanted to see what these zones looked like. I began with the papers which I had ranked 10, and then worked down the list until I got to rank 4, so there are some 800 papers which I've done this for.
This post is my initial results. I used the set of results which I had previously downloaded, so this includes papers up to some point in 2013.
When I came to go through the papers and categorise them it was obvious that some of my rankings may need adjusting, and that by the time I got a reasonable way through the process my initial thoughts on what the categories should include have changed. Basically, I'm just saying that this is a work in progress.
This is the table of topics:
I've limited myself to papers with abstracts - so I've got some better information on what information the paper contains, there are just over 1000 of those.
My first pass was to look through the papers and give them a "usefulness" rank. Papers which I thought would be very useful were given the rank of 10, with smaller numbers for papers which were less relevant. My "default" rank for papers on HSP which aren't obviously useful is 4, which allowed me to score lower than this for papers which mention HSP in passing rather than being specifically about HSP (or some aspect of this).
My second pass has been to read all of the abstracts again and divide the papers into categories. Evan Reid had indicated at the AGM in the summer that HSP research could be divided up into three zones, and I wanted to see what these zones looked like. I began with the papers which I had ranked 10, and then worked down the list until I got to rank 4, so there are some 800 papers which I've done this for.
This post is my initial results. I used the set of results which I had previously downloaded, so this includes papers up to some point in 2013.
When I came to go through the papers and categorise them it was obvious that some of my rankings may need adjusting, and that by the time I got a reasonable way through the process my initial thoughts on what the categories should include have changed. Basically, I'm just saying that this is a work in progress.
This is the table of topics:
| Topic | Earliest | Latest | Papers |
| Treatments | 1984 | 2012 | 20 |
| Clinical studies | 1980 | 2012 | 93 |
| Clinical and Genetic studies | 1981 | 2013 | 51 |
| Genetic studies | 1996 | 2012 | 67 |
| Genetic identification | 1983 | 2013 | 241 |
| Assessment tools | 1975 | 2013 | 25 |
| Reviews | 1953 | 2013 | 34 |
| Models | 1998 | 2013 | 62 |
| Biology | 1976 | 2013 | 100 |
| Prevalence | 1985 | 2009 | 11 |
| Multi-illness studies | 1975 | 2013 | 113 |
| Uncategorised | 1946 | 2013 | 376 |
I've used the "biology" category as a bit of "not sure" category at times, so there may be a few papers in this topic which are not strictly to do with the biology of HSP. Other papers cover a few different topics, so there will be many papers which could easily be put into different categories.
I suspect that readers would be most interested in the treatment category. These papers comprise:
9 papers on Baclofen
3 papers on Botox
2 papers on Levodopa
1 paper each for: Gabapentin, Electrical stimulation, Progabide, Methylphenidate and Ranibizumab
There was one further paper which looked generally at matching drugs to diseases based on genetic analysis.
In addition to some of these papers, my most relevant papers covered:
Depression, bladder function, cognitive performance, sensory function, continence and the prevalence of HSP. All in all there are about 30 papers which I thought would be most useful.
This is how all of those categories look on a graph:
Next steps:
Create a page on this blog to hold such information
Add papers published since end of last trawl
Classify papers with ranks of 3 and below
Review classification and topic designations
Ultimately I'm aiming to draw out analysis for different aspect of HSP, but I'm a way off that yet.
9 papers on Baclofen
3 papers on Botox
2 papers on Levodopa
1 paper each for: Gabapentin, Electrical stimulation, Progabide, Methylphenidate and Ranibizumab
There was one further paper which looked generally at matching drugs to diseases based on genetic analysis.
In addition to some of these papers, my most relevant papers covered:
Depression, bladder function, cognitive performance, sensory function, continence and the prevalence of HSP. All in all there are about 30 papers which I thought would be most useful.
This is how all of those categories look on a graph:
Next steps:
Create a page on this blog to hold such information
Add papers published since end of last trawl
Classify papers with ranks of 3 and below
Review classification and topic designations
Ultimately I'm aiming to draw out analysis for different aspect of HSP, but I'm a way off that yet.
Monday, 24 November 2014
A trip to the bowel doctor
Today I went to see the colorectal surgery team, so thats another part of the health system ticked off. As a reminder, this was suggested by the HSP specialist as I'd been experiencing bowel urgency problems, and this is not viewed as a common HSP symptom.
So, I wasnt sure what to expect on this visit, could it be about seeing if we could rule out other things, or would it be about management of the situation. It turned out to be a bit of both.
In the ruling out of other things front: I was examined, and things are not quite as expected, so I'll have a further appointment to have some nerve tests done, and then we'll hopefully know a bit more. The specialist I saw has not had any previous experience with HSP.
here's a description of things I've learnt about the bowel today:
Your bowel is controlled by two sphincter muscles, an inner one and an outer one. The inner one stays tight all of the time, and its main job is to prevent leakage. This muscle is not one which you have control over. The outer sphincter also stays closed all the time, but you control over this one, which allows you to choose when you want to go.
There are two types of incontinence - one where you know that you've got a problem - and the issue is getting to the toilet in time, and another when you dont know you've got a problem - and you end up with a mess to deal with.
On the management front:
Management strategy 1: It is possible to re-train the muscles for some people. But the general thought was that as the correct messages are likely as not failing to get through, this strategy may not work.
Management strategy 2: Use Loperamide / Imodium. Some people are on this all the time. This can help by making your stool/faeces/feces firmer, and therefore giving a bit more time to get to the toilet. However, as my urgency issue is not predictable this makes it difficult to use pro-actively. It could be taken when loose stools are noticed, or taken when it is known that you may have difficulty finding a toilet in time.
Management strategy 3: This was mentioned in passing, and not appropriate for me now. You can get a home colonic irrigation kit. Some people will use this 2-3 times a week, and others will use this after they have been once, which then makes you empty for a while, meaning that you cant have any incidents.
These are the management strategies which were mentioned, suggesting that there are some more. Perhaps I'll find out more at my next appointment. The one which springs to mind is checking that my diet helps the situation rather than hinders it.
So, in conclusion, it looks like I'll need to go to the chemist and get some imodium to add to my collection of tablets. I'll need to understand how quickly this works and how long it lasts.
So, I wasnt sure what to expect on this visit, could it be about seeing if we could rule out other things, or would it be about management of the situation. It turned out to be a bit of both.
In the ruling out of other things front: I was examined, and things are not quite as expected, so I'll have a further appointment to have some nerve tests done, and then we'll hopefully know a bit more. The specialist I saw has not had any previous experience with HSP.
here's a description of things I've learnt about the bowel today:
Your bowel is controlled by two sphincter muscles, an inner one and an outer one. The inner one stays tight all of the time, and its main job is to prevent leakage. This muscle is not one which you have control over. The outer sphincter also stays closed all the time, but you control over this one, which allows you to choose when you want to go.
There are two types of incontinence - one where you know that you've got a problem - and the issue is getting to the toilet in time, and another when you dont know you've got a problem - and you end up with a mess to deal with.
On the management front:
Management strategy 1: It is possible to re-train the muscles for some people. But the general thought was that as the correct messages are likely as not failing to get through, this strategy may not work.
Management strategy 2: Use Loperamide / Imodium. Some people are on this all the time. This can help by making your stool/faeces/feces firmer, and therefore giving a bit more time to get to the toilet. However, as my urgency issue is not predictable this makes it difficult to use pro-actively. It could be taken when loose stools are noticed, or taken when it is known that you may have difficulty finding a toilet in time.
Management strategy 3: This was mentioned in passing, and not appropriate for me now. You can get a home colonic irrigation kit. Some people will use this 2-3 times a week, and others will use this after they have been once, which then makes you empty for a while, meaning that you cant have any incidents.
These are the management strategies which were mentioned, suggesting that there are some more. Perhaps I'll find out more at my next appointment. The one which springs to mind is checking that my diet helps the situation rather than hinders it.
So, in conclusion, it looks like I'll need to go to the chemist and get some imodium to add to my collection of tablets. I'll need to understand how quickly this works and how long it lasts.
Sunday, 26 October 2014
Reversing Paralysis - Spinal Cord Regeneration
This week (in the UK) there has been a very interesting story in the news - A man in Poland has been able to walk after surgery to repair his spinal cord. He had been paralysed from the chest down after a knife attack in 2010.
You can read the news story here:
http://www.bbc.co.uk/news/health-29645760
http://www.theguardian.com/science/2014/oct/21/paralysed-darek-fidyka-pioneering-surgery
http://thenextstepsci.org.au/research/
This was featured in the BBC Panorama program, which you can watch on the BBC iPlayer until Oct 2015: http://www.bbc.co.uk/programmes/b04mm8zl
I watched the program after hearing about the story on the news, with a thought that this may yield a treatment for HSP. Here is my take on the story (as presented on Panorama).
Researchers had identified that the olfactory system (sense of smell) was the only system in the human body where nerves were continually regenerated throughout life. In all other systems nerves, once damaged, do not re-grow.
Much research has been done over many years to identify if the olfactory cells could be used to encourage re-growth in other nerve systems, with work being undertaken at UCL (University College London) - Professor Geoffrey Raisman featured on the program. They had undertaken research with mice which had shown potential. Olfactory ensheathing cells (OECs) were shown to regenerate the olfactory nerves, and the research involved extracting these cells and transplanting them to examine if they would regenerate other nerves.
The research had concluded that the cells needed to be extracted from the olfactory bulb, which is located in the brain. This means that very invasive surgery is needed to extract them - the program didnt go into the details of the cell extraction.
A team in Poland, led by Dr Pawel Tabakow of Wroclaw Medical University, was keen to take this further. Darek Fidyka had been paralysed from the chest down after a knife attack in 2010. He volunteered to undergo the surgery to extract the cells from his olfactory bulbs. These cells were then grown in the lab for a couple of weeks before being injected into the spinal cord at the injury site.
The story showed the operation to implant the cells into the spinal cord. The damage site was bigger than expected from the various MRI scans - half an inch (10-15mm) rather than a nice clean slice. There was a small section of cord which was intact, but most of it had gone in the attack. The view was that whilst the cells would encourage nerve re-growth they would not bridge this gap, so a section of nerve from the ankle was inserted to form a bridge. Some cells were injected into the section of the cord which remained, but most were injected into the spinal cord either side of the bridge. They were only able to extract a very small amount of these cells, which remained a small amount even after 2 weeks growth in the lab. It was quite amazing to see the surgery.
The surgery was undertaken in 2012. After a few months intense physiotherapy there was some activity, and this developed over the next two years such that there was muscle re-growth, control from the brain and the brain was receiving feedback - i.e. there was re-establishment of 2-way communication through the spinal cord. Darek was also able to regain some bladder, bowel and sexual function again. After two years he was able to walk again and is able to drive.
This is one example. The research had been conducted to demonstrate that the surgery and cell implants had done the job, although there is still some scepticism. The next steps for this research is to repeat the process in a few more patients which give a much more robust dataset to demonstrate that it works.
The story mentioned that the results were published, so that the scientific community can scrutinise them. That article (or at least an abstract for it) can be found here:
http://www.ncbi.nlm.nih.gov/pubmed/25338642
My take on the key points are:
So the question I consider is: how likely is it, given that our spasticity is like paralysis, that this kind of treatment could be used for HSP?
My understanding is that the degeneration of the nerves in HSP is along the nerves rather than across them, and they degenerate from the end, rather than at a place mid-way along the spinal cord. The main problem, from my limited understanding, is that in HSP the nerves degenerate from the end, so I suspect that even if a long piece of nerve could be used to bridge over the degenerated area of the spinal column the issue will be that the join between the upper motor neurons and lower motor neurons would still be missing. So my suspicion is that unless a "bridge" can be created between upper and lower motor neurons, this is unlikely to help people with HSP in the short term.
However, the program was keen to point out that this effectively showed proof of concept, and that the researchers were keen to share their knowledge. This may mean that the technique or the knowledge may be used to help understanding or provide new ideas for treatment. The comparison was made in the programme with antibiotics - we take these as a given now, but re-wind about a hundred years (to 1928) and Alexander Flemming first discovered the antibiotic effects of Penicillin. Now we have many many more antibiotics which are much more powerful than Penicillin, but that was the starting point. Perhaps that kind of path can follow from this process.
You can read the news story here:
http://www.bbc.co.uk/news/health-29645760
http://www.theguardian.com/science/2014/oct/21/paralysed-darek-fidyka-pioneering-surgery
http://thenextstepsci.org.au/research/
This was featured in the BBC Panorama program, which you can watch on the BBC iPlayer until Oct 2015: http://www.bbc.co.uk/programmes/b04mm8zl
I watched the program after hearing about the story on the news, with a thought that this may yield a treatment for HSP. Here is my take on the story (as presented on Panorama).
Researchers had identified that the olfactory system (sense of smell) was the only system in the human body where nerves were continually regenerated throughout life. In all other systems nerves, once damaged, do not re-grow.
Much research has been done over many years to identify if the olfactory cells could be used to encourage re-growth in other nerve systems, with work being undertaken at UCL (University College London) - Professor Geoffrey Raisman featured on the program. They had undertaken research with mice which had shown potential. Olfactory ensheathing cells (OECs) were shown to regenerate the olfactory nerves, and the research involved extracting these cells and transplanting them to examine if they would regenerate other nerves.
The research had concluded that the cells needed to be extracted from the olfactory bulb, which is located in the brain. This means that very invasive surgery is needed to extract them - the program didnt go into the details of the cell extraction.
A team in Poland, led by Dr Pawel Tabakow of Wroclaw Medical University, was keen to take this further. Darek Fidyka had been paralysed from the chest down after a knife attack in 2010. He volunteered to undergo the surgery to extract the cells from his olfactory bulbs. These cells were then grown in the lab for a couple of weeks before being injected into the spinal cord at the injury site.
The story showed the operation to implant the cells into the spinal cord. The damage site was bigger than expected from the various MRI scans - half an inch (10-15mm) rather than a nice clean slice. There was a small section of cord which was intact, but most of it had gone in the attack. The view was that whilst the cells would encourage nerve re-growth they would not bridge this gap, so a section of nerve from the ankle was inserted to form a bridge. Some cells were injected into the section of the cord which remained, but most were injected into the spinal cord either side of the bridge. They were only able to extract a very small amount of these cells, which remained a small amount even after 2 weeks growth in the lab. It was quite amazing to see the surgery.
The surgery was undertaken in 2012. After a few months intense physiotherapy there was some activity, and this developed over the next two years such that there was muscle re-growth, control from the brain and the brain was receiving feedback - i.e. there was re-establishment of 2-way communication through the spinal cord. Darek was also able to regain some bladder, bowel and sexual function again. After two years he was able to walk again and is able to drive.
This is one example. The research had been conducted to demonstrate that the surgery and cell implants had done the job, although there is still some scepticism. The next steps for this research is to repeat the process in a few more patients which give a much more robust dataset to demonstrate that it works.
The story mentioned that the results were published, so that the scientific community can scrutinise them. That article (or at least an abstract for it) can be found here:
http://www.ncbi.nlm.nih.gov/pubmed/25338642
My take on the key points are:
- Invasive surgery is needed to extract the cells
- Invasive survey is needed to inject the cells into the spine
- The procedure was shown to work at a small site where there was relatively clean damage of the cord
- Other sections of nerve can be used to act as a bridge to replace missing parts of the spinal cord
- It is a long and hard process getting movement back
- The repair seemed to have benefits in other lower body functions
So the question I consider is: how likely is it, given that our spasticity is like paralysis, that this kind of treatment could be used for HSP?
My understanding is that the degeneration of the nerves in HSP is along the nerves rather than across them, and they degenerate from the end, rather than at a place mid-way along the spinal cord. The main problem, from my limited understanding, is that in HSP the nerves degenerate from the end, so I suspect that even if a long piece of nerve could be used to bridge over the degenerated area of the spinal column the issue will be that the join between the upper motor neurons and lower motor neurons would still be missing. So my suspicion is that unless a "bridge" can be created between upper and lower motor neurons, this is unlikely to help people with HSP in the short term.
However, the program was keen to point out that this effectively showed proof of concept, and that the researchers were keen to share their knowledge. This may mean that the technique or the knowledge may be used to help understanding or provide new ideas for treatment. The comparison was made in the programme with antibiotics - we take these as a given now, but re-wind about a hundred years (to 1928) and Alexander Flemming first discovered the antibiotic effects of Penicillin. Now we have many many more antibiotics which are much more powerful than Penicillin, but that was the starting point. Perhaps that kind of path can follow from this process.
Thursday, 16 October 2014
Symptoms Update (and a trip to the Doctor)
I mentioned a few months ago (in May: http://hspjourney.blogspot.co.uk/2014/05/hsp-clinic-visit.html) my visit to the HSP Clinic at the National Hospital in London. Not long after that I received a copy of a letter which had been written to my doctor in order to take further the various things which we had discussed at the HSP Clinic.
It was only after a few months had passed that I realised that nothing seemed to be happening, and I popped in to the doctors. Waiting in the box behind the counter was a prescription for Detrusitol, which had been sitting there since June, but nothing else had happened. It seems that I should have made an appointment to discuss these. So, that is what I have done.
The main function of my trip to the doctors was to move forward the various appointments that had been suggested at the Clinic, and these are now starting to come through. Readers outside the UK may be interested to know that I get a letter which allows me to go onto a website and choose my own appointment (and if it wasnt a specialist I was seeing, I'd be able to choose where I went too).
So, thats put the whole thing back by a few months, and its made me realise that I have to be the one who drives the process forward. Despite any best interests or good meaning, there is only one person who is responsible for me seeing the various specialists, and that person is myself [This is something I picked up from a seminar at work some years ago in the context of personal development - you are the only person responsible for your training and progress. There is no point trying to blame someone else/the system if you dont do what you want to do].
Back to the first part of the topic title - symptoms update....
I've now been taking Detrusitol for about 3 weeks. My prescription is for Detrusitol XL (a 4mg dose) and I take one tablet per day. The active ingredient in this medicine is tolterodine, and it is taken for overactive bladders. Other names for this medication include Detrol, My doctor said that there are other medicines available which achieve the same outcomes.
I noticed a reduction in urgency pretty immediately with this medicine, which is a good thing! I've not noticed that I've been going to the toilet any less frequently, but the drop in urgency is a real benefit.
The most common side effect is a dry mouth, which I get. Of course, my first reaction with a dry mouth is to have a glass of water, which perhaps goes somewhat against the grain of the objectives as I'm just filling my bladder up more quickly!
I havent really noticed any other side effects, which means that either they are not there, or they are not at a level which I would notice. The other common ones include tiredness, fatigue and headaches. I get bothered by these sometimes anyway, and I've not noticed them appearing more often in the last few weeks.
The other main symptom update to report is my use of the stairs. I realise now that I'm actively using the bannister/hand-rail to get up and down to majority of stairs, whereas a few years ago I didn't need to at all. This is a subtle change. I do this more for steadiness than for balance/trips/falls, and I can still nip up and down stairs without using them. Just yesterday evening I arrived back to my nearest train station and walked up the steps to cross the footbridge - the handrails were filthy, but I still continued to use them (it had been a long day, I'd been on the go for about 17 hours). I must send the station an e-mail about this.
One final point for today on symptoms is to note that I bought a new pair of shoes the other week. I only mention this so when I next need to replace them I can find out how long I've had them.
Various Tolterodine / Detrusitol links:
http://www.patient.co.uk/medicine/tolterodine
http://en.wikipedia.org/wiki/Tolterodine
http://www.medicines.org.uk/emc/medicine/7685/SPC/Detrusitol+XL+4mg/
http://www.nhs.uk/medicine-guides/pages/MedicineOverview.aspx?condition=Urinary%20retention%20and%20incontinence&medicine=Tolterodine%20tartrate&preparationTolterodine%204mg%20modified-release%20capsules
http://www.nlm.nih.gov/medlineplus/druginfo/meds/a699026.html
http://www.nhs.uk/medicine-guides/pages/selectorshow.aspx?medicine=Detrusitol
http://www.netdoctor.co.uk/seniors-health/medicines/detrusitol.html
It was only after a few months had passed that I realised that nothing seemed to be happening, and I popped in to the doctors. Waiting in the box behind the counter was a prescription for Detrusitol, which had been sitting there since June, but nothing else had happened. It seems that I should have made an appointment to discuss these. So, that is what I have done.
The main function of my trip to the doctors was to move forward the various appointments that had been suggested at the Clinic, and these are now starting to come through. Readers outside the UK may be interested to know that I get a letter which allows me to go onto a website and choose my own appointment (and if it wasnt a specialist I was seeing, I'd be able to choose where I went too).
So, thats put the whole thing back by a few months, and its made me realise that I have to be the one who drives the process forward. Despite any best interests or good meaning, there is only one person who is responsible for me seeing the various specialists, and that person is myself [This is something I picked up from a seminar at work some years ago in the context of personal development - you are the only person responsible for your training and progress. There is no point trying to blame someone else/the system if you dont do what you want to do].
Back to the first part of the topic title - symptoms update....
I've now been taking Detrusitol for about 3 weeks. My prescription is for Detrusitol XL (a 4mg dose) and I take one tablet per day. The active ingredient in this medicine is tolterodine, and it is taken for overactive bladders. Other names for this medication include Detrol, My doctor said that there are other medicines available which achieve the same outcomes.
I noticed a reduction in urgency pretty immediately with this medicine, which is a good thing! I've not noticed that I've been going to the toilet any less frequently, but the drop in urgency is a real benefit.
The most common side effect is a dry mouth, which I get. Of course, my first reaction with a dry mouth is to have a glass of water, which perhaps goes somewhat against the grain of the objectives as I'm just filling my bladder up more quickly!
I havent really noticed any other side effects, which means that either they are not there, or they are not at a level which I would notice. The other common ones include tiredness, fatigue and headaches. I get bothered by these sometimes anyway, and I've not noticed them appearing more often in the last few weeks.
The other main symptom update to report is my use of the stairs. I realise now that I'm actively using the bannister/hand-rail to get up and down to majority of stairs, whereas a few years ago I didn't need to at all. This is a subtle change. I do this more for steadiness than for balance/trips/falls, and I can still nip up and down stairs without using them. Just yesterday evening I arrived back to my nearest train station and walked up the steps to cross the footbridge - the handrails were filthy, but I still continued to use them (it had been a long day, I'd been on the go for about 17 hours). I must send the station an e-mail about this.
One final point for today on symptoms is to note that I bought a new pair of shoes the other week. I only mention this so when I next need to replace them I can find out how long I've had them.
Various Tolterodine / Detrusitol links:
http://www.patient.co.uk/medicine/tolterodine
http://en.wikipedia.org/wiki/Tolterodine
http://www.medicines.org.uk/emc/medicine/7685/SPC/Detrusitol+XL+4mg/
http://www.nhs.uk/medicine-guides/pages/MedicineOverview.aspx?condition=Urinary%20retention%20and%20incontinence&medicine=Tolterodine%20tartrate&preparationTolterodine%204mg%20modified-release%20capsules
http://www.nlm.nih.gov/medlineplus/druginfo/meds/a699026.html
http://www.nhs.uk/medicine-guides/pages/selectorshow.aspx?medicine=Detrusitol
http://www.netdoctor.co.uk/seniors-health/medicines/detrusitol.html
Wednesday, 10 September 2014
Autumnal Survey 2014
Update: This 2014 survey is now closed. For details of the results of this survey and any current surveys, please see this page: http://hspjourney.blogspot.co.uk/p/my-on-line-resarch.html
Original Post:
After the success of my survey last year, the results of which has given me my most read page, I decided to undertake another survey this year.
My main focus for this survey is medication so that I can understand the range of medication used by people with HSP. So I've put a handful of questions together about this. I also touch on diet, exercise and relaxation to get a more complete picture.
Like last year I will collect results until around the end of the year and analyse these to publish on rare disease day 28th Feb 2015.
Also like last year, all questions are optional, and I don't collect any personal info apart from your name. If you took part last time, I'd appreciate using the same name to allow tracking.
I would appreciate any readers with HSP to complete this.
http://www.surveygizmo.com/s3/1797674/HSP-Medication-diet-and-exercise
Since I published the results of last years survey there have been a few more responses which I can analyse.
Original Post:
After the success of my survey last year, the results of which has given me my most read page, I decided to undertake another survey this year.
My main focus for this survey is medication so that I can understand the range of medication used by people with HSP. So I've put a handful of questions together about this. I also touch on diet, exercise and relaxation to get a more complete picture.
Like last year I will collect results until around the end of the year and analyse these to publish on rare disease day 28th Feb 2015.
Also like last year, all questions are optional, and I don't collect any personal info apart from your name. If you took part last time, I'd appreciate using the same name to allow tracking.
I would appreciate any readers with HSP to complete this.
http://www.surveygizmo.com/s3/1797674/HSP-Medication-diet-and-exercise
Since I published the results of last years survey there have been a few more responses which I can analyse.
Subscribe to:
Posts (Atom)