Thursday, 28 November 2013

My Cold War

Some people have asked me what I thought about the hospital's response to my questions. Well, I guess I was hoping for a little more insight into the prognosis based on the outcome of others who had experienced similar plateaus. Even if we do represent only 1%, that's still around 40 people in the UK. I think consultants are just reluctant to speculate on such things. And, in fairness I didn't specifically ask about prognosis.

So winter has arrived (yes I know it's not officially until 21st December, but being friggin cold and depressing is a good enough to constitute it being winter) and I am not (why isn't there a shortened version of "am not" apart from "ain't", which I hate. Why not "am't", it would save me time! ) looking forward to the next 3 months. Apart the practical issues of going out this time of year, it's also much more difficult to muster the enthusiasm or effort to go out when it's cold and grey. The thought of sitting in the car in bushy park car park sometimes just isn't appealing enough. I have become better equipped recently with my Russian hat, but a cold wind is still a show stopper.

Stefan Evanski fighting his personal cold war 

Indoor destinations are obviously better but I struggle to think of many locally; there's only so many times one can go to Mercedes World or Brooklands within 3 months. I would like to get up to London again and go to the museums and Tate Modern but going by car is too difficult for the carers, not to mention me. I have considered going to the Tate by train, I think my wheelchair batteries could manage the return trip along the Southbank. Maybe if we get a sunny mild day with the guarantee of no rain, I will attempt it.

But the place I miss the most is my favourite part of the UK; North Wales. Specifically the location of many wonderful holidays both as a child and more recently in Penmaenmawr. It has been around 4 or 5 years since I last visited but I think that returning is very unlikely. The 4 hour journey would be very tough but the accommodation would be the biggest problem as I need both a standing hoist and full hoist, so I think it's just too difficult.

Penmaenmawr from the stunning Sychnant Pass 


Tuesday, 19 November 2013

Kings College Hospital Response

You probably remember my recent post regarding my plateau of progression and that I wrote to Kings Hospital for a response to 3 questions. Here is the reply 

Dear Steve 

Many apologies for the long delay in my replying.

To respond to your questions:
1. A pattern of plateau is rare. I would say fewer than 5% experience this, and probably fewer than 1%.

2. The diagnosis of ALS is based on the pattern of weakness and its distribution. This would not change since the condition does not reverse, so you would still have ALS. The condition has stopped progressing for some reason.

3. It would be ideal to study people who plateau, but the problem is how. Most research needs large numbers of people to obtain statistically meaningful results, and because this event is so rare and is in a rare disease, we would need a considerable length of time to recruit enough people. Most funding agencies will not consider this.

We have studied prolonged survival, which is a similar but not identical issue, in two studies. The first was of the clinical characteristics of people with long survival. We found that the pattern of ALS did not help in predicting who would plateau or progress very slowly. The PLS pattern showed very slow progression, but half the people who lived a very long time had the typical ALS pattern with no obvious pointers to a slowing of the disease (Prolonged survival in motor neuron disease: a descriptive study of the King's database 1990-2002. Turner MR, et al, Al-Chalabi A. J Neurol Neurosurg Psychiatry. 2003). The second study was a genetic analysis of everyone from several centres, to see whether any gene variants were associated with a longer survival. We found that variants of the KIFAP3 gene were associated with better survival (Reduced expression of the Kinesin-Associated Protein 3 (KIFAP3) gene increases survival in sporadic amyotrophic lateral sclerosis. Landers JE, et al, Al-Chalabi A, Brown RH Jr. Proc Natl Acad Sci U S A. 2009). This has not been replicated by others. In a related study, we collaborated with colleagues from Holland who found that the UNC13A gene could affect survival (Genome-wide association study identifies 19p13.3 (UNC13A) and 9p21.2 as susceptibility loci for sporadic amyotrophic lateral sclerosis. van Es MA, et al. Nat Genet. 2009), and colleagues from Belgium who found that variation in the EPHA4 gene could EPHA4 is a disease modifier of amyotrophic lateral sclerosis in animal models and in humans. Van Hoecke A, et al. Nat Med. 2012). We are in the process of performing full genome sequencing of 50 people, half with very long survival and half with very aggressive disease, to see if there are any genetic differences that might be relevant.

I hope that helps.

With very best wishes,

Friday, 15 November 2013

MND ain't all bad!!

My current carer Kadvi, her daughter Kelli and my new carer Katinka 

Monday, 21 October 2013

New Eyegaze System ?

As you know, my life is dependent on my eyegaze computer, maybe not literally, but without it I wouldn't have any quality of life. However my system is almost 5 years old and it's starting to creak. There are also some hardware problems which I have to contend with. A complete system reload might solve some of the issues but then how long is the hard drive, which has been spinning at 5000 rpm, 24-7 for 5 years, going to last. Yes I could replace the hard drive but that doesn't solve the issue of the system suddenly losing all power.

So I am having to consider a new system. Apart from the significant cost, which I will come back to, it's a big decision. Despite the problems with my system it is completely familiar and I use it instinctively. The thought of learning a new system is quite daunting. On the other hand, the new system apart from being reliable,  would have webcam, run more quietly , have improved sound, better environmental controls, the list goes on.

BUT, these systems still cost a small fortune (around £10k) . I paid for the first system myself and didn't expect to be around long enough to need to worry about replacing it. Survival is a costly option.

So... to get to the point, I am looking for corporate donations to fund it. I have delayed publishing this blog for several months because I feel uncomfortable asking for charity, but eventually others will probably benefit from these systems, so I am trying to view it as a delayed donation to the MNDA.

I can't promise any significant publicity for those donating, other than a mention on the blog, but it will be a very appreciated donation. And as I said  when I no longer need it, both systems will be donated to the MNDA so another person can benefit from it.

So what I am asking is that you send this blog entry by forwarding this link

to  any company you think might help. They can then click below to make a donation with Paypal after watching my video

I DO NOT want personal donations because you have already been generous previously. Just forward this blog entry to companies that you think might help

Thank you


Monday, 7 October 2013

Reasons to be Cheerful?

It's been almost 6 years since my official diagnosis and I still seem to be beating the odds. The graph is of my progression over this period. It's very unusual progression because whilst progression can vary enormously between people the progression is usually more linear with any plateaus lasting months not years, whereas I have plateaued since 2010.

It's not totally representative of my physical state because during this time there have been subtle changes which have had much bigger implications. Losing the final, albeit miniscule strength in my hand, means I can't drive my wheelchair which is a massive loss; the combination of being unable to move without assistance and then having to laboriously type out where I want to go. The second bit can sometimes be impossible when outside in the sun. The other thing which has weakened is my neck which is making travelling in the car difficult. It's been several months since I have gone anywhere without my carer either driving or as a passenger to assist with lifting my head or adjusting my chin support or neck pillow. I think me and tracy are equally nervous about going anywhere without assistance.

I haven't had any checkup appointments at Kings Hospital for about 2 years because there seems little point and it's a big hassle to get there, so I emailed them last week with three questions. The first was, how often have they seen someone plateau for several years. Secondly, is it possible that my MND has changed to a less aggressive type than ALS (and how would I find out) and finally, is it worth doing a study on patients with prolonged plateaus to discover common factors. I hope to get a response this week.

Obviously on balance this is a good situation although it might lead to some financial implications if my remaining life policies expire before I do!!! And that endowment plan from my first flat 23 years ago might actually now reach maturity in 2015, paying out a fraction of what that slimey Norwich Union rep promised, instead of the full amount if my demise occurs beforehand.

The other aspect of my unusual progression is dealing with the change in thought processes. For the first year certainly, and probably for most of the 3 years that I declined, I found it difficult to put the thought of dying out of my head. After all, I was deteriorating at a fairly typical rate and did reach an FRS score lower than many people I knew who had already died. There wasn't any reason to think I would survive any longer than average either. It's obviously impossible to know for certain but I think I had come to terms with the inevitable. Something I certainly couldn't say now.

I am aware that I have accepted my current state of disability (however shit it is) as both normal and permanent. So I have to keep reminding myself that things could change at any time and I will have to go through the first year's emotions all over again. I guess it's no different to cancer patients that go into remission.

I am also aware that I am not the only person who this unexpected situation affects. I know from the boys point of view I am more use alive than dead, and maybe in the future they might exhibit some qualities from being exposed to the current situation. But one can't avoid the fact that family life has been decimated by this illness and in many ways my prolonged survival has made things more difficult. There are almost no family activities we can do together and there is no semblance of a normal relationship. We have already both agreed that next year's holiday will be without me and but  hopefully I will be able to go on our next cruise in 2015. I don't mind because I know I wouldn't enjoy it.

So, reasons to be cheerful? I think probably yes, but there's no doubt it's not without some negative side effects which may become more significant in the future.

Friday, 27 September 2013

Goodwood, Brooklands and Manics

ok just a quick update on the last couple of weeks. Last time I mentioned the Goodwood Revival. Well, unfortunately we picked the wrong day. I had been watching the forecasts from the previous weekend, at which point the Saturday was the wet day but as the week went on the Sunday became wetter and wetter. Unfortunately they were accurate and from 2 30 it pissed down. This year there was a 9 strong party; Tracy, the boys, sue and david, adam and my carer kadvi. Knowing the likely weather we positioned ourselves on the covered wheelchair viewing platform from where you get an okayish view (once you have reminded the row of people standing at the front of the enclosure that it's not a photography platform)

We arrived around 11 30 as usual, missing the first couple of races. I had come prepared with blankets, waterproof and hat, all of which were employed during the day. The boys weren't very interested this year and by the end of the day, I think every wheelchair visitor was well aware that playing xbox is greatly preferable to watching a "bunch of old cars drive in the rain", when you're a 12 year old boy.

The rest of the party were less vocal about any dissatisfaction and alternated with being with me and looking around the various period exhibits and shops. But by four o'clock the horizontal rain and gale force wind resulted in the first stage of an early retreat. Me, kadvi. Adam and david lasted another 30 minutes but decided to take advantage of a break in the rain, returning to the car. It was such a shame because on a late summer's day it's a magical place, but in the rain it's miserable. The journey home was very slow and despite kadvi doing a great job of supporting my head from the back seat, it was a horrible journey home. And at the time I said to myself "That's the last Revival I am going to". I am not so sure now, 2 weeks on.

Last week I continued the theme of historic cars with a trip to Brooklands. It's been  18 months since I last went so it was nice to go. They have recently moved the nearby bus museum there so there was something new to see. Although it's nice seeing someone else enjoy it, especially the opportunity to go on Concorde. The weather was beautiful and I had a lovely afternoon.

On Tuesday this week we went to see the Manic Street Preachers at Shepherds Bush Empire or as every venue now seems to be prefixed, the O2 Empire. It's a bit of a pain to get to but the wheelchair viewing platform is great. Sandra, my carer for this week, kindly agreed to drive which made parking easier and the journey more comfortable. The gig was brilliant; long term readers will know we are long standing Manics fans but this is probably the smallest venue we have seen them play. This was also the first gig for ages when it wasn't freezing cold which made it far easier for me. It's been so long since I have been out at night, that in itself was a treat.


The song above is from the new album which has much more acoustic approach than previously but they played a good selection from their back catalogue and below are short clips of some of the other songs they played

Saturday, 7 September 2013

Cycle to Paris for MND

Next week, my friends are cycling to Paris to raise money and awareness for MND. I am so grateful to them for making such an enormous effort. This is Phil's information of the ride and if you would like to support them, visit

Thanks for taking the time to visit my JustGiving page

So - what's this all about then? Well, 8 of us all aged 43 to 51 decided to ride (off road) London to Paris in September 2013, around 250 miles approx while raising funds, and just as importantly, awareness, for the MND association.

This was inspired by our friend Steve-he was diagnosed with MND back in 2007, and did a cycle himself for the same charity back in 2008- details of that ride can be seen  at
Sadly Steve's condition is such that he can no longer join us - he would love to be able to. You can find out a little more about how MND affects every moment of Steve's life now in this short film he made for MNDA, and also in Steve's own blog.

Do please take a look at these- this page is not only about raising money, although of course that remains important- the more people are aware of this horrible disease and how it affects people, the better.

Next, the money bit - I can assure you that donating through JustGiving is simple, fast and secure. Once you donate, they’ll send your money directly to the charity.
So please dig deep and donate now.

Thank you,

Friday, 6 September 2013

Post Cruise

so, been back now for 2 weeks but still missing the cruise ship style. Yes, there are some advantages to being home; my profiling bed and my bidet loo (Kadvi was VERY glad to see that again!! ), but I think I could easily live on a cruise permanently. It's perfect for me. A different location every day without getting in a car, entertainment on tap, and smooth floors everywhere. I reckon £75k per year will cover it!!
Have been out a few times recently, mainly to Bushy Park but also met the boys at Chessington World of Adventures. The visit wasn't altogether successful. Firstly, although my carer gets free entrance I am expected to pay full price, a hefty £44. Even though I explained I just wanted to watch my kids and it's blindingly obvious that rollercoasters aren't my thing, they only offered a concessionary price of £30. Then the park was so busy that the boys only got on one ride. I DID get to watch some of the 10 minute sealion show (the bits when the man in front of me remembered to crouch down) and we went into the aviary too. On the plus side no birds shat on me.

The boys were taken to Alton Towers overnight by Tracy and uncle Jeremy at the end of the holiday. Being a school inset day ensured the ride tally was better than chessington although the locally situated Thorpe Park will no longer seem satisfactory.

An event I am going to is the Goodwood Revival next weekend. It's the only motorsport event I bother going to and it's a great day out IF it doesn't rain. If it's wet, the lack of covered wheelchair viewing platforms makes it a pretty difficult day. So here's hoping...

But I can't moan about this summer's weather, it's been fabulous and with the help of carers it's enabled me to go out much more than last year.


Saturday, 17 August 2013

Cruise 2013

Well we made it to our 3rd cruise. No dramas getting here, just the usual major organization of everything. There is so many things to remember to take. Boxes of feed, my own bedding, mattress wedges, multiple chargers, medication, the list seems endless. Also this year I have brought my own hoist. We have hired a full hoist for bed transfers but other transfers are MUCH easier using standing hoist. And it's proved to be an excellent decision because it has been impossible to get me sitting comfortably using the hired hoist. It's taken 4 nights to get the bed setup correctly so I am not very uncomfortable by 3am. I have only woken Kadvi once at a horrible hour so far and have managed to perservere other nights until a more reasonable time. But last I cracked it and I was comfortable all night. So it's already day 6 and we have just left Toulon and are now heading for Italy for the 3rd stop; our first stop being Gibraltar, which was without any exploding oil storage facilities this year. Although Daniel was keen to point out the remains of the location to Kadvi with "I was there" enthusiasm Tomorrow's stop has an excursions to Pisa which tracy and the boys will go on. I knew beforehand that for me, any sightseeing would be restricted to areas in the immediate vicinity of the ship or maybe slightly further where shuttle buses are available. Jake and Luke have been reluctant to participate in many activities except eating and watching the cabin TV which we have found frustrating but have given in trying to persuade them otherwise and getting the "Kevin and Perry" reaction for the next 20 minutes. Daniel has been much better at participating and has also been very helpful driving my wheelchair and coming to find me every so often. The biggest change for me compared to our previous cruise in 2011 is my inability to drive my wheelchair which is real pain in the arse. At home this restriction is irritating but here, with so much wheelchair accessible space it's particularly frustrating. I can't even turn to look in a different direction. This isn't so bad in the evening when we tend to be together, but during the day when we are typically more indepenent, it's a bit of a pain. Obviously Kadvi can take me to different locations but having spent 3 hours getting me up and ready every morning, I am trying to give her 3 hours uninterrupted break during the afternoon, because flights back to the Estonia are always available at every next stop!!! Evenings are better for me, when I can watch the nightly theatre shows, then move to one of the many bars to watch the house rock band. I think Tracy is enjoying it although I think, like for me, it's a bit lonely at times with only 3 truculent 12 year olds for conversation. I felt for her the other night as I left for a relatively early night and she was left by herself. It takes about an hour to get me to bed, so although Kadvi has been brilliant, I am conscious that whatever time I return to my cabin, she won't be able to go to bed for another 90 minutes. The weather has been good and as I write from italy it's around 30 degrees. . .

Monday, 1 July 2013

The July blog

Don't be alarmed... I know two blogs in as many weeks is a bit of shocker.

The live-in carer situation is working well, although due to the lack of progress finding a replacement 2nd carer, Kadvi, the 1st carer is doing 3 week shifts at the moment so is probably getting sick of the sight of me. Although bedtimes are no later because of the necessity for a double up carer, the daytime is much more flexible and during the last week I have gone out almost every day. It's only been local but it's a very welcome change. Kadvi has been very flexible with her break times and has combined outings with her running, so it's working well. I just hope she stays a while.

I tested a new eyegaze system recently, not primarily for me but for a friend who is considering buying one. Saying that, I wouldn't say no to a nice new eyegaze system. My current system is completely familiar to me and I know it's faults and have the necessary work arounds. But it IS starting to creak after almost 4 years constant use, and I am increasingly concerned with being left high by it failing. In an ideal world I would have this as a backup system and get a new system with all the new features.

Our cruise is getting nearer and I am starting to look forward to it now the carer has been sorted. Also I just discovered my standing hoist fits in the car so I can it on cruise. It will make transfers much quicker and easier, and more importantly it will allow me to maintain leg strength.

Wednesday, 12 June 2013

I Know ...."It's about bloody time.... "

Well it's been absolutely ages since I updated this and I expect most of you have stopped checking so it might be Christmas before you see this. To be honest not much has happened since the last update.

The most noteable thing is the completion of a garden log cabin to give some space for the live-in carer. The first live-in carer started mid May and got upto speed very quickly, the second carer started last week but has struggled to cope with everything and we have mutually agreed to part company. The agency, Enara, have been their usual incompetent selves during the process having allowed completely inadequate training time thus setting the carers up to fail. Of course the agency blame social services for not approving additional funding for more training and heaven forbid that Enara cover the cost themselves. Instead we now have to start all over with a new carer. The shortsightedness is staggering

I had another week's respite break in Princess Alice Hospice at the beginning of May to give Tracy some space. I arranged the break in February in case the care home respite breaks weren't in place. It proved wise because they weren't arranged, and still haven't been. It's incredible after the force in which social services intervened last December, that the core facility which might prevent another crisis point being reached, has progressed literally nowhere. It just confirms my opinion that much of social services is more about meetings, ticking boxes and arse covering than actually helping people.

Anyway the cabin looks great inside and out thanks to Tracy's considerable efforts and at some future point will be used for its original purpose of a place for the boys to have friends over.

The lowsy weather has prevented many excursions out and I only managed to sit outside during the last couple of weeks. Really hoping we don't get another summer like last year. I a doing ok although I have had long periods of feeling very down, but in terms of MND progression, nothing's changed significantly. But I have had some harsh reminders recently with 3 acquaintances recently losing their battles. The first was Alistair the Optimist, who produced his own album last year. The others passed away within a week of each other last month, all in their 40s.

To end on a brighter note, we are looking forward to our cruise in August which barring more exploding oil storage facilities should be excellent. And finally, those of you on facebook will already know I am sporting a new look. So for the rest of you here it is.

I am still unsure about it but it was done for purely practical reasons because the skin under my chin was being rubbed raw by the chin support. Here are the rather alarming results of my mate Chris' effort in Photoshop

Saturday, 2 February 2013

2013 so far

just a quick update as I have received a few messages wondering if everything is ok. In short, it is, but I just haven't been feeling particularly communicative recently. Also I am finding it difficult writing longer pieces. My eyegaze accuracy seems to be worse now and I spend more time deleting erroniously selected letters or words. It drives me f***ing mad and I eventually give up. So after spending an hour writing necessary emails, usually including ones to my incompetent care agency, I can't face embarking on blogging.

My neck is continuing to weaken so I am spending more time with my head resting on my chest.

So to bring you up to speed, Christmas went well, with our respective families visiting on christmas and boxing day. Haven't been out until recently but last week managed three trips out; Thursday afternoon, Les Mis at Esher cinema; Rocky Horror at Wimbledon theatre on Friday night and watching the boys bowling on Sunday.

We are currently preparing accommodation for a live-in carer. The live-in option has advantages and disadvantages but the PCT wouldn't fund anymore hours and the live-in route is cheaper for them.

Despite a live-in carer probably starting in a couple of months my care agency have decided to give me a new, completely inexperienced regular carer for the intervening weeks, expecting me to endure the stressful training period, only to start all over again with the live-in carer! Needless to say they have received some fairly strongly worded emails.