Hi everybody, my name is Stella. I have been following the community blog for some weeks now and feel it's about time I contributed. I am 57 years old and was diagnosed with Hughes nine years ago, although looking back at my medical history it's obvious I've had it since I was about 20 but of course nobody knew what it was then.
When I was at St Thomas's earlier this year I saw Beverley Hunt for the first time and she suggested that I write everything down that had happened to me and send it to her. I found it very yet therapeutic to do so and would recommend it. Having a site like this means there will always be somebody to talk things over with which is great. Having written this all out once for Prof Hunt please forgive me for just copying it into my blog. I cannot type any more and use Dragon speak voice recognition system on my computer. I am very envious of those of you who can still work and hold a job down. The brain damage I have sustained means my thought processes unimpaired and my memory is crap!
I apologise in advance for how long winded this is and please feel free to scroll down to the end:
'The following pages are an attempt to describe the impact of Hughes'Syndrome on my life. It has taken me some time to do it but I feel it has been a very worthwhile exercise. Firstly, to make a record for others. But secondly for myself to write everything down. Nobody usually asks how I feel about it:
There is absolutely nothing wrong physically with my hands, but they just do not do what I want them to! I guess this is consistent with the brain damage I have sustained.
Dressing: mostly this is all right as long as I lay my clothes out the right way and put my shoes in front of me the right way round. I cannot tie bows and therefore lace-up shoes are a no-no. I have one pair remaining but I have put elastic laces in them so that I can just push my feet in. Belts are also impossible to thread but once they are on I can do them up. Large buttons are okay but small ones do not work at all. I avoid complicated pieces of clothing and rarely wear tights! I find it very difficult to put things on hangers the right way round.
Driving: I can no longer drive and, as I live in rural Sussex this makes getting around very difficult. I rely very heavily on my husband and friends to take me out. We have a bus service which runs every two hours. However, I cannot read a timetable or get money from my purse easily. Coupled with a very poor sense of direction, I have yet to try this on my own. Getting lost is a big problem. When I was still driving I would learn certain routes and they were instilled by repetition. As a passenger this no longer works and it doesn't seem to matter how often I repeat a route. This makes me fearful in unknown places. Even going to the loo is tricky as each one is different - light switches door locks, dispensers, hand dryers etc.
Food preparation: I can cook, albeit slowly. I have to use a particular potato peeler and I can chop as long as I'm careful. I find I need to concentrate on one thing a time. I find recipes difficult to follow as I cannot retain the information and need to refer back to the recipe all the time and, as my eyes don't seem to function properly, I constantly have to to refind my place. I can cook simple dishes relatively well but cannot deal with lots of things once, i.e. timings and complicated dishes.
Writing: my physical writing skills are very poor. I can write a shopping list or a birthday card, but the writing no longer looks like mine. My handwriting used to be quite fluid and bold but is now badly formed. I have to think about what I am writing and quite often will write a letter. 'b' instead of a letter 'g ' for instance, if I don't concentrate. Anything that requires me to write in straight lines or in boxes on a form is very difficult and filling out a form is hugely challenging. I can write a cheque I if I concentrate. I deal with these difficulties as much as possible by using Dragonspeak on my computer, which is a voice recognition system. I used to be a 60 words per minute typist but my typing skills are now virtually non-existent although I have noticed recently that I can find the keys on the keyboard a little quicker. Luckily, my spelling skills seem largely unaffected and I can still visualise the words in my head as I spell them.
Reading: this has improved quite a lot. At one time I could not concentrate on a book at all and had to read magazines and short articles etc instead. As I was an avid reader since childhood this was very hard to bear. However, I can now read books as before, albeit very slowly. Newspaper articles with columns can prove difficult as I want to read across the whole page. This is also difficult in museums or galleries where text is often written in columns.
Maths: I used to be quite good at mental arithmetic, but now find it quite hard. Adding figures up on a piece of paper is impossible as I just cannot make sense of it. I resort to a calculator or, if I'm in a shop, by giving too much money and always getting change or asking for help in counting. When I have to count things out I have to do it one object at a time. I struggle to count groups of things. For example, if I were asked how many spoons were on the table I could not look at them and tell you. I would have to move them one a time and count them out as I did so.
Laying the table is a challenge for the same reason. I count out four knives, four forks etc. Then I have to walk around the table laying out each place setting. I cannot reach across the table and lay the cutlery out upside down.
Ironing/laundry: I tumble dry most things and avoid as much ironing as I can. However, I can still manage to do it, albeit not very well. Folding things is very tricky. If I can lay them out, e.g. on the floor or a bed, it is a little easier.
Bedmaking: changing a duvet cover or pillowcases is a nightmare! I quite often end up in tears of frustration and embarrassment.
Walking: I have no trouble walking for long distances, although balance and judgement can be an issue, for example when climbing over a stile. I have also managed to fall off steps and a stage due to misjudgement and lack of concentration, but luckily have not hurt myself badly. My left foot is permanently numb. I have had nerve tests, which were normal and I understand that this is again down to brain damage. I find judging traffic a problem and, if I am on my own, I always use crossings.
Maps/plans/directions: all a mystery to me! I need something physically in front of me and the right way round to make sense of it.
Art: I had to give up my artwork as I could no longer draw or paint what I saw. I knew he end had come when I couldn't draw a triangle shape! However, in the last few months I have gone back to classes and things seem much better. Whilst painting actual objects is difficult I have adapted by doing much more abstract and expressive work and thoroughly enjoying it! I am even planning a charity art event to raise funds for the Hughes Syndrome Foundation.
Concentration: one thing at a time-if I am distracted I forget. Whilst I feel my basic intellect is much the same, taking on complicated thought processes is impaired, I think, possibly because my memory doesn't retain the information. I cannot scan documents to get the gist of something but if someone does he précis for me I can understand perfectly well. I volunteer in a charity shop one day a week and can use the till reasonably well unless I am interrupted. Again, it is doing one thing at a time. However, I enjoy my time in the shop and find it very helpful in terms of interacting with new people and doing something independently. I have also made some very good friends who accept as I am.
Memory: my lack of memory is a great sadness to me. Luckily I have been married for a long time and my husband reminds me of so many things that I have forgotten. If I am reminded that we have been somewhere but I cannot remember he quite often is able to tell me some little detail that will reactivate something and over a period of time (maybe days) some remembrance will return.
Grief and depression.
Grieving for my lost self is like a bereavement as is losing my memories. When I am depressed, which luckily only occurs occasionally, I find it very difficult to handle - I want to rise above it, but fail miserably. I sometimes despair for the future, especially if something should happen to Dave. He is my memory and constant support.
The grief over my children's deaths was severe (especially Alice). I marked anniversaries in my head until she would have been 21 and then I let her go. It still makes me sad but I have got over it.
I have always believed myself to be a strong person and I consider myself very fortunate to have such a strong marriage with the family who love me and whom I love. I am therefore disappointed with myself for not being able to cope with my limitations better. I find it hugely frustrating not to be able to carry out everyday tasks but I find the mental limitations worse. I am very grateful that I look perfectly normal and present well but at the same time people (including some friends) do not realise how limited I am. They see me functioning perfectly well in a social setting (albeit with some help) but not all the underlying issues.
Medical background\medical staff
Some medical staff have been very good, albeit unable to help, others mediocre. One or two have been awful. It was such a relief to get to St Thomas's, where staff actually understood what was going on and could treat me appropriately.
I first went to a GP in the late 70s with badly aching joints and very bad reactions to insect bites. I was given a blood test and told nothing was wrong. When I was 25 in 1980 I became pregnant. There was nothing wrong at first, but then my blood pressure started to rise. I ended up with pre-eclampsia and had to have an emergency section at 32 weeks. The baby lived for two days. A week or so later I went to my GP with pain in my right side. She was excellent and sent me to hospital straightaway where I was treated for a pulmonary embolism and put on heparin disperse the blood clot. After that I was put on an anticoagulant for six months and then told I could try for another baby. He was born at 30 weeks in 1981 and also died at two days old.
My consultant at the time then decided we should try heparin and I self injected for my third pregnancy resulting in the birth of my son in 1983 and another in 1986. I have no issues with either my GP or consultant at that time as they both did their very best for me, given that there was no knowledge at that time regarding Hughes syndrome.
I carried on for the next few years with little recourse to doctors. I was still getting joint pains, allergies etc but nothing I couldn't cope with. I have worked in the health service for most of my working life and I do remember when I was working as a medical secretary in hospital, I asked one of the consultants if my past problems and current ailments could be linked to lupus. She nearly laughed in my face! My current problems started when both my children were in secondary school. I was becoming increasingly tired, could no longer knit or sew and typing was like wading through treacle (I used to be a 60 wpm typist). I started to get visual disturbances and sometimes I couldn't see out of one eye. One day on my way to work I virtually collapsed. My GP at that time didn't know what was wrong until I mentioned blood spots under my fingernails and he then suspected lupus, which later tests confirmed. I was put on low-dose aspirin by doctors at St Helier Hospital in Carshalton. I later moved to Redhill with the diagnosis of lupus with antiphospholipid syndrome and continued with the same treatment under another Dr. She was very 'hands on' but suggested no other treatment despite my reporting worsening visual disturbances and memory difficulties.
When I moved to East Sussex in 2006 I was in a fairly desperate state and knew something was seriously wrong. I was struggling to read and write and felt increasingly ill and helpless. My memory was very poor, as was my concentration. I was still having migraine like symptoms. I was permanently confused and, whilst I was still driving, I felt I was losing my judgement and reflexes. Over the next year my sight deteriorated through cataracts such that I was virtually blind in one eye. I have had surgery but no longer have the confidence to drive. My GP was very sympathetic and supportive but could offer no answers other than to try thyroxine which did help slightly. He referred me to rheumatology consultant at the Conquest Hospital in Hastings. I have to say in no uncertain terms that he and his department were absolutely useless! The first time I saw him. I explained how my symptoms were worsening and how it was affecting my life. He offered no help or support whatsoever. My next visits were to his rheumatology clinic nurse. I remember on one occasion sobbing for help as my life was falling to bits. She told me to pull myself together as I didn't have lupus at that badly and go back to my GP if I was worried. Nobody in that department knew anything about antiphospholipid syndrome or what it can do to you. I insisted on seeing the consultant again and was reluctantly given an appointment for three months hence. When I saw him he spent some considerable time telling me how he knew Prof Hughes personally and how they had played tennis together years before but gave no practical help whatsoever. Increasing my 150 mg dose of aspirin or anticoagulant therapy was never even mentioned.
My GP, in desperation, I think, finally referred me to a neurologist at the Conquest . I underwent an MRI scan and a lumbar puncture, but she could find nothing untoward. However, she was very sympathetic to my situation and, finally, it was she who referred me to St Thomas's. They in turn finally prescribed anti-coagulant therapy. The effect was almost instantaneous: I felt a fog lifting from my brain. Unfortunately, the damage had been done and an in-depth MRI at St Thomas's revealed some permanent brain damage, the effects of which I have already mentioned.
By the time I got to St Thomas's, I had finally realised myself the significance of Hughes syndrome. If I had been able to function properly I may have been able to do more research for myself, but I put my trust in the medical profession. I feel that my initial diagnosis nine years ago of lupus with antiphospholipid syndrome was not helpful. All the medical people that I had contact with seemed to concentrate on the lupus aspect and dismiss the APLS. Ignorance, and to a certain extent indifference, by the medical profession has, I think, the meant I have far worse brain damage than I should have sustained. When I first presented with migraine like symptoms nine years ago I was an independent woman, still holding down a job, driving reading, writing and looking after my family. Today I am in receipt of disability living allowance, unable to work (and therefore unable to contribute to the family income) or drive.
I feel very saddened that such lack of treatment has left me so disabled. It affects not only me but my close family as well. My husband has to carry out a huge amount of simple tasks on my behalf and I can no longer be the help and support to my 87 year old mother in any practical way.
I have found a complete lack of interest by most medical staff. I have tried to talk to all of the doctors I have come into contact with regarding in the prevalence of Hughes. They all seem to think that it is something very obscure and rare and of no interest or consequence to their other patients.
My fears for the future
When I am low I worry for my future. It is a very selfish thing but I worry that if I am left on my own I will be unable to cope. I rely heavily on my husband for many things, and it would be difficult for me to remain independent without him. My worst times are the week or two before a period (currently every two months) so I am hoping that these bad times will lessen or cease once I am post menopause. I am 57 after all!
My hopes for the future
I hope that over the next few years I will regain some measure of independence and feel more and more confident. I have already adapted to many things, and can read, write, use a computer, etc much more than I did. By using Dragonspeak I can e-mail, use the Internet, send texts etc and, whilst slow and needing a lot of help, I find this very useful.
I have a good social life and many good friends for which I am eternally grateful.
I am no longer embarrassed in shops when I take a long time to do something - they just have to wait! Unfortunately (or fortunately). I do not have an obvious disability and people have absolutely no idea what Hughes Syndrome is. I quite often resort to 'cerebral lupus' if asked. One of my hopes for the future would be that Hughes syndrome would be better recognised by the general public but, failing that, that it was recognised by GPs and hospitals as time is of the essence when treating it. My other ambition would be that all young women were routinely tested upon seeking contraception or pregnancy as I believe it would mean so much less heartache. It seems to me it would also make financial sense in that less resources would have to be directed to multiple pregnancy loss, premature baby units and all the other complications that Hughes currently incurs, not to mention savings in Disability Living Allowance etc.
Overall, I feel optimistic for my future. I believe I have made some progress over the last couple of years, for example, taking up my art again and regaining some of my confidence. Some of this, I'm sure, has been by adapting to and accepting my limitations, but I also think some things are getting better: my thinking processes seem to be clearer and I am more capable of trying new things, although still far too reticent in unknown situations. I'm working on it! '
For those of you who persevered to the end well done. Stella