Matt Bellamy, watch out. Jake plays Hysteria by Muse

It Didn't Take Long ....

Didn't take long to find something to whinge about in 2014 although it's bugged me for ages; disabled parking

The blue badge scheme is a great idea but it is subject to so much abuse and misuse, its value is undermined. There are 2 obvious problems and 1 more controversial one. First there are the people without badges who are simply too f***ing lazy and selfish, so simply ignore the restrictions. When I could still speak I used to enjoy challenging these people just to hear there excuses and watch them squirm! The most frequent response was "oh sorry, I didn't realise it was disabled" or "I was only going to be 5 minutes". Even if in the unlikely event of this being true, it's of little consolation when I arrive during those 5 minutes and can't park. The next offending group are those with legitimate blue badges but seem to have forgotten the disabled person! These people are harder to challenge because the number of excuses is greater and occasionally more plausible, such as they're picking up the disabled person, to which you can't really argue, although 95% of the time it's total b******s

I HAVE actually challenged someone using eyegaze computer voice, but eyegaze typing doesn't lend itself to arguments and they are long gone before I have finished typing "you are a selfish c***"!

The final problem is more controversial. I have lost count of the times I have been sitting in Bushy Park car park and seen cars with blue badges park next to me, and someone emerges with the tiniest of limps and proceeds to walk a mile around the lake. I don't want to get involved in a cripple competition but it does annoy me. It's not such a problem in car parks with multiple disabled parking spaces but when the single space outside a London theater has been taken by a "limper", it is infuriating

I don't know what the answer is, I have thought maybe they should introduce a two tier system which helps ensure the most premium spaces can only be used by the most needy. I guess it's too costly and complicated to achieve and like many of those limpers do already, people will simply exaggerate their disability to get the higher level. While on the subject of questionable applications for a blue badge, there is a more serious implication of exaggerated claims. Because one of the criteria for a blue badge is qualifying for the higher level of disability living allowance, it means many people will be receiving several thousand pounds each year with questionable legitimacy.
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MND

Here are some links to accompany my video

The Fiat Doblo In Front instructions are here which explain the wheelchair loading procedure

Fiat Doblo In Front instructions

The narrative for the video is here

Video Narrative

The company who write The Grid 2 software and supply a wide range of communication devices including eyegaze systems are Smartbox. I can't recommend them highly enough. Absolutely superb.

http://www.smartboxat.co

The wheelchair computer mount and power supply which powers USB devices were supplied and fitted by Smile Rehab. They also supply a wide range of products including wheelchair controls. They are very helpful.

http://www.smilerehab.com/

The company who do the Fiat Doblo conversion are Wilson Healey
but they now sell the vehicles through Accessible Vehicles
http://www.accessiblevehicles.co.uk/

My Wheelchair
Invacare tdx-sp


This is the standing hoist I use. It gives excellent leg support

sara 3000 standing hoist

I didn't demonstrate my toilet for obvious reasons, but here are the details

http://www.clos-o-mat.com/

The software I use to control the other computers is Dameware Mini Remote Control. It's gone up in  price considerably recently, but if you need to regularly control other computers, this is much better than Teamviewer or log-me-in, because no user intervention is required on the remote computer, it doesn't even need to be logged in.

mini-remote-control

These are the details of the bed

volker-3080-bed


Tacky Spray
stickum


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Happy New Year

Well, looks like I have made it into another year. Have to say I am glad to see the back of 2013. There were highlights including fabulous weather, a very enjoyable cruise and finding an excellent live-in carer. But the year was marred by the totally unnecessary and intrusive dealings with social services. Anyway that's all finished now so hopefully 2014 will be an improvement

We had a good christmas with both of our families visiting. This was the 1st year that the boys weren't up before daylight opening Father Christmas' presents (they probably twigged several years ago). We had a nice afternoon with my family, and played a couple of games in the evening before watching Dr Who. The school holidays have dragged . I could sight the atrocious weather as a factor, claiming the boys would have otherwise been going out on their bikes or playing football in the park. But in reality I doubt if any of them noticed the rain or wind because like most 12 year old boys, they are too busy worshiping Xbox, the god of gaming.

New Year's Eve was the usual affair of watching Top 50 whatever programmes before watching the fireworks. I managed a tiny alcoholic tipple courtesy of an oral syringe, the 1st drink in 3 years !!

So with that, I wish you a very happy new year

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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 

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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,

MND ain't all bad!!

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

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