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Sylvie Nicholls, Roy’s wife of 27 years, shares her story of her life with husband Roy, who was diagnosed with Adult Onset SMA in 1996.

At the age of 28 Roy first encountered SMA, and for the following 28 years dealt with the continuous changes this condition brought him. He had late Onset Type 3 SMA which took many years and tests to diagnose, so perhaps I should explain.

Roy had a normal childhood with a quiet enthusiasm for life enjoying ice skating, railways and appreciation for detail and precision. As a toddler, I am told he did have a penchant to walk on tiptoe (according to Professor Hughes at Guys) an early indicator of SMA.

I met Roy aged 21 in 1977 when we both volunteered at the Bluebell Railway in Sussex, he as a Guard and Shunter and me in the ticket office. Roy’s role was quite demanding physically but he enjoyed the change to his everyday job as a structural engineer. In 1984 the first signs of SMA appeared with foot drop. Roy’s ability to move swiftly alongside large steam locomotives and carriages was impaired becoming a health & safety problem, so instead he joined me in the Booking Office. Roy also was extremely good at model railways, and his layout modelled on the Bluebell could be viewed at various exhibitions in the south east at this time.

After marrying in 1985 we moved to mid-Wales oblivious to the health decline which was about to unfold. Roy was employed as a Highways Engineer for Powys County Council. This was a wonderful opportunity to roam the remote countryside checking culverts, bridges and other highway structures for defects before drawing up reports. He taught himself computers including CAD (Computer Aided Drawing) which produced top quality reports ready for contracting out the work to the Highways Department.

The early 1990s saw further deterioration in Roy’s lower limbs. Our GP referred Roy to Shrewsbury Hospital, where the consultant sent him to Smethwick for “electric testing” for conductivity of the nerves within the muscles. Roy had never had reflexes in any part of his body and this was now taken note of. It was evident that although there was electrical activity in his feet and above the knees, there was a distinct lack in the calves. Roy was offered splints to support the foot drop and maintain his mobility, but no reason was forthcoming for the cause of the problem. The skeletal sight of Roy’s lower limbs legs was really noticeable and caused concern.

In the quest for diagnosis Roy frequented The Agnes Hunt Hospital at Gobowen, and Coventry Hospital too as well as blood samples sent to the John Radcliff Hospital in Oxford for genetic testing. It was finally in 1996 that a diagnosis of SMA was given. We were told there would be deterioration throughout all muscles in the body, but life expectancy would be normal. Without anyone else in the family having any such condition we were advised the strain Roy had “inherited” could virtually be unique. As Roy said, “I don’t mind being special, but I would rather have something better to be special about”.

As mobility became more impaired this put pressure on at work. He experienced intense tiredness, but still tried to scramble about on all fours as he surveyed river beds and bridges. It became quite dangerous work, as mobile phones in mid wales at this time experienced great difficulties in receiving signals, and Roy needed to be able to contact someone if an accident occurred.

Roy had always been left handed, but as grip started to fail he managed to use his right hand allowing him to be able to make notes whilst surveying. At least back in the office his competence on the computer enabled this aspect to remain intact. For a while the condition plateaued, but towards 1998 upper body strength was noticeably reduced meaning difficulty in getting up if he fell. Regular strength tests showed Roy had severe disabilities and only maintained mobility by utilising alternative muscles whenever possible.

In 1999 Roy decided that employment was no longer viable, but it took three medicals and five months for Powys County Council to accept Roy had to be medically retired. By now he had virtually no strength in his upper torso, and his neck was weak, but somehow the upper legs still maintained some bulk in the muscles to enable Roy to remain on his feet. The local office was keen to maintain Roy’s expertise (especially with computers) and offered to install any access facilities for him to continue employment. The pressure of work and disability made the decision for him, and as it came at a time when myparents were in need of help in Kent we moved from the isolated village in Wales, south again.

Luckily I went straight into a new job whilst Roy got used to being retired at the age of 44. His daily routine to walk to the local paper shop each morning continued for a few months, until he fell into the road one day. Luckily a passing motorist picked him up and returned him home where the neighbour nursed his cuts and bruises until I returned at the end of the day. This became a decision time. Does he buy a wheelchair or continue to walk with a stick? For him this was difficult as it was an admittance of true disability. However suddenly Roy had independence again with the electric wheelchair, no longer did he need to check each step as he moved and he could actually appreciate the scenery again.

In 2004 we took a holiday in Switzerland with the electric wheelchair (which came into two pieces for the flight) enabling Roy to reach the top of mountains with the superior transport system only the Swiss can provide. On return Roy decided to start a model railway layout again but this time base it on the Swiss railway scenery/system which he had experienced.

Further weakening of Roy’s body prevented his use of a normal car and he waited eighteen months before receiving a brilliantly adapted Motability vehicle. He drove from his wheelchair without the usual steering wheel and foot pedals. Instead he had a three pronged device into which he slipped his left arm which just by gentle movement at the wrist steered the vehicle. To his right a simple forwards/backwards lever enabled Roy’s right hand to provide the accelerator and brake. With a half second delay in the steering Roy never went faster than 60 mph to ensure our safety.

After diabetes took hold in 2007 Roy felt really frustrated. He was not fat (just nine & a half stone), but exercise was not an option and his body had little strength to try. During 2010, Roy had two incidents when I came home and found he had been stuck on the toilet for a couple of hours and couldn’t get up. We felt that now I should consider leaving work and ensure our lives together had more value and meaning. So in June 2010 I retired to care of Roy 24/7 and we moved to Herefordshire to be in a rural environment which Roy so enjoyed.

The last two years have been quite turbulent health wise for Roy. It was unsafe to leave him for more than a few hours, and so twice a week I engaged a carer to give me some respite. This was very difficult as neither of us wanted that type of intrusion, but luckily we found someone who filled the role and understood our need for privacy. By now Roy could not make a cuppa and to eat had a mechanical arm which meant he could still feed himself without human intervention, but all other basic needs were provided by me. We bought an adjustable bed, and Social Services provided a Bath Seat which meant I could get Roy easily into the Bath. A shower was out of the question as Roy had no stability and as soon as he was undressed Roy froze. The bath enabled him to maintain warmth as I washed him. In the last year a Bio Bidet was provided by the authorities as the use of toilet paper was impossible for Roy but he retained continence throughout.

In 2011 Roy and I started a disability group in our county called Herefordshire Disability United which has been very successful. Roy designed the website ensuring that it is accessible to those with sight impairments, if they hover the mouse over the words, it reads out the text. This was another time when Roy taught himself a new computer language of html code for the benefit of others. Our group facilitates like minded organisations and support groups to come together for consultations and Open Meetings. We have befriended many including the council where we are known for standing up with a voice for disabled people.

On 26th September Roy drove to his last such Open Meeting, as the following day he was unwell and admitted to Gloucester Hospital with stomach pains. Without any real symptoms cancer had invaded and Roy had a 16mm mass on his liver, a secondary cancer. Roy refused biopsies to find the primary source as the cancer was also seen in parts of the bones as well. Knowing he hadn’t the health to fight, and being that no chemo or radiotherapy could have an impact on his body Roy decided that his life was soon to be over. We spent time discussing the necessary and ensured Roy would see his mother (coming down from Ipswich) one last time, before agreeing that Roy could be placed on the controversial Liverpool Care Pathway.

True to our lives together, we decided together, how the path of medical intervention would pan out. Roy spent ten days on the pathway (instead of the average 36 hours) making his feelings count. He left a message to others saying “I’ve had a good life, but unfortunately I have been dealt with this cruel disease of Liver Cancer, which along with the SMA I can’t beat. I wondered what would happen with this SMA and how things would deteriorate. I thank you all for giving me such a great life”.

During Roy’s last days alive he expressed a Gift Wish to be given in lieu of flowers at his funeral. I also sold the stock of the Swiss model railway collection with his consent, and together this has raised £1,500 for The Jennifer Trust (now SMA UK). I decided to write Roy’s story so that others may see that life can still be worthwhile with SMA if you have the determination to accept the challenge.