What is MND/ALS?

What is MND/ALS?
MND/ALS is characterised by progressive degeneration of the motor nerve cells in the brain and spinal cord. The motor cells (neurones) control the muscles that enable us to move around, speak, breathe, and swallow. With no nerves to activate them, muscles gradually weaken and waste. Symptoms may include muscle weakness and paralysis, as well as impaired speaking, swallowing, and breathing. In most cases, it does not affect intellect, memory or the senses. Progress is relentless and generally rapid, with a life expectancy of between 2 and 5 years from the onset of symptoms. There is no known cause and there is no known cure. See links on the right for further information.

Saturday, 2 July 2016

The Realities of MND - Two Years

Two years ago today, on the 2nd July 2014, my life was changed forever. I knew what my neurologist at Royal North Shore Hospital, Sydney was going to say to me following nine months of Nerve Conduction Tests, Transcranial Magnetic Stimulation (TMS), Electromyography (EMG), Magnetic Resonance Imaging (MRI) scans,  multiple blood tests and 3 weeks on Intravenous Immunoglobulin (iViG) therapy. Months prior I had informed my neurologist of my Google self diagnosis so it wasn't so much of a shock for me when she confirmed Motor Neurone Disease was the cause for the twitching and spasms in my arm and leg muscles and also the cause of the muscle loss between my right thumb and index finger. Apparently my MND was slowly progressing so my prognosis was 15-20 years which was comforting news however my research had not brought up any information on slowly progressive MND. My doubts were confirmed a month later when I sought a second opinion from Professor Matthew Kiernan's team at the Brain & Mind Institute (University of Sydney).

The month following my diagnosis was one of the most challenging times of my life. Not only did I have to deal with a terminal diagnosis but I also started a new job, my relationship broke down after 4 years (albeit amicably & unrelated to my diagnosis) and I had to move house! Just one of these events would be enough for most people to handle. However nothing could have prepared me for the further challenges that lay ahead.

As some background info, I first visited Australia in December 2007 and instantly fell in love with the country. Everything was so accessible. Half an hour of city centres you could be at one of many beaches or remote national parks. The weather was definitely a plus too! Within nine months I had moved to Sydney at the age of 30, the final age to enter Australia on a Working Holiday Visa. In retrospect I should have made the most of that opportunity to gain employer sponsorship or find a partner but unfortunately due to family reasons I returned to the UK in the middle of 2009 but my love affair with Australia continued.

NYE December 2007

Over the next four years I continued working in the travel industry in London and managed to get Down Under five times on various work and personal trips. In 2013 my partner had a work opportunity come up in Sydney and so off we both went to live the dream in Australia on a four year work visa sponsored by his employer, which we both knew we would want to convert to permanent residency followed eventually by citizenship. Sadly for me, thanks to MND,  that dream was not to be. I first noticed muscular twitches in June 2013 and saw my doctor the week before we moved to Sydney.  She was concerned and wanted me to have some blood tests done however in the excitement of moving to the other side of the world I didn't bother.

So it is now two years later following my diagnosis and I am a completely different person from the guy who received devastating news but took it on the chin, thought positive and went straight back to work after the appointment. I wasn't in denial,  I never have been in denial of my illness or my mortality  but I have always been a true believer of the power of positive thinking and living in hope. I think over the past three to six months as my condition has deteriorated I have begun to however disbelieve both of those ideologies as simple wishful thinking.

By late 2014,  I was  living in flat share near Coogee Beach in Sydney. My doctor had prescribed Riluzole, the only drug available for MND. Apparently it can give you up to three months extra at the end! Wow! Ultimately I later decided I probably wouldn't want 3 months extra at the end!! I was also prescribe Baclofen to relax my twitching and spasming muscles, but new symptoms started to rear their ugly face.  MND New South Wales and the Brain & Mind Institute were an invaluable source of support and information.  The latter providing me with regular check ups, referrals, dietary advice & orthoses as and when required during my time in Sydney.  In return I volunteered to be a guinea pig to assist in their research into neurological conditions.

I made a conscious decision to stay in Australia for as long as I could as I knew that I would reluctantly have to return to the UK at some point,  leaving my dream behind. However this just turned out to be one of a multitude of losses.  Between diagnosis in July 2014 and June 2015,  when I eventually returned to the UK,  I had dropped from 74kg to 48kg.  I was unable to eat solid food and lived on soups, mashed potato and porridge!  I used to love Thai and Indian food so this was a major downgrade due to losing the ability to swallow.

Kata Tjuta 2015

My voice had also begun to weaken which became increasingly frustrating when people struggled to understand my mumblings. My life long lisp was bad enough!! It was also embarrassing. I wondered if my speech issues were caused by Baclofen affecting my tongue muscle, so I stopped taking it.  My right hand had begun to claw and I was no longer able to write, being right handed. My left hand had also begun to lose muscle between the thumb and index finger. My right leg had developed foot drop and I had begun to walk with a limp. After finishing work and medically retiring at the end of March 2015, I spent the following three months travelling around Australia seeing places left on my bucket list (Fraser Island, Noosa,  Ayers Rock, Darwin & Kakadu National Park) generously funded by ex-partner, now best friend. I knew it would be my last time in Australia, the country I had become so fond of, so I wanted to make the most of my remaining time there.

Once I had returned to the UK, I moved in with my Mum in a little Yorkshire village, worlds away from Sydney CBD and Sydneys beautiful beaches!  My Mum however was ready for battle to get the multidisciplinary care team together. By the end of August I had had a PEG feeding tube 'installed', I spent ten days in Kirkwood Hospice to get into the routine of using the PEG and also a newly introduced Non Invasive Ventilation (NIV) / Nippy machine. Once home I had a hospital bed delivered and care package commenced. This involved a carer coming in to shower and dress me everyday, which was increasingly becoming difficult for me to do, yet another loss. District nurses would also visit daily to administer my medication. In September I had a Quickie Salsa M2 powered wheelchair delivered however living in a town house I could only use it outdoors, we were on a list for accessible housing though which we eventually moved into a week before Christmas!

One positive was meeting my childhood heroes 'Five Star' at an 80's festival in July and having 'Rain or Shine' dedicated to me! Not one to be defeated, I also managed to see Madonna on her Rebel Heart tour once in Manchester and twice in Berlin! Accessible seating in Berlin was easy to arrange including a backstage tour with one of her (gorgeous) dancers, not so easy in Manchester which only materialised following an appeal for help on Twitter! Easyjets wheelchair assistance was fantastic too

By the end of 2015,  I was finally wheelchair bound as both legs had weakened and we took delivery of a wheelchair accessible vehicle. I had also admitted defeat and stopped trying to speak as any sound that came out of my mouth was unintelligible to everybody but me so I decided to get to know my EyeGaze machine thoroughly as it would now communicate for me for the rest of my life.

The worst thing about MND is that it is like running after a bus, you can never keep up. What I have to deal with on a daily basis is communication issues, the EyeGaze isnt perfect and needs recalibrating everytime lighting changes.  It also isn't the fastest machine in the world so takes patient listeners otherwise I can be two or three conversations behind.  That said,  I am grateful to be able to communicate when many others before me didn't have the opportunity.

Breathing has become a major issue. I now wear my ventilation mask 24 hours a day. I struggle for breath within 30 seconds of the mask being off. I sometimes wonder had I staggered the use of the mask rather than rely on it so heavily so soon, maybe my breathing would have been a lot better today because essentially my NIV is now a life support machine as due to muscle loss my diaphragm can no longer lift my lungs.

Mobility is the next issue. Both of my hands are now clawed and arms 99% useless from the elbow down. Imagine having an itch you can't scratch, daily torture for me!  I cant type anymore so use my MacBook remotely via TeamViewer on my EyeGaze. Both of my feet are no longer able to provide balance to my body anymore, and to add to that my neck has weakened to the point where I can no longer hold my head up unless in a reclined position. When I lay in bed I can virtually no longer move without assistance and I am reluctantly having a ceiling tracking hoist installed in the coming weeks. After three falling incidents, one where i was out cold on the floor until my personal assistant turned up and found me, the hoist can't come soon enough, as much as I don't want it !!

The other issue is getting to the loo, I now need help to and from the bathroom and with pulling down / up my trousers which is completely humiliating. Lucky I have an electric toilet seat funded by the MND Association which does all the cleaning!!!! Even more indignifying is when I'm alone and the laxatives i take to promote movement in failed bowel muscles kick in. Shit happens! And for my poor carers that has happened four times in the past six weeks!

And then there is the taboo subject nobody likes to talk about! Skip this paragraph if you are sensitive to relationships and sex!  Do I automatically become a virgin again after sixteen months of absolutely zero intimacy with another person?  I frequently have a browse on Grindr or Tinder but never message or reply to anybody because what do I have to offer?  I can't exactly go out and meet people in bars or clubs and it's not as though I have privacy or the physical ability to go on dates! Its hard knowing that I will never get the opportunity to fall in love again or have companionship, a partner to cuddle up with in front of the TV or fall asleep with my arm around in bed. I had read a beautiful article about disability and intimacy on Twitter a few months ago,  but the direction of the story isn't for me. Check it out here: http://goo.gl/sH0SDA

It does make me laugh when people call me inspirational or brave etc! I'm none of those things, I'm just a normal guy who wants to share his stories and experiences of MND and trying to stay around as long as I can however I'm not so naive to think I will make my 40th birthday target in January 2018! But that said I am grateful of the time I have with family and friends which is a whole lot more than everyday innocent victims of terrorism get, Orlando & Jo Cox come to mind. So for now I will keep on fighting, adapting and sharing my experiences until MND, well, you know!

Its not all doom and gloom though, thanks to my PEG feed I'm up to 62kg, I have a fantastic care team and a legendary mother to fight my every corner, some amazing friends and wonderful family who visit as often as they can. Still going to concerts,  Mum & I saw Coldplay in Manchester last month, we also have a family only holiday in the UK to look forward to in August, then my ex-partner / best mate that I 'speak' with almost daily on FaceTime is visiting from Sydney in October. Hoping to also visit Edinburgh having never been to Scotland.  Good times!

My biggest frustration and regret is not having Motor Neurone Disease but having to leave Australia, somewhere I have always felt comfortable, relaxed and 'at home'. It is for that reason, when the time comes, I would like my ashes scattered from the boardwalk below Waverley Cemetery on the beautiful Coogee to Bondi coastal walk in Sydney during sunrise to finally achieve my dream of settling in Australia.