When is a chair not a chair?

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meyra nemo

This chair costs a mere £25,000

Picture of the chair that JK Rowling used when writing Harry Potter

This chair sold for £278,000

Or should I say, not just a chair? A wheelchair for Matthew is like back, legs and arms to the rest of us. Due to Duchenne muscular dystrophy, Matthew’s muscles are becoming so weak that not only can he not stand up, he relies on steel rods in his spine to maintain his posture, and he relies on the arm supports fitted to his wheelchair to help him to feed himself, or even just scratch his nose.

Matt has been using a powerchair for several years, and as far as getting about is concerned, what he has at the moment works just fine. But a replacement like the Meyra Nemo would give Matthew some of his independence back.  Because he can’t support himself at all, Matthew needs two carers to help him with his basic needs.  It’s bad enough having to rely on one person to help you go to the loo, but Matthew has got used to that.  Needing two helpers, and the space for them to manoeuvre  him, severely limits the places Matthew can go. So that’s one thing…..

Now imagine spending all day sitting in the same position. Every day. Of course the people looking after him will move him if he asks. but would you want to keep asking someone to tilt you this way or that every so often?  And that is just slight changes in position.  The most beneficial movement for Matthew is for him to spend some time each day in a standing position, and he is one of the few people of his age, with his condition, who can do that, with support.  It is incredibly beneficial, because it allows his internal organs to recover somewhat from being permanently squashed. He does it at the moment with the help of a standing frame, but again he needs two people to help him to do that. This is generally his dad and stepmum, at home.  If he feels like a stretch during the day, when he is at college, that is just his hard luck..

With a chair such as the Nemo, it would be Matthew’s decision when to move to a standing position, so not only would he feel more comfortable, he would also be able to bring himself to full height when talking to his tutors and college friends – a much more equitable situation than at present. Or he could remain seated while talking head to head. The important thing is that he would have a choice. He would also be able to lie down without having to ask to be put to bed.

As the strength of Matthew’s hands and arms deteriorate, it makes sense for him to develop his IT skills.  Matthew is an expert in music technology applications, but it is becoming increasingly difficult for him to use a traditional laptop or PC keyboard.  Wireless technology means that he would be able to link to a computer straight from his chair, and potentially control all sorts of Bluetooth devices through it – great news for a music-lover!

Matt is very fortunate to still be able to manage solid food.  I have just heard of a boy who is not quite nine who is starting to struggle with eating, and it is very common for those in their teens to have had to switch to liquids only, and ultimately tube feeding. Clearly, the ability to move to a standing position will aid Matthew’s digestive processes while he is still able to chew.

But, like so much of Matthew’s life, raising funds for the chair has become a race against time.  If he’s going to get the most out of it, he needs to start using it now.

If you can help, please visit the justgiving page set up by the TurboTrust to help with the purchase:

https://www.justgiving.com/Standing-tall

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Matthew Barrett’s campaigning and fundraising – part 1

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Matthew’s dad, Garry, had been campaigning and fundraising from the moment he got the terrible news about Matthew’s future, I met him in 2001, around the time that a new charity was being set up by Nick Catlin, his wife Janet, and Paul Fitzpatrick – all parents of Duchenne boys.  The charity was based on a similar one in America, set up by Pat Furlong who had lost two sons to the disease, and was initially called Parent Project UK (PPUK).  As time went on, more people with the disease became involved, so it was thought appropriate to remove the emphasis on parents and rename the charity Action Duchenne.

Action Duchenne’s current website can be viewed here  http://www.actionduchenne.org/

 

The search for a cure

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Over the fourteen years since I first met Matt, I’ve been involved in all sorts of campaigns and events where we tried to raise awareness of Duchenne muscular dystrophy and funds to help develop a cure.  In the early days we went to conferences to learn about developments and advances in treatments. We kept being told that a cure or effective treatment was just around the corner, and if only we could raise enough money for the clinical trials, there was a good chance that there would be a drug or combination of drugs that would halt or slow down the progress of this heartbreaking killer.

I specifically remember that in 2007, we were told that there would be a good chance of an effective treatment in 5 years time.  That would have been 2012.  In 2009, Professor Dame Kay Davies, a leading researcher into one type of treatment for Duchenne, said the same thing. That would have been 2014.

In a way, Kay Davies was right.  There was an effective treatment (Translarna) which was identified in 2014 and approved for preseiption in many European countries, but it took until April 2016, after much lobbying and campaigning by the Muscular Dystrophy charities, before NICE finally agreed to recommend the treatment for people with Duchenne. Read the BBC news story here.

This is a huge victory for the Duchenne community, but tinged with sadness for many, as only 13% of people with the disease in England and Wales will be eligible to receive it -those who are over 5, ambulant, and whose DMD is caused by a nonsense mutation.

Matthew is ineligible on two counts – he is no longer ambulant and his DMD is caused by a missing gene (deletion) rather than a nonsense mutaion. You can read about the different types of mutation here: USNational Library of Medicine

We are still supporting the search for a cure, of course, but for Matthew at the age of 21, his family and friends are focusing on ways to improve and prolong his quality of life.

Department of Health 2014

Campaigning whatever the weather – outside the Department of Health in 2014

Downing Street 2014

Then on to Downing Street for a TV interview

Duchenne awareness month – 2014

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Our second event this month was attendance at the Muscular Dystrophy Campaign’s meetings at the Vassall Centre, Fishponds. The first meeting was a comprehensive update on current research projects for Duchenne. Neil Bennett, Research Communications Officer for the Muscular Dystrophy Campaign, explained about the Duchenne Research Breakthrough Fund which has target of £2.2M for 2016/7 specifically targeted for research into Duchenne. Supporters can set up their own campaign towards the fund, naming a specific individual who has inspired the campaign.

As well as finding specific treatments for Duchenne, MDC are committed to working towards making those treatments available to people in a timely manner.

Neil explained that the decision about whose research projects are funded is made by a committee at MDC consisting of himself, the Grants Manager and the Director of Research, informed by input from stakeholders including patients.

The MDC supports communication between scientists, specifically through the European Neuromuscular Centre which published the standards of care for Duchenne Muscular Dystrophy in 2010.

Neil outlined the ways in which MDC disseminates information about research, citing Target research and the weekly web-based news service which can be accessed here: http://www.muscular-dystrophy.org/research/news<//

Neil then ran through a list of the most promising treatments on the horizon at the moment – Translarna (formerly known as ataluren) which has been granted conditional approval by the European Medicines Agency and is therefore the most promising hope for people whose DMD is caused by a nonsense mutation. FAQs about Translarna, including an explanation about the differences between the various causes of DMD – nonsense mutation, deletion etc – can be found here http://www.muscular-dystrophy.org/research/news/7593_translarna_faq

He spoke about the two front runners in the exon-skipping treatments from Prosensa and Sarepta, Summit’s utrophin upregulation (which would benefit all regardless of cause of DMD) and some other projects which are being part-funded by the Duchenne Research Breakthrough Fund – for details see http://www.muscular-dystrophy.org/research/grants/current_grants

Neil stressed the importance of having administrative support to run clinical trials and the enormous cost of conducting meaningful trials.

St Matthias Library staff bake for Children in Need

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Here at St Matthias Library, University of the West of England, we’ve gone a bit crazy about baking. Our biscuit bonanza was rapidly followed by a Jubilee cupcake challenge, then we watched Paul Hollywood on the Great British Bake-off and thought we’d try our hands at fancy breads for ‘Matthew Kneads Your Dough’to pay for continuing hydroptherapy sessions.

After so much practice, we were ready to set up a stall and sell our wares, so for Children in Need we decided to make some cakes, biscuits and bread to sell. Rather than putting a value on our efforts, we just asked for donations, and raised a whopping £92.00. We certainly had lots of variety, catering for every taste from vegan to gluten-free, and a couple of us tried our hands at Pudsey cakes – harder than they look!

Encouraged by this year’s rather low-key effort, some of us are planning to go all out next year, and expand our range into preserves and craft items. Watch this space!

IMG_1121All sorts of cake2

Jannette, Donna and Adam - with Pudsey

Jannette, Donna and Adam – with Pudsey

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