making muscle wasting history

Up until I was a teenager of 16, DMD, Duchenne's was a term that had little meaning to me. It was a word to describe why my muscles were weak. It had no boogieman qualities to me, just something that helped me explain to friends (it wasn't as if it was discussed every minute). I knew I'd be in a wheelchair when I began stumbling and bumbling at around 7. It was a huge shock to me naturally but the event doesn't bring up any lasting pain for me. I took in my stride (no pun intended, but you may chuckle lol) I mean I cried and I dispaired but very quickly I just got on with life, if anything it was a relief not to injure myself falling over, once you bite your tongue a few times it loses its charms if indeed it had any.
So again I settled in to a new routine (in my life I've had more new routines than a dance troupe), felt pretty 'usual' and still never worried I was getting worse, I knew it would but I really never thought about it.

I never noticed a decline in breathing or thought much of my arms slowly getting worse, I simply wasn't getting checked up by any doctors so I thought well thats that, everythings fine. Wheres my teenage mutant hero turtles video mum? I would say and 'Oh I can't wait to get that game!' Also I enjoyed school, learning, being smart and being thoughtful.

This happy existance was all I could see, the word 'progressive' flew by, but I didn't think that meant me getting worse, perhaps I thought it similiar to prog rock but who knows (by the way I like prog rock band porcupine tree but I'm drifting here). When I saw older guys in fancy wheelchairs I was more facinated than worried. My mind was just refusing to connect A to B.

I was brought to earth in a monumental way shall we say. My Duchenne's moment of realisation came. In the year of our Lord 2000, I passed my GCSE's woah, go me! I got 11 A - D grades. September comes it's A/S level time and boy what a shock! I found it almost inconceivably difficult to get my head around it. Geography required us to go on 'expeditions' they laughably call field trips, asking students to wade into rivers neck deep, I mean I haven't got the yellow submarine wheelchair so invariably Geography had to be dropped. Biology quickly followed the amount of work was in tidal wave proportions, I couldn't in any practical sense use the library and write handwritten notes essays lectures books tutorials flow diagrams and make coffee. It was too much for my hands, I had my carer as a scribe but they would need to come to library with me but you simply can't interfere with their coffee break time, it's simply more important lol I have no animosity there just wasn't time to do everything. So now just chemistry and business studies remained.

Chemistry was so confusing because the teacher says 'you know all that GCSE stuff you did for the last 4 years, it was all wrong, it was the made up walt disney type of chemistry, it's different now' (I added the walt disney but thats the gist) My headached, atoms no longer behaved themselves with neat concentric circles of electrons, now it was p and s patterns and a fizzy mush of electrons doing who knows what jumping from energy shell to another totally at random. As a test that quickly came upon us in 2001 was failed by me in truly epic style even by my standards. My first 'U' I thought yes 'U r thick' it means, but no it means 'unclassified', so bad my name never even earnt a mark. Feeling rather embrassed I departed for break but was called into the SENco's office for a 'it appears you are struggling with Chemistry' talk, evidently I was, but I just wanted to disappear into an electron cloud and cry for 30 seconds. I held it together however this was a 3 mile island not a chenobyl, It was suggested and implemented in ooo about an afternoon that I drop chemistry. No complaints here.
Ah business studies I kept her going somehow for a few weeks but as if too annoy me more I caught a pesky cold.

Wake up time was approaching. This cold just wouldn't go it constanly got worse and worse, my breathing became attrocius and my lungs full of muck, but I thought won't be long before it improves.

It never did, for two days I hadn't slept, my chest was on fire, the pain was immense and I was shattered. Day three it got worse delirium started setting in, I mananged to eat one chip (fries), but I was desperate for the cold to get better. On the fourth day without any sleep my dad just couldn't stand to see me this bad, he phoned a retired muscle consultant I used to see 5 years before until he retired and wasn't replaced ever. He suggested going to a hospital in Cardiff called Llandough (Lan-doc). So after a twenty mile drive with me swathed in blankets shivering but boiling hot we made it.

So in I go and am bungled into a bed via a hoist and sling that bruise my body. Told off for coughing as if you can stop on command. While being poked and prodded and generally messed about a doctor told my folks i may need a mini tracheotomy to clear my chest if I got no better.
In the afternoon tired and hurting a nurse comes in with a suction catheter to shove up my nose to clear my chest. Well its disgusting feeling a tube slide its way up your nose and 'around' the back of your sinuses with mucus being moved, ewww, then the tube is shoved near your lungs. Suction was engaged and muck started moving but didn't get far at all. It appeared not to work so the nurse slid the tube out the way it came.

I felt ok, thinking well that was pointless. Five minutes later in front of mum, dad and my uncle, a huge clump of muck moved and covered my entire trachea, air couldn't get in and I blacked out thinking well that is that for me. Now I can't remember what happened for the next few minutes but my mum has filled in the blanks.

My eyes rolled back and I collapsed falling backwards on the bed, nurses rushed in pressed big red crash alarm sirens and every other doctor and nurse came in. I owe my life to a quick thinking dr called Sian, she immediately cut a small hole in my neck and inserted a mini trach tube so that a suction catheter could clear the blockage. It did but I was still critically ill, so I was rushed into intensive care.

Once there I was put on a full face-masked ventilator called a Nippy 1, (I still have one in my bedroom now), after a few life giving breaths I came to in a foggy mush namely because my glasses were removed. The first dr I saw I mumbled and gargled ' Thank....you....for saving.... my life'. That initial cold turned into bi-lateral bronchopnuemonia wouldn't you know. The dr told me to rest and then I fell asleep for the first time in 4 days. I was in a sleepy haze through a combo of drugs and medications for about a week. I did slowly get better and two weeks later I was home and the trach was gone.
It was a terribly stressful time for me but I thought maybe I could carry on with my life but an ugly set of events came upon me.
You see my lungs and chest muscles had gotten a lot worse and my cough was no where in sight.
So on september 11, 2001 I was rushed into Llandough again with more mucus blocking my throat and again a mini trach was put in with me conscious this time and boy its uncomfortable and the blood well I was plastered in it to say least but hey it worked and my chest was cleared, another few weeks of intensive care and ward stay, home I went again with the trach taken out.

My nightmare year continued when a month later in October my chest yet again filled with muck and back to Llandough I went, entirely fed up and annoyed at seeing hospitals. This time it was decided that my latest mini trach would be a permanent arrangement, which freaked us all out a bit but I just knew it was necessary.
This time I had gas and air for mere minutes while the trach hole was made and the plastic mini trach inserted. About early November I left the hospital for my new mini trached routine.

Nine years later I still have the hole in my neck.

Every month since Nov '01 I have the mini trach tube itself changed for a new one, it's a five minute job, makes me cough and involuntary tears flow for a few seconds. Then its about 10-30 minutes of chest suction involving yet more suction catheter tubes.

I was now fully aware how deadly and how menacingly nasty this Duchenne's was. I lost my will to do anything much at all after that year, somehow through home tuition I got a D in A/S business studies. Slowly I didn't go out was not interested, thought i'd catch more pnuemonia and then thought who would employ me anyway, work just didn't figure as a viable option. I played ps2 every day whilst watching discovery channel and UKTV science channels now called 'Eden' or 'Edam' or whatever it is now until 2005, when my hands packed in and contracted into fist shapes. I moved on to the pc and used a mouse and for the next 3 years played yahoo pool and volunteered with an online pool league hosting tournaments.

I got bored with this and increasingly was too tired to play so I picked up painting. I made a website called duchennemen then was found by Julia Ascott a former employee of Action Duchenne (thank you!) and I finally could help and see and talk to others with DMD and fight back in a way.

After 16/17 I had a very different relationship with DMD, it had and continues to make me ill. I started reading all about it after then, reading symptoms with an 'oh yeah' i've got that feeling. I saw heart troubles and yup you guessed it my hearts affected now. I'm not angry, sad, bitter just numb I guess and very uncertain. Since finding AD I found a sort of purpose and my painting has vastly improved and gives me joy.

Throughtout all of this I've never been intersted in finding a 'cure', obviously I would love a treatment that fully reverses DMD but not at any or all cost. I'm not helping Action Duchenne for me in particular, its when I see younger individuals with DMD I think something should be done for them.

For my older friends with DMD I want us to have better services to give us healthcare and some dignity in life, we're used to DMD we just want a system in place to help us live with it better. So that we don't need to fight for every tiny little thing, like suction machines and ventilator provision even just monitoring our condition to even more basic things like a mini trach tapes. That has to be sorted before we can even contemplate work or anything else.

So my question to the guys over 18 is what was your Duchenne defining moment?

Thanks for reading :)