When is a chair not a chair?

This chair costs a mere £25,000

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

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/

 

Matthew Barrett’s life in pictures – early days

This gallery contains 6 photos.

The search for a cure

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.

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

Then on to Downing Street for a TV interview

Whatever happened to 2015

Well, I don’t seem to have touched this blog for a whole year, but now we have something particular to campaign for, it’s all systems go. Please read about Matthew’s campaign for a new wheelchair, and I’ll do a summary of 2015 as soon as I can.

Duchenne awareness month – 2014

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

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!

Jannette, Donna and Adam – with Pudsey

Tuesday 9th July

A busy day, but enjoyable. First we went to Matthew’s sports day, at Crownbridge School. It was great to see children of all abilities taking part and enjoying the races – it really was the taking part that mattered, rather than winning, although the wins were celebrated too.

Mr Scales and all the staff made sure that everyone got a chance to take part, and supported the ones that needed a bit of extra help or encouragement. Matthew? Well, he was disappointed not to do well in his first race – he was the only one in a power chair so he should have romped home, but it seems that someone had regulated his speed! He did better in his second race, though I’m not sure if he came in first, second or third! He was much happier though, and then it was off to Cardiff to lobby the Welsh Assembly.

We missed the gathering on the steps of the Senedd, but were in time to hear about the efforts of
practitioners in Wales to develop a more integrated service, and were of course particularly interested to hear about the problems and solutions for those undergoing transition, and to be reassured that Matthew is still part of Action Duchenne’s Takin’ Charge project, and will be able to fully participate next year, when some of the activities will focus on music technology.

Matthew’s transition has been complicated by his mother, who at first was agreeable to him going to live with Dad, which is what he said he wanted to do, then decided to fight the move in the courts, where, amazingly, in spite of all the evidence to the contrary, it was decided that he would face too much disruption if he moved schools and lost contact with the carers who go in to put him to bed (the same carers who dislocated his hip by moving him by his legs). Remember, this is a 17-year-old lad who will have to face change when he reaches the age of 19 and will have to leave Crownbridge.

Takin’ Charge will be so helpful to him. He has started to speak up for himself so much more – incredibly, it all seemed to start when he had his tonsils out and evidently found it easier to speak! Since then there has been no stopping him at times. He is very clear when speaking to friends that he intends to live with his Dad as soon as possible, but clearly he doesn’t want to upset his mum so he becomes less assertive in that environment, leading some people to think that he has changed his mind.

We are reliably informed that once he reaches the age of 18 (just a few more weeks) he will be free to choose where he lives and what he does with his life, so look out girls in Weston Super Mare, Matthew is on his way!

What it is like…

What it is like to be Matthew’s dad

I have to be on call for Matthew 24 hours a day. For example, if he puts his arms over his head when he is asleep, he can’t put them back straight by himself, so he will call me during the night to ask me to help him. He often needs to call me to help with going to the loo.

In the morning, it takes about two hours to get Matthew washed, dressed and to have his breakfast. All meals take about an hour. He can still feed himself, but sometimes needs help because his arms and hands get tired. He has a neater-eater but wants to be independent for as long as possible.

Whenever Matthew is visiting we have to anticipate his needs – for example we make sure everything is put away to make room for his wheelchair. Visits have to be well-planned. When we go out for a meal, we don’t check out the menu first – we have to choose somewhere that is wheelchair friendly, and we can only go to see films if they are on in an accessible cinema. There are lots of shops that we can’t get into, especially the small local shops, but I don’t like to leave Matthew outside on his own. Although he is 14 he is vulnerable because of his condition.

Getting around is difficult. We often have to go quite a long way out of our way to find a dropped kerb that the wheelchair can cope with. Going anywhere by public transport is practically impossible – he can’t get onto coaches, there are hardly any taxis that will take a powered chair, and although (with planning) he could travel by train, not everywhere we want to go is near a railway station.
So, living with Matthew is complicated and takes a lot of forward planning, but underlying all the practical difficulties is the knowledge that we are watching him die. His deterioration is so gradual that you don’t notice it day by day, but if you compare what he can do now with what he could do six months ago, or a few years ago, it really hits you hard.

Although watching him deteriorate, and knowing what the eventual outcome will be, is heartbreaking, I have to hide all the pain from Matthew, and carry on as normal. I feel that it is my responsibility to make sure that Matthew does as many different things as possible while he is still able to do them. This is why I try to raise money to take him on holiday and take him on days out, but unfortunately I also have to ask for money to help me with Matthew’s day-to-day needs. This is partly because Matthew has two homes, and his disability living allowance goes to pay for the things he needs at his mum’s, but not for what I need to have him to stay here, but is also because he needs help over and above what the authorities will pay for (his Neater-eater, for example). I am determined that Matthew will be able to visit me just as any able-bodied son would be able to. Even though I am not with his mum any more, I am still Matthew’s dad, and if I was unable to have him to stay with me I would feel that I had abandoned him.

Matthew compares the effects of his condition with a rechargeable battery. When he goes to bed, he recharges and has strength in his muscles. As the day goes on, he loses that strength, but the trouble with rechargeable batteries is that over time they get so that they won’t charge up fully, and that is what is happening to Matthew. Now he is finding that he is stiff in a morning when he wakes up – he has even started asking for his physiotherapy. I guess what he needs is new batteries, or to put it another way, he needs a treatment that would stop him deteriorating further – and he needs it now!

I am angry that Matthew is having to suffer for something that is not his fault, that he hasn’t brought upon himself, but there doesn’t seem to be enough money to help him fight and get through it, but other people, such as drug addicts and criminals, get the benefit of all sorts of treatments and rehabilitation, when they really have had the choice about how their lives have gone. If they could live in Matthew’s shoes for a short time, they would think twice about the abuse they are subjecting their own bodies to.

Matthew and I have been involved in campaigning for Action Duchenne for a number of years now. We have had some successes – being talked about in Parliament, getting a grant from the Department of Health to pay for early trials for the MDEX (exon-skipping) treatment and just getting people to know about Duchenne muscular dystrophy and what it involves. We have also been overwhelmed by the level of support we have received from people we have met – MPs, people wanting to do sponsored walks and marathons, musicians, a traffic warden with a villa in Florida, a social club in Wales, not to mention all the people we have met when we have staged events or have been collecting at the Motorway services. The trouble is that even though we have so much support and good will, it is not enough to ensure that we can beat this disease before it is too late for Matthew. He is already three quarters of the way through his life, and I reckon he has already lost three quarters of his muscle function.

I found this on my computer when I was looking for someone else.  I hope it goes some way to helping you to understand what it is like to have a Duchenne boy in the family.

Duchenne Awareness Month – June

The diagnosis of Duchenne Muscular Dystrophy is a life-changing event. It would be so easy to react by turning inwards, despairing about the future, and concentrating on giving their child the best possible life, while trying to prepare for his or her early death.

Heaven knows, there are enough hard decisions and battles to fight when you have a child with any disability, that there sometimes isn’t enough energy left over for worrying about the rest of the world.

Mercifully, many Duchenne parents have not been content to just sit back and wait for their child to die, and a number of organisations have been created by parents, intent on finding a cure, not just for the potential benefit of their own child, but for generations as yet unborn.

The common aims of all these organisations unite them in the Duchenne Alliance – an international organisation that recognises the individual aims of each group, and supports them in the shared objectives of finding a cure for Duchenne and providing a better life for those that have the disease in the meantime.

The Duchenne Alliance have declared June to be Duchenne Awareness month. Please visit their website and thier Facebook page, where you can read about the courage of some of those living with Duchenne and those who have lost their sons and daughters to the disease but still fight for the cure that will save future generations.