Sheila Jones: My Life with SPS
I am a 5 feet tall, 8-stone non-smoking lady who eats healthily and used to love walking and take lots of exercise. Early in 2010, when crossing open spaces, my legs felt slightly stiff, particularly when carrying something in each hand. No-one seemed to notice anything amiss so I decided I must be imagining things. However in the July, at a family gathering, my brother asked me if I was all right as I “seemed to be limping or something.” Then, when walking the half mile to Tesco, I started to freeze at the side of the road when attempting to cross. After waiting for it to pass, I went on my way.
Sometime early the following year, I found if I stood still, probably talking to someone, I found I couldn't move - it was like being glued to the floor and I had to ask for assistance. I found out later that this is known as delayed gait instigation. I convinced myself that it was all psychosomatic (l know how real that can be). Then I started falling after going rigid and being unable to grab something or somebody. Only my legs and trunk were affected. One morning I prepared to go out into the garden down the back step. I held on to the door frame but suddenly went into spasm which pulled my hands away from the frame … and down I went, face first on to the concrete path below. My glasses were pushed into my face, hence two black eyes; I sustained a large gash on my jaw and extensive facial bruising. Amazingly, this is the only time l've had to go to A & E.
ln order to get around I had to hold on to something. Even using the bus was difficult. I then started having frequent falls and in September of 2011 decided I needed medical help (perhaps I should say at this point that I was a former medic and my GP had picked up on this on a previous occasion. The GP had no idea what was going on but as I stood up to leave, I went into spasm quite unexpectedly. lt was late in the afternoon and all my symptoms got worse as the day went on. He wagged his finger and said that's not psychosomatic.
My routine blood tests were due so, it was decided to get these done before going further as the GP felt sure they would be normal. The results were normal, but a fortnight later I received a call from the surgery saying he had made an appointment for me to see him and to make sure that I could come. I found out that he had asked for an additional blood test: ANA (anti-nuclear antibody) which had come back as 'suggestive'.
He referred me to a local neurologist, but in the event of no diagnosis would then refer me on to a rheumatologist. The referral to the neurologist came for ten weeks hence, which seemed like an eternity, but things are even worse now. The date for the appointment was for the Friday afternoon before Christmas Eve and I had ten people staying for three nights as all my family live away. Somehow I coped.
I do not believe in putting symptoms into Google and I just knew if I did, it would come up as Parkinson's or MS, but felt I had to do something. I had heard of something called Stiff Man Syndrome and thought that that was exactly what l had, so I did look up that condition now known as Stiff Person because more women than men suffer from it. First of all I checked to see whether it was an auto-immune disease, which it is. Then I looked for more information about it. On the internet support site there was a list of possible symptoms. I was amazed to see that many applied to me. I was pleased to see that there were two specific markers for this disease though not everyone has them: high anti-GAD and specific EMG.
My GP is on good terms with the local Immunologist and had had some more tests done in the meantime; all consultations took place by phone at this point. He rang me to say that all these tests had come back negative. I asked him whether any of these tests were for anti-GAD. His reply was "what's that?” He'd never heard of Stiff Person Syndrome. I then referred him to the SMS website and could hear him tapping away on his computer with sounds of 'oh, oh oh!' as he read the symptoms. Many GPs are against patients doing their own research, or bringing in copies of the Daily Mail, and I told him that this was why I was wary of telling him all this. He said he had no problem with this and was going to get in touch with the immunologist immediately and ring me back.
The immunologist said that my symptoms justified this test and he still had my blood sample so he could proceed immediately. About a week later, and just three days before my neuro appointment, my GP rang to say the result was over 2,000 which was indicative of SPS. lt was such a relief to get a definite diagnosis. He asked me what the treatment was and said he would fax the result through to the neurologist and would issue me with a prescription for diazepam if I so wished. I told him I was reluctant to go on to anything which might compromise the results of any further tests at this stage but if I was desperate I would order one.
The neurologist asked me to go through my history, carried out various tests, but the result of the anti-GAD had not gone into my file at this stage so he hadn't seen it. I noticed that my reflexes seemed rather exaggerated. I mentioned the anti-GAD result and he found it waiting to be filed and said that I was only his third case in 13 years as a neurologist and his second case was on the ward and much worse than me. He called his Registrars in, told me to walk across the floor and asked them for a diagnosis. For them to see one case was rare, but two in one day! Well, after some hesitation, one of them tentatively ventured to suggest that it was another of 'one like they had just seen on the ward!’ I was put onto 3x2mg diazepam straightaway, to be taken gradually over a few days. It wasn’t until a few days after reaching the maximum dose that I suddenly felt I'd got my life back! An EMG later proved positive too.
ln July 2012, the spasms started to return and friends noticed my stiff walking again. ln October, the diazepam was doubled. This had a good result but my neurologist was horrified as he said it was rather near the addiction level. I am now on 20mg a day but no signs of addiction or withdrawal symptoms if l'm late with my medication.
ln September of that year l woke one morning with an odd feeling in my back which became continuous muscle spasm and by the time I was half-way through preparing the evening dinner I was crying with pain. ln desperation I threw caution to the wind and took an extra 2mg diazepam. Relief in 15 mins!
The onset of cold weather induced robotic walking and, if standing for long periods, displayed gait instigation. From October 2014 onwards, l experienced spasms and frequent falls. Diazepam was increased to 5mg 3 times a day. This continued until April 2015 when I saw the neurologist again who added Baclofen to my prescription and said he would see me in six months.
The Baclofen had no effect. By December I was on the maximum dose, still no effect. At the end of December I was put on Tizanidine and gradually reduced the Baclofen. The Tizanidine didn’t work either, but adversely affected my liver so had to be discontinued.
Five months later I saw a registrar from the August intake who obviously knew nothing about SPS and just read out the last letter to my GP of which I had a copy. She asked me if I would like an appointment in four months. Of course, l said yes but there was nothing available! Eventually, the four months became eight l then saw a different registrar who knew all about the disease and I got the impression he had probably seen a case.
He said to make an appointment for six months when it would be November and I knew that cold and windy weather would affect my SPS. This time
there was an appointment available. This was cancelled by the hospital as were two more. In November we went to Tenerife. I was a different person. No support needed. What a difference the warmth made. ln January 2016, even with a stick, I made three attempts to cross the road at a controlled crossing but couldn't so had to wave the traffic on. One motorist realised I was in trouble, pulled into a side road and helped me cross. Next day I fell in the garden. We had a couple of warmer days shortly afterwards and l managed without my stick at home. As soon as the weather turned cold again I had to use my stick all the time. lf I went out, whoever was driving had to support me, even with my stick.
ln February, I had a series of severe muscle spasms in my legs and had to come downstairs on my bottom. Took 5mg of diazepam which worked as before. Massage of my legs made the pain worse. The end of the month was very cold and my legs were like sticks. Had trouble with stairs in a restaurant and on my way home in the car, my husband driving, my legs were stuck out straight in front of me all the way. March produced a number of spasms, being blown over by the wind, a number of episodes of having to ask for help to cross the road and to resume walking after standing for a while. My husband had to go away for a few days and I was unable to carry anything involving both hands so put my meals on a tray and pushed it along the carpet to my chair on my hands and knees. Still able to use the bus and drive. Went to empty the compost bin, fell backwards into a bush but no harm done except to the bush! Leaving a warm room after choir practice, my left leg went rigid from the hip downwards which was very painful. Had great difficulty getting into my friend's car and my own front door to which I had had disability bars fitted. The same thing happened the following week. Fell in the bedroom due to startle effect, resulting in bruising to leg and arm. The next day I fell twice within half-a-mile when crossing the road. The second time was at a controlled crossing. Managed to walk home with a slightly swollen ankle and cut and bruised elbow. Later, I was unable to put any weight on the foot but pain alleviated by co-codamol. Next day I ached all over but was able to put weight on my foot.
In April we went to Gran Canaria. The temperature was a constant 70 degrees but I needed a stick and additional support. I was unable to cross roads or open spaces. Even crossing from the buffet station to our table caused me to go into spasm. Fellow guests spotted that I was in trouble and took me to the table. Thereafter, my husband had to collect my food, carry it to the table and then go and fetch his own. I fell backwards when taking a photograph and have to be firmly supported since. I no longer drive.
In mid-May l had to have a tooth removed. The dentist gave me an injection either side of the tooth and my legs started jerking from the knee. This passed and I waited for the anaesthetic to take effect. The last time I had any treatment was before SPS, a re-filling, my lip and nose had become numb but this did not happen and when the dentist started the extraction I almost shot up to the ceiling. He stopped of course and asked me what sort of pain it was. I said it was nerve pain. He gave me another injection saying that if this didn't work he would have to refer me. This worked sufficiently for him to extract the tooth but induced the jerking again for a short time.
I am unable to stand up in the shower, so in June I acquired a seat but we are going to have the bathroom altered to make cleaning and getting in and out of the bath easier. And as a shower curtain is no longer suitable, we will have a bath with a screen. Had another fall in the lounge against the arm of the chair which bruised my ribs. A few days later, I had a spasm in the kitchen.
I handed in my notice to the chamber choir which I have been with for over 30 years. Now finished gabapentin. In July, I fell backwards in a shop resulting in a bruised hip and a large lump on the back of my head but, amazingly, no brain damage. On my return home I had spasms in my legs, Myclonic spasms? A few days later I fell in the garden and spiked my breast - very painful and black and blue next day. At the end of the month we went to visit a stately home. Climbing the stairs was fine but coming down, the stair rail ran out for a short distance and though I was offered help I had to decline because help must be static, so I came down the short distance on my bottom and then resumed on the stairs as before.
Still getting regular spasms for no obvious reasons. After cooking an evening meal, my husband has to carry the food because I need a free hand at all times. Often, l'm so shattered I have to crawl to my chair – another open space. I have had to get help with the gardening unless I can do something on my hands and knees. I have had to discontinue my charity work because I need to be mobile. I can no longer shop on my own or go for lovely long walks which I have always enjoyed. My life doesn't seem to have a purpose any more.
I saw my neurologist in October 2015 and he said he wanted to see me in six months. They were fully booked up at that stage and the receptionist showed me a bulging envelope full of slips for people waiting for appointments. This included first referrals. I received a letter last week with an appointment for next month. Exactly a year almost to the day! I despair for the state of our NHS of which I was a part for nearly nine years.
Update March 2019
I rang the neurologist's secretary and asked if there was any way I could be sure of seeing the neurologist himself and not one of the August intake. She told me not to register electronically but go straight to the desk and ask if I could do this. This was granted. When my turn came I was told he had gone! The registrar was very nice and had actually seen a case that morning (the second person with SPS on the books). I was the third and none had been recorded since then. My current medication had not been working for some months and I knew he couldn't prescribe what I thought I could try (Pregabalin/Lyrica). He told me that the neurologist would see patients on an ad hoc basis but I would only get two or three days’ notice. I was getting desperate by this time, only able to walk with a three-wheel mobility aid, so readily agreed. I received a message a few days later asking me to report to the neurology ward at Preston hospital. Here, I was asked to walk across the room without my three-wheeler which I couldn't do without support. He agreed to prescribe Pregabalin but warned me that I would put on weight ('only a few pounds'). I put on half a stone and now have many clothes which I cannot wear any more, despite going on a low carb diet. It was worth it as I could now walk without even a stick as long as it was warm (70 degrees). We went to Lanzarote in November and I walked 7.5 miles to retake some photos having had my camera stolen while navigating some difficult downward steps. At last I felt normal. I could even carry my food to the table without freezing.
The neurologist had said that he would like to see me again in six months. This time I was able to get one but have since had three cancelled appointments so we are up to nine months! Apart from the weight issue and the current cold weather which makes me dependent on a stick again and late in the day, additional support from someone's arm if I have to go out, I am better off than a lot of SPS sufferers. I had read somewhere that people with this condition did not respond to local or topical anaesthetics. First, I had to have a tooth out and after an extra injection, my gum and nose did not go numb and I decided to go ahead anyway as I have a high pain threshold and didn't want to have a general anaesthetic as I had had problems in the past. I managed to withstand the pain without any trouble. Then I developed wet AMD, treatment for which involves injections into the eye every month. Three times the normal topical dose of anaesthetic didn't work, so it's just a question of grin and bear it. I 'jump' when the needle goes in but otherwise OK.
I now have difficulty with words as detailed on the closed website by many members. Never seen this on the list of symptoms before. Reassured that I'm not developing any form of dementia!
Update August 2019
I got an appointment with my neuro at last. He discontinued the diazepam as it obviously wasn't working any more and put me on clonazepam which I have to take at 12noon and 4pm. I have to set my alarm on my phone or I forget. Next, he upped the pregabalin. It doesn't seem to have made any difference but I decided to try and boost my confidence and make myself more independent by taking the bus into town. I was a bit slow getting on the bus, but managed to browse the market and go into some card shops. Then decided I'd had enough. It's quite difficult managing a purse, stick and shopping so I made my way to the bus station. Then guess what - I fell. It seemed to be triggered by the sight of some stationary vehicles at the crossing which I wasn't going to use anyway! Someone came over from the barber's opposite and cleaned and bandaged my skinned knuckles which were bleeding all over the pavement (I'm on an anticoagulant, too).A lady kindly stayed with me until my husband came to fetch me and wanted to know all about SPS, so something good came out of it. So much for independence.