The story of david
I am a sixty-six year-old male born in Scotland who has lived with back problems since 1973. While holidaying in Oregon in mid 1986, I went white water rafting on the Rogue River on an inner tube. Unfortunately I was thrown off and hurt my spine. My legs were paralyzed for a period of about twenty minutes. I still managed by the grace of God to get down the rest of the river using my upper body only.
I stretched in bed in late 1987, and unbearable pain traveled up my spine to my head. This resulted in a period of six weeks of being unable to work. I was sent to Bridge of Earn Hospital in Scotland for treatment. I was lying in agony on the floor they were useless; one nurse walked straight passed. Thanks to a brilliant Sports Medicine Chiropractor from Dundee, I finally got back on my feet.
We moved to Australia in 1988. I had motor vehicle accident in 1989 that again exacerbated my back problem. In 1995, I was given a spinal manipulation and fitted with upper body brace. I completed a program at RPA Pain Management Clinic. The year of 1997 brought with it a Bilateral Facet Block at RPA Hospital, which was unsuccessful.
In 1999 I was diagnosed with severe disc degeneration at L2-3. I was fitted with a Poly Spinal Jacket made to my exact measurements. The process involved being put in a plaster cast, which was very scary. By this point, doctors had ordered multiple studies, including-ray films; CT Scans; Discograms; bone scans; MRIs; blood tests, and psychological tests.
From this period forward, my mobility went downhill. During a period when I was doing consultancy work for a large distribution company, I was unable to lift my legs to climb ladders. This resulted in me being unable to work, and in 2000, I was placed on a Disability Support Pension. The Centrelink doctor was astounded I had been able to work that long.
My journey with Stiff Person Syndrome began around 1997. At age fifty, the disease manifested itself by my lack of ability to move. I was only able to move my head when lying on the floor; something I frequently did to relieve back pain. It was very alarming. I mentioned this to my excellent physiotherapist during my treatment. She then arranged for me to see a specialist at Royal North Shore Hospital. What a waste of time was. While driving my car, I would often lose the ability to move my legs unless I lifted them with my hands. (While breaking or accelerating.) This got progressively worse through time. I knew something was wrong.
By October 2004, I was unable to move my legs while lying in bed at night, and had terrifyingly severe tremors. An ambulance was called, and I was hospitalized. During my time in hospital, I was found to be severely B12 deficient. I was also suffering from Sub-acute Combined Degeneration of the Spine. This was diagnosed by a myriad of tests, including standing upright with legs together, and closing your eyes. I just fell over. When sitting on the edge of bed with the bed arms folded, I would try and get up. I could not.
I was then started on a monthly regime of intra muscular injections. All was good for about 3 months, when the stiff muscles, ataxia cramps, loss of various movements, edema, etc. started. My Neurologist was just not listening to my concerns, and increased the injections to fortnightly. He had diagnosed my condition; end of story. We were not impressed.
I was then put on a weekly exercise program at the Rehab department of my local hospital. I was told that I was not allowed to drive by umpteen doctors. I was put on Baclofen, and did not tolerate it well. I started to get on with my life. I bought myself a mobility scooter to get around on. Then I started to have intermittent paralysis of legs, arms, and hands. I could not eat a meal without the arm paralysis happening, so I was unable to go anywhere without a caregiver. In most cases my wife took that role. I was not allowed to use my scooter any longer.
We decided to ask for a second opinion in early 2007, and went to a Neurologist recommended by my GP. There, I underwent further tests including: MRI, SSEP (evoked potentials), nerve conduction tests, EMG, and specialized blood tests. The results were all normal. The doctors said “It’s all in your head.” My own GP, and the people who saw me at Rehab, did not agree with this diagnosis. I had further nerve conduction tests that too were normal.
The year of 2008 enabled me to again control my situation. I was scripted by Paraquad and Camden Hospital for a powered chair complete with captain’s chair, tilt mechanism, and chin control. It was so good to have regained my independence once again. I nicknamed it “THE BEAST”, as it is very large.
About late 2009, I was beginning to have upper body seizure episodes in bed. The seizures were sometimes quite violent. They would be anywhere in number from 1 – 8 times a day, and had different levels of strength and duration. My legs would also get incredibly tight and very painful during the later part of the day.
My condition began to improve in regards to my arms and hands. As soon as I did any exercise, especially in the gym or walking, my legs become very heavy and painful. The paralysis episodes would occur, or my legs just collapsed below me. I was then having physiotherapy every 2 weeks at my local hospital through late 2012. I am now 65 and no longer meet the criteria for funding.
During 2011 – 2012, I then started having very severe upper body seizures this would result in my body being propelled backwards at speed. Anything I was carrying would go flying. You should see the roof in my lounge where the TV controller has hit it! The seizures happened up to six times a day at one point. Eventually, the seizures slowed down to five to six times a week.
With prompting from my lung specialist, we decided to search for a new neurologist who specialized in movement disorders. I searched the internet and found my current neurologist. She was on the Periodic Paralysis International Medical Advisory Council. I sent her an email and overtime she responded to it.
In November of 2012, I took delivery of a new manual wheelchair. This chair was much improved on the old one. It much lighter easier for my wife to handle, and it fits nicely onto our new wheelchair rack on the rear of the car. In December 2012, the real journey of discovery commenced after a physical examination. My Neurologist thought I had a variation of Stiff Person Syndrome and at least two other neurological deficits. Blood tests were unremarkable. I had further EMG testing done with needles in my hands, thighs, eyelids and next to my spine. These results were also unremarkable. I also had a full spine and brain MRI. In the meantime, she started me on Gabapentin as I could not tolerate Baclofen or Valium.
About 3 months later, I then started having very severe leg spasms whole leg and feet twisting violently inward. This resulted in the muscles pulling all the way to my hip, which was very painful. I started hydrotherapy on weekly basis with constant supervision. During this time, my condition became much worse. My legs, hands, arms, and feet would invert to the point of agony as soon as they were unsupported.
Then in late June, I presented to my local hospital in terrible agony. I had the presence of mind to ask them to contact my neurologist. Although on leave, she arranged for me to be transferred to one of the top hospitals in Australia. I was placed under the Professor of Neurology. Then I was put through so many tests; ultrasounds, CT scans, and further needle nerve tests. The test results picked up the Neuromyotonia. They prescribed Clonezapam, which was then changed to 3600mg Gabapentin and 75mg Dantrolene. During this time I was in a very deep depression. I was shell shocked and did not know what was going on. Every time I tried to walk, my legs collapsed below me. I had full body paralysis while lying in bed. It was very frightening. I also had upper body seizures, which caused me to throw things everywhere. Eventually, through medication and my own ability to better control the spasms, things started to get better. The geniuses at the hospital would still not give me a definitive diagnosis. I lay between Stiff Person Syndrome and Isaacs’ Syndrome. Or possibly a syndrome called after me. Stiff Person Syndrome was the most likely. After 3 weeks, I was transferred back to my local hospital to begin extensive and exhaustive rehabilitation.
During the 5 weeks I spent there I had to learn to walk again; something we take for granted. This was incredibly hard as you have to retrain the brain again. The hospital also gave me access to a psychologist. This, along with the visits from the pastoral care worker, (who actually was a friend from my Church) brought me out of my deep depression. During these sessions I really opened up and laid all things bare.
I was then allowed to return home, but was still having to use my walker or wheelchair. I had to have services put in place to shower me, etc. This was tough for me as I am a very independent person, and stubborn as a mule. Now private physiotherapy is ongoing, and has helped me immensely to get fitter and stronger. It took me six months to be able to walk confidently without any aid, and to walk up some stairs. I am not allowed to walk outside for fear of seizures or falling. I still must have a caregiver with me at all times. My beloved wife usually fulfils that demanding role.
I have since seen my neurologist again, and we are greatly comforted that any future episodes that require hospitalization will be at St Vincent’s and nowhere else. I will also be under her care. The seizures and stiff legs are a constant reminder that I have to take it easy. I have to stay safe, and not put myself at risk. Outside, I have to be in my wheelchair at all times. The other day I was sitting in the pub having lunch when I had a seizure. It was so violent that it thrust me backwards. I overbalanced in my wheelchair, my knees hit the table, and I was saved from serious injury by my wife and a patron grabbing hold of my chair. It was very frightening and timely reminder to stay safe.
My medications were changed in December from Gabapentin to Lyrica to help better control my seizures, and to date this has been fairly successful. Although I have concentrated on back problems, SPS, and Isaacs’ Syndrome, I also have osteoporosis, osteo- arthritis, type 2 diabetes (diet controlled), asthma, biapical pleural plaques, tachycardia, gastric reflux and depression. All require medication and ongoing care and intervention.
My disabilities do not define me, and have in many ways been a blessing. It has allowed me to be a very significant part of my grandchildren’s lives, to volunteer in many capacities, and to advocate for others with disabilities and their caregivers. Caregivers so often the unseen and unsung heroes of our society. I also have a strong faith that, with the love and care of my wife, is what keeps me going in our daily struggles.