Monday, 21 April 2008

Karen’s FSH Journey. everyday account of living with Fasio Scapulo Humeral Muscular Dystrophy...... the ups and the downs the frustrations and the positives.....oh yes there are positives.

I’ve had FSH all my life. I was born with a defective gene passed on from my Dad. There’s a 50-50 chance of it being passed on.

From an early age I always knew I was different. At school I never looked the same as any of my friends, they could all wear trousers, skirts and pretty dresses. I always had a pot belly that made me look like a refugee from a third world African country!
I got used to the stares and nudges when the other kids would poke fun and call me fish lips because I could never remember to make a conscious effort to keep my lips together. School photographs were a nightmare, my face always looked lopsided and I never seemed to smile although I thought I was at the time.
I was useless at games and PE, no one ever wanted me on their team because I always came last, fell over or dropped the ball at the vital second. When I got to secondary school I found a nice loophole for avoiding PE/games. I loved music and was given the chance to play an instrument, I jumped at it when I discovered the violin lessons were the same time as PE/games. I actually enjoyed playing, and developed my own style of holding the violin much to my tutor’s dismay.

Even now I can still hear my mums voice “Will you turn that leg in when you walk no wonder you’re always falling over” my Dad used to fall over a lot too we were always being told “You’re a clumsy pair of clots try looking where you’re walking”.

When I hit my mid teens that was when the pain started, crippling mind blowing pain down the left side and back side of my neck and into my left shoulder, so bad I just wanted to hit my head against a wall. work was impossible I’d trained as a hairdresser. I really loved it and had worked in London at the Vidal Sassoon Hair Academy but I was finding it difficult to lift my arms and that along with the pain and needing to be on my feet for long periods of time meant I had to give up my career.

So then began the many trips to the doctors and loads of pain killers and heat wraps, but nothing helped. Eventually I was sent to the Woodlands Orthopedic Hospital where a very nice doctor wrongly diagnosed me with arthritis thus began years of exercise. This was with a very stern Sgt major type lady physiotherapist who made all her patients quake with fear if we couldn't quite stretch as far or as high as she said we had too. Much worse though were the manual manipulations done under general anesthetic. The pain would last for weeks afterwards. Eventually I stopped going and decided I would just have to cope on my own and learn to live with it.

During the next few years I got a job as a receptionist which wasn’t too bad as I could sit down most of the time. I was still in pain and fell over frequently but that was me, everyone knew it was Karen’s thing; I’d always got scabby knees and bruises.
I met my husband, and got married when I was twenty and still I didn’t have any idea what was wrong with me. I’ll never forget my wedding practice, my Dad and I had to walk down the aisle and synchronize our legs so we wouldn’t trip each other up. I still chuckle when I think about it now, we must have looked quite a sight arm in arm concentrating on our feet and where to put them. Still we pulled it off and I even managed to stand up after having to kneel for what seemed for ever.

I had our son when I was twenty four followed by our eldest daughter two years later, during both pregnancies I found I was pain free (apart from the actual labour). I still had issues raising my arms and I still fell over but the pain went. I mentioned it to my GP, he said it was coincidence but it was the same when my youngest daughter was born a few years later.

My condition got worse in my early thirties. We didn’t have a car so it was a case of walking and public transport, a total nightmare getting on and off a bus with three children, buggy, bag and no ummph! in the legs to get up the steps. Its amazing how my three survived given all the falls I had when I used to take them out, I couldn’t climb steep steps or hills so they would always go on ahead and check if the way was “mumable”. They grew up knowing no different but I think they were often asked at school “Why does your mum walk funny and keep falling over?”

The pain had by now spread to my hips and legs and walking was getting a real problem. By chance I attended a well women clinic at our new GP's where the nurse mentioned how my left leg was totally out of line. She had a word with the doctor who referred me to see a general medical consultant at the then Birmingham General Hospital, he wasn’t really sure what I had but said all the signs pointed to Cushing Syndrome which was quite a shock especially when brain ops were mentioned. I had loads of blood tests and was told I would hear something shortly.
A few days later I had a call, could I go to the Neuroscience department of the Queen Elizabeth Hospital Birmingham, and be prepared for a few days stay?
My case had been sent to a consultant neurologist who decided to run a whole battery of new tests,
X rays, MRI scan, coordination, and electrical impulse tests which were quite painful, and finally a muscle biopsy done under local anesthetic as they weren’t sure how I would react to a general, I actually watched the whole thing in the reflection of the operating lights above me, to say I felt everything was an understatement.

Two days later my consultant asked to meet my Dad and for us both to attend a case conference. He asked my Dad a few questions and examined him, and then announced to us that we both had a condition called Facio Scapulo Humeral Muscular Dystrophy. It's inherited and I’d got it from my dad and he had probably got it from his dad, there was no cure but as my dad was still fairly mobile I may be okay but it would be a case of wait and see. I could go home now and he would see me every twelve months to see how I was doing.

I remember sitting in the car on the hospital car park, it was the week before Christmas 1988  and I felt as if my whole world had fallen apart.
We managed to get through Christmas but I knew the New Year was going to be a trip into the unknown, I remember feeling very frightened and very alone.

Once the shock had worn off and I realized things hadn’t really changed I was still the same person with the same problems. I just had a fancy name for it, though at the time I didn’t actually understand the long term consequences.
I decided to find out as much information as possible. We didn’t have the internet back then so it was a case of looking in the telephone directory. There was an entry for the Muscular Dystrophy Group (now the Muscular Dystrophy Campaign) so I phoned them and they took my details and promised to send me some info which duly arrived. It wasn’t much but it was a start and gave me something to take to the local library to continue my research.
There weren’t any FSH support groups back then, the MDC had conferences but these always seemed geared up to fund raising and were mostly attended by parents of children with Duchenne MD, I don’t remember meeting any fellow fsher's. I became a member but didn’t get involved as I didn’t think there was anything there for me.
Today there is a lot more information and support available and the MDC is a excellent way of sourcing this as they can you in touch with various groups and fellow sufferers locally and nationally.

A friend of mine had mobility difficulties and told me about disability living allowance it sounded good but there was a problem it was for disabled people and I wasn’t disabled…was I?

At the time I was using a walking stick and relied on my neighbour's to take my children to school as I couldn’t get on the bus and the school was to far for me to walk, my husband did the weekly shop as he had the use of the company van, all in all I was spending more time in the house. I realized by accepting I had a problem I could actually gain some independence so I applied and to my shock found myself accepted for DLA. What a difference having a car made school runs, brownies, trips to the shops, doctors and I could visit my parents which had previously entailed two buses and an uphill walk.

I had learnt a valuable lesson to keep my independence I had to accept that I did have a problem but by using what ever aids/help were available I could still stay in control. Over the years I’ve been on a slow downward spiral, and as my body has changed I’ve tried to adapt and find other ways of doing things. One thing about us fsher's we are a very inventive group of people.

……. "I wish someone would invent an “industrial” strength helping hand I’ve lost count of how many I’ve broken. Then again I don’t suppose they were designed to do the things I’ve done with them over the years, cleaning the bath, polishing, cleaning the windows, oven, freezer, the car, and even a bit of gardening" …..

As my condition has progressed I’ve accepted the changes I’ve had to make. My first car was a little two door Corsa but now six cars later I have a highly adapted  Kia Sedona Pilot affectionately known as the "beast" I'm able to drive this from my electric wheelchair  as I'm now to weak to do sliding transfers.
I exit and enter the car via a remote controlled electric sliding side door, the suspension also tilts to allow an electric ramp to be deployed. The car also has various hand controls for the hand bake, brake and accelerator. Its a great car and does the job except when people park right up close so I can't deploy the ramp and get back in after a shopping trip etc...

I must confess I found the transition from walking to wheelchair the most difficult to come terms with. Initially I was given a chair which required someone to push me, I’m afraid my pride wouldn’t allow that, apart from the fact I had no control of where I was pushed there’s nothing worse than having someone talk to the back of your head, and being parked up "out of the way" really ticked me off. I still had a little use in the tops of my arms so after much pleading with my OT she agreed to let me have a self propelling chair, with a booster cushion, it was hard work but a least I was independent. I think I looked quite sporty with my fingerless gloves and I was happy doing my own thing albeit quite slowly.
I also found it useful as a walker. I could walk for a while and when my legs got tired I could sit in my chair, also it meant trips to the doctors, dentists, theatre, restaurants or visits to friends and family were not such a big problem. I would now have my own seat and not get into the embarrassing position of not being able to get up from chairs that were always too low. In a way it was a good halfway measure as it didn’t seem such a big issue when I finally had to move onto an electric wheelchair.

I’ve been really quite lucky, my local council are very good and to date have looked after me very well, you just learn that things do take time to get into place and with FSH you do have to think long term and never give up till you get what you need. The council gave me a grant which paid for a Wessex through the floor lift, a ramp up to the side of the house and alterations to the bathroom. My OTs provided some great aids my electric riser and recline armchair which I wouldn’t be without, various trolleys, sleeping aids, walkers, personal items etc and some not so good ones like the toilet riser that catapulted my youngest out of the bathroom across the landing and into her bedroom! She wasn’t hurt but we laughed till we cried when she did it, needless to say that went back.
Even now some of the things I’m given to try are of no help but my motto in life is “You have to try everything at least once”. My other favourite is “act your shoe size not your age” a sense of humour is good to have as it keeps you sane when all you really want to do is give up and cry, or scream with frustration.

Update: in 2011 we had major building work done to adapt the house to my ever changing needs full story and photographs to follow (see below)

Having FSH has made me the person I am today. I can’t say it’s much fun having FSH it’s a truly frustrating, devastating and a soul destroying condition, there are days when I sink into the depths of despair. I carry the guilt that I may have unknowingly passed it on to my children and future generations. I feel anger when I can’t do something, I hate my dependence on others, and want to scream at the very people that I love most deeply that they don’t understand how I’m feeling.
I worry what will happen to me in the future, who will look after me, will I be able to cope if I can’t feed or wash myself or have to suffer the final indignities of having others to do the most personal things that I can’t manage myself.

It’s not all been doom and gloom and there are some perks to being "unabled" or a wheelchair user. Free touchline tickets for England Rugby Internationals at Twickenham and getting up close to the players has been quite a good one, as were tickets for centre court at Wimbledon sitting in touching distance of Rodger Federer and Andy Roddick was really memorable.
Most places now do offers of free entry or discounts for carers, and holidays are a lot easier to plan as society is starting to realize that things have to change to allow easy access and facilities for those of us who need help to enable us to keep our independence and dignity.

Some of the stories of the situations I’ve found myself in will make you laugh, like the time I got stuck on the loo at Wimbledon, nearly missing seeing Nadal play, or the time we were sent through the players changing room after a rugby match at Gloucester, and no I didn’t shut my eyes!
Even when I fell over while on holiday in Majorca and ended up with ten stitches and my leg bandaged from ankle to thigh. There was a positive out come, I was treated like a V.I.P and had a whole row of seats to rest my leg on during the flight home and was whisked through customs and baggage with two personal assistants the nearest I’ll ever get to being treated like a film star.

I consider myself lucky in that I have three amazing children that I’m so very proud of. They give me the will to carry on. We’ve all had to get through so much over the years not just the practical problems of living with FSH and all that it entails but the emotional roller coaster that we’ve been on has left its mark on all of us

Things have changed and FSH has a higher profile and there seems to be more of us about, I’m a member of the Muscular Dystrophy Campaign and the FSH Muscular Dystrophy Support Group UK. They are a good source of information and can often put you in touch with others in your local area.

Perhaps one day a cure will be found, if not for me perhaps in time for those who will follow, but until then the journey continues and I wait to see what the future will bring, be it good or bad, I’ve survived this far and fully intend to continue the fight I find myself in with FSH.

© copyright April 2008 updated Jan 2015

* Fame at last........said with tongue firmly in cheek!!

The consequences Of FSH - MD and Should I Have My Children Tested?
When I was originally diagnosed it was difficult to take in all the possible consequences of having fsh. I was talking to my mum about the implications and was very shocked when she confided how badly my Dad had taken the news, he was quite guilt ridden that he had passed on something so dreadful and he was very angry that his father had passed it on to him, his thinking being that his father must have known that there was something wrong with him and the fact he went on to have seventeen children truly angered my Dad.

I remember talking to my Dad and said that he shouldn’t be angry with his father or himself because muscular dystrophy wasn’t known about way back then let a lone fsh, and just as his father hadn’t known that he had fsh, my Dad hadn’t known either so he shouldn’t blame himself, because I certainly didn’t blame him, as far as I was concerned it was just one of those things.
Out of seventeen brothers and sisters my Dad had drawn the short straw, none of my uncle, aunts or cousins have shown any symptoms to date.
I have a younger sister who is now fifty two and luckily seems to have missed out on the faulty gene.
I also have a half sister who must be a carrier as she has a son affected, but her other children seem ok.

All this made me think about my own children who had been born before I knew that I had fsh. I’d always believed that I had arthritis so had never had any concerns that I could pass on anything so serious.
At the time of my diagnosis my children were aged two, five and seven, genetic testing was available and we were asked if we wanted the children tested, it was something we had to think long and hard about.
My consultant arranged for the children to see a pediatric consultant who had them doing all the usual neurological tests, puckering the lips, rocking on their heels and checking how tight they could they shut their eyes. He couldn’t find any clinical evidence to say they were displaying any symptoms but the only way to be sure was to have the genetic tests done.
After much thought we decided against testing, if they had shown any symptoms we would have gone ahead with them, but as they weren’t we thought it best not, because once you are given a label it can dramatically affect you emotionally and practically for the rest of your life.
We didn’t want the children to think they couldn’t achieve their full potential because at the back of their minds there would always be “I can’t/shouldn’t do this because I have fsh. I didn’t want them to be different from their friends, its so easy to make excuses not to do things when you are told you have something wrong, and practically it could affect the chances of getting life insurance, a mortgage etc when they were older.

We were always open with the children about my condition and they were aware it was hereditary but we didn’t dwell on it, and although they were very aware of my decline it never prevented them from getting on with their lives.

I’m sure in retrospect we made the right decision. My son is thirty three, and is a physics teacher, he has a very painful condition called fibromyalgia but he gets on with his life. He's married and he and his wife have decided to have genetic testing if and when they decide to start a family.

My eldest daughter is thirty one and a Intensive care nurse. When she was in her teens she was diagnosed with severe asthma which proved difficult to get under control, but it didn't stopped her doing cross country mountain biking, swimming, abseiling or any other dare devil things. She too is now married and again she and her husband will consider all their options when the decide to start their family.

My youngest daughter is now twenty eight and was tested for fsh when she was twelve as she was having joint problems and it needed to be ruled out, her test came back negative for fsh, which was a great relief.
When she was seventeen she was the diagnosed with a condition called Woolf Parkinson White which is quite dangerous as it can without warning cause the heart to stop beating, she had to under go heart surgery to correct this. Less than six months later she had a gap year and took herself off to New Zealand and Australia for six months. She is now a fully qualified physiotherapist and married, but she and her husband have decided not to have children.

I guess what I’m trying to say is don’t rush into genetic testing if your child isn’t displaying any symptoms, in our case all three have developed other conditions equally unpleasant and debilitating and they didn’t need the extra burden of an fsh diagnosis as well.
Obviously if children do show symptoms it’s a totally different matter they should have a diagnosis to enable them to get help and support to keep them independent.

With the advances of modern technology all my children have decided to use genetic testing when they want to have children, or if in the future they develop symptoms then they will also ask for a diagnosis to confirm they do indeed have fsh.

I’m sure that the decision we made when my children were young was the right one for us.

(c) copyright August 2008 updated January 2015

Useful Links

FSH Support Group UK
Muscular Dystrophy Campaign UK
NMC Midlands (neuromuscular centre) Coventry
NeuroMuscular Centre, Cheshire
The American FSH Society (They

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My Blog Diary

Tuesday 29th April 08
Visiting the Naidex Exhibition at the NEC Birmingham

Today I went to the Naidex show at the NEC, its an exhibition to show case all the latest innovations in the disabled world everything from wheelchairs to house adaptions so its quite a good place to go to if you are on the look out for something special or just want to check out whats new.
I spent a few hours having a quick look round but what I was looking forward to most of all was meeting up with some of the peeps I'd been chatting to online I was a bit worried about meeting new people, we all chat and get on really well online would it be the same when we all met face to face?
There were five fshers, Traceyanne, Paul N, Ian, Paul A (honorary fsher) and myself, along with our carers and helpers.
There was definitely no need to worry we all got on like the proverbiable house on fire, and before we knew it three hours had rushed by, there was still lots of things at the show we hadn't all seen so we had to say our goodbyes, its was lovely to meet up with people I now consider good friends as well as fellow fshers, and really hope that more of us from the forum can get together in the near future.

Wednesday 25th June 08
Visiting the Neuromuscular Centre, Cheshire.......

The Neuromuscular Centre, Cheshire is the only one of its kind in the uk, and specializes in treating people with muscular dystrophy and other neuromuscular conditions.

Over the last couple of years my condition has deteriorated and after a series of falls that left me in A&E with varying degrees of injury including knocking myself out when I fell in my kitchen I decided that I really had to do something to try and slow down my decline.

After one of my falls my GP had referred me for physio at the local hospital, it wasn't really successful as the physiotherapist wasn't clued up on MD and told me "You do know there's no cure for your condition?".......... as if I hadn't already worked that one out.
The one positive outcome was that the physiotherapist did refer me to an orthotist who was brilliant he listened to me when I told him that I wanted to continue walking for as long as possible but I needed to do something about the falling over. He came up with toe-offs, which are leg supports which help to prevent the foot drop which is a problem with md. Along with some new elbow crutches I now felt a bit more confident about walking, I can't walk far but at least I'm not using my wheelchair full time around the house, though I do have to use it when I go out.
I was happy with the toe-offs but felt I could be doing more. I'd read about the NMC on the Muscular Dystrophy Campaigns website but had always thought it was only for children with md. It wasn't till I found the FSHer's website and got chatting to people on the forum who were having physio at the NMC I realized it was open to anyone with md or a neuromuscular condition all you needed was a referral from your GP or consultant.

I asked my GP to refer me and within two weeks my appointment arrived, I was a bit apprehensive and it was a round trip of 140 miles, but nothing ventured, nothing gained.

On my first visit I had a full assessment of my condition and how it affected me including some breathing tests to see if my lung capacity was as good as it should be. It lasted a couple of hours and I can honestly say it was so good to talk to someone who understood what I was saying and knew about all the problems that I had. Lynne who did the assessment was really positive and suggested that I would benefit from some physio, which would include passive stretches and active exercise. So my next appointment was booked for two weeks later.

Wearing some loose fitting clothing and feeling abit worried about what to expect, like "would it hurt?" I was introduced to Nick my physio.
Once on the tilt table I began to relax and started to enjoy myself everyone is very positive all the physio's, staff and fellow users are really friendly and the banter between everyone make's for a great atmosphere.
Nick began by stretching my neck as I'd mentioned I'd been having problems, it crunched and was really painful when I turned it side to side, he supported my head and gently pulled and turned it, after a few stretches I was able to move it pain free, and an added bonus was I think I actually grew a couple of inches taller. lol
He then moved onto the stretches starting with my legs and explained that muscles need to be stretched regularly to stop them becoming tight and short.
The physio's help by supporting the limbs and gently stretching the muscles of the legs and arms. the stretches shouldn't be painful and they make the muscles feel looser and more comfortable. After about an hour of stretches and some gymball work we finished off with 20 minutes on the passive cycling machine.

I've been attending the NMC every two weeks for the last few months, I really feel much better for going because as well as the physical benefits, mentally I feel better too I think its because I feel that I'm doing something positive to keep my mobility and independence a little bit longer.
The only problem I have now is that I enjoy the passive cycling so much I want one of my own so I can go "cycling" when ever I want too!

Update June 2014
I've not been to Winsford in a while, due to the distance I had to travel however I've not given up my physiotherapy. I am a founder of NMC Midlands based at Hereward College in Coventry UK. It took a lot of work but the centre opened its doors in 2011, see the side blog for further information.

Wednesday 20th August 2008
I’m trying to be positive……..a bit of a ramble!

I’ve been spending a lot of time stuck in the house even though I have an electric wheelchair which can be loaded on to my fully adapted car which is parked on my drive.
The reason for this is I no longer have a carer to go out with me, my daughters used to spend a lot of time with me but they are grown up with lives of their own, one is at university the other at work, and weekends are spent with boyfriends etc which is as it should be.

I took a business course at college last year but had to drop out after three weeks as I had a really bad fall whilst unloading my wheelchair, I ended up being taken to hospital with severe bruising and a face that looked liked I gone ten rounds with Mike Tyson.
A couple of weeks later while in the kitchen I lost my balance and fell over and banged my head on the wall and knocked myself out, yet another trip to A&E.
Then less than a week later I had another fall no damage this time just a bit embarrassing as I had to call the ambulance service as I was home alone and was jammed between the sofa and the fire place. ( which was quite funny looking back) lol

I figured it was time to apply for some help, I’d heard that I was entitled to an assessment from social services and that it may be possible to get something called direct payments which can be used to buy the services of a personal assistant to help with going out shopping, appointments, a little house work etc.
Great just what I’m looking for, just one problem I can’t get past the “jobsworth” person at social services who after I had explained what I was enquiring about told me…….
...”you don’t want direct payments to much paper work involved you won’t cope with that “…… fine so it now appears my brain has gone along with my body…..
.....” but if you require help with personal care I can arrange that, washing, dressing, toileting etc”….she seemed to get a little annoyed when I told her……..”I want someone to go out with not someone to wipe my bum"
Needless to day I’m still stuck at home reliant on friends and family to go out, but I've not given up I'm just going to approach things from another direction. A tactical diversion me thinks lol

It’s so frustrating I want to take up the offer of playing boccia in September, also there’s a local card making/ scrap booking course starting soon which I would like to go to, I also have hospital appointments and a few conferences I need to attend.

The last few weeks have not been good, I recently lost a very dear friend to cancer, it was less than twelve months from diagnosis to her losing her fight for life.
Another friend has had so much happen to her over the last few years she’s is a bit of a walking miracle, osteoporosis, a brain hemorrhage, cancer of the kidney and various intestinal problems, yet she remains positive and gets on with life.
My best friend was diagnosed with MS a couple of years ago and although some of her episodes are quite severe she determinedly carries on working, raises her family and has two grey hounds which she exercises daily without fail.

Today I’m in pain my shoulder and back hurts as do my legs, neck, hands and fingers. I look at myself and my friends and think that life’s just not fair, but then who said it was fair?
……but hey the suns coming out and I’m alive to see it, so perhaps its not all bad and maybe tomorrow will be better…….

Saturday 23rd August 2008
.......I'm Feeling better today and looking forward to tomorrow as everyone will be home for dinner, and Monday is a bank holiday.

21st September 2008
A new member of the family......

I lost my border collie a few years ago at the age of sixteen, we had spent many years together and as my fsh had become worse and she’d slowed down due to old age we became good company for each other. She didn’t want long walks and was quite happy to spend all day asleep in front of the fire.
Last week instead of the usual walk there was a family day at the local greyhound stadium, there were dog shows and the usual fund raising stalls and a group of dogs from the rehoming centre wearing little coats with “I’m looking for a new home” on them, I usually go over and give them a few doggie treats and a bit of a fuss.
Today was a little different as one of the dogs stayed with me even when the others moved on in search of more treats, she was such a sweet little thing and when she put her head on my knee and gave me one of her sorrowful looks I was smitten so we spent the whole day together, she seemed very happy to walk alongside my wheelchair.
As the day drew to a close the handler looking after her came to collect her and asked if I was interested in adopting her, well what can I say?…………
I’m now the proud mum to three and a half year old Boogie a gorgeous black greyhound. I’ve had her about five days and she’s has been absolutely perfect.

Greyhounds are the ultimate couch potatoes, they are happy with two 20 minutes walks, and so far we’ve had no “little accidents” its still early days and we are still getting to know each other but I’m hoping that she will become the perfect companion for me, she gives me a reason to get up in the morning and you can’t be sad when you watch her as she discovers what its like to live in a house for the very first time. Visit for more about the greyhound trust.

18th November 2008
I think I must be getting old......

One of my passions has always been reading and my idea of heaven is to spend a couple of hours in my local library working my way through the shelves in the detective section or delving in the biographies and historical non fiction departments.
I’m a huge fan of Agatha Christie’s Poirot and Miss Marple. I’ve always thought I was born in the wrong decade I love the elegance of the twenties and thirties.

So when my mobility declined and I found it more difficult to get to the library I just assumed that it was just one more interest that I would have to for go.
I happened to mention it to a friend of mine and she told me that our local Library ran a scheme called “Library Services At Home” for people with disabilities.
I gave them a call and hey presto I now have a monthly delivery of books which keeps me going through the dark winter nights we are having at the moment and it doesn’t stop with books I've had Video’s, dvd’s and cd’s. There's also a monthly magazine with book reviews, recipes quiz's and local new with a whats on section. They also do large print books and I've recently started to have audio books a really relaxing way to listen to a good book.
I would thoroughly recommend it, ring your local library to see if they offer the same service.………………Oh yes I also receive a copy of “mature times” in my box of books…….. I don’t look or feel that I? lol

27th December 2008
End of the Year Thoughts.......

It’s been a while since my last post, partly due to a total lack of motivation but also due in part to my fsh which seems to have gone into a downward decent which has seen my already limited mobility further reduced to the state that I’m now finding standing quite difficult I seem to have less coordination and I’m finding that simple thing’s such as reading and typing really tiring.
I suppose having a cold and then the flu virus hasn’t really helped matters, and that along with the cold, dark gloomy days has made me feel some what fed up.
I’m still trying to get some help and I’m still waiting for an assessment from social services, but after many phone calls and being told that I’m in the “system” I’m not really confident that any help will be forth coming.
I may also have to stop my physio at the Neuro Muscular centre in Cheshire as my daughter who used to accompany me has just changed her job and no longer has Thursdays as a day off. I could manage to drive there myself and the staff have offered to help me when I get there, but it would mean that I wouldn’t be able to stop if I needed the toilet as I’m unable to unload my electric wheelchair.

There have been some positive’s in my life, I now have two greyhounds my first hound Boogie joined us three months ago and after a few ups and downs settled in quite well except that she was really nervous of going out for walkies and often went into earthquake or statue mode when she became frightened so on the advice of the Retired Greyhound Trust where we had Boogie from we decided to adopt another dog.
So welcome to Fletcher a three year old male who is a very big boy but a total gentleman. Unfortunately Fletcher has had a very hard life as a racing dog and has the scars to prove it he is in desperate need of lots of TLC, and trips to the vets.
In his first week with us we discovered that he has a heart murmur, and only today we found signs of some kind of skin problem which means we will be back at the vets tomorrow, he’s such a gentle soul and just wants to be hugged and loved so whatever it takes to get him right we will do it though I fear that we may have to for go a few things to pay for it.

I’ve also become more involved with the FSH Support Group UK, and have been busy organizing a “Get Together” next April which has been quite time consuming but very interesting I just hope it’s a success as it would be really good to get as many fellow fshers in the uk together as I really believe that it helps to know that there are others facing the same problems and we are not alone

The end of one year and the start of another was always quite inspirational to me when I was younger but now as I think about it I find myself realizing that life as it is at present is somewhat mundane and that the days, weeks and even years seem to merge into one and that time is no longer important or indeed relevant to my world.
Is that good?.... I don’t know, most days are the same routine of getting up sitting in my chair waiting for members of the family to return home, watching TV, perhaps a bit of reading or making the odd phone call to friends or family for a chat.
Only the changes in the seasons now seem relevant now. To me spring is the most wonderful time of the year when for a few months my world as seen from my windows comes alive.
We have at least now passed the shortest day so we are moving in the right direction, how good it will be able to feel the warmth of the sun again.

Happy New Year Everyone

Wednesday 18th February 2009
What price independence?..........
................well so far the total is £34,694.00, and thats probably a under estimated figure.

The last few months have been difficult and I've finally had to admit that I need to make changes to my lifestyle.
I've come to realize that without help I'm unable to function everything from showering to hospital appointments and days out are governed by "Who's available" or more accurately "Who wants too?" come/help me.

I can no longer safely use the shower which is over the bath as this involves me lifting my legs to get in and out. Last week I managed to get my right leg out but couldn't lift the left one. So I was stranded half in and half out. Luckily one of my daughters was around and after a few frantic shouts of "Help I'm stuck" which at first fell on deaf ears she finally appeared and rescued me from my predicament.
It's now been agreed that I will only shower if there is some one around, which means I have to get up at 5.30am to get showered and dressed.
Everyone leaves the house at 7.00 am and no one gets home before 6pm so it's a real pain especially as we only have one bathroom and four people trying to use it at the same time.

I saw an occupational therapist who recommended a level access/wet room shower for me, and said I would be eligible for a grant.
I applied for a grant from the local council only to be told after a twelve month wait that we no longer qualify as the criteria has changed, basically unless my husband gave up his job and claimed state benefit there would be no point in applying.
If my husband gave up his job we wouldn't have a roof over our head so we wouldn't need a grant to get the bathroom adapted, the man on the other end of the phone wasn't amused when I pointed this out.
I've now had a quote to get the work done £4,850.
The last time I checked I hadn't won the lottery so it looks like I'll be getting up at 5.30am for the foreseeable future, and when I get well and truly stuck perhaps a hose down in the back garden will suffice.

Why should my independence rely on someone else working/not working?

When I attended my last appointment at the neuromuscular rehabilitation clinic in September last year my consultant told me that my current wheelchair no longer meets my needs as my condition has deteriorated.
I need a chair that will get me into a standing position, I also need it to be easy to load into my car or suitable to be able to drive from. I've checked a few out and have been quoted around £13,841, the NHS doesn't supply the type of chair I need they will only be able to give me a voucher for £2000 to go towards purchasing one myself so that leaves a shortfall of around £11,841. They suggested I get in touch with various charities to apply for help in paying for one.

So I now have to go begging to charities to keep my independence.

I'm also have problems getting in and out of my car and I can no longer use the hoist to load/unload my electirc wheelchair (the one that's no longer suitable for me) on my own.
I phoned motability and was told I should apply to them for a drive from wheelchair car and they would allow me to terminate my present car early even though I have another twelve months to run on the agreement.
This sounded good the lady went on to say that these vehicles are very expensive, I'm looking at around £16,000, but I could apply to their grants department for help with funding..........

........well the application form came to day, and guess what?

Yet again my independence relies on someone else's income.

I know that I have been lucky in the past to have received help with living with FSH.
I would be lost without my lift, wheelchair and car which have kept me mobile and independent to date, but now I need help more that ever before, I don't expect to get something for nothing and have always contributed as much as possible, I've even taken out loans in the past to finance the advance payments on my mobility cars, but we're just not in the position to do this again.

It's so annoying and frustrating I have FSH but MY independence relies on other people working and THEIR income.

Monday 16th March 09

I'm sorry that my blog hasn't been updated recently, I'm organizing the "Spring Get Together" for the FSH Support Group UK. I was told when I offered to do it that there would be around 40-50 members attending, I must have done something right because we have 87 bookings, it looks like it's going to be a very busy day but a very interesting one. Not long to go now, I'll post and add photo's to let everyone see how it went

Tuesday 14th April 09
FSH-MD "Spring Get Together"

Last September I attended the FSH Support Group conference which was held in Birmingham it ran along side the MDC national annual conference, it was a good day on the whole but many of us were dissappointed with the short afternoon session allocated to the support group.
We all sat in rows listening to a presentation on the cause and on going research into fsh. I have to admit to being rather bored with the whole thing, I know what I have, I know there is no cure. What I really wanted to hear about was how do I I live with this infuriating condition and how do other fellow fsher's cope.

Well to cut a long story short I offered to organize an event. Right from the start I wanted it to be less formal than the usual conference format definately no long rows of chairs with everyone looking at the backs of people's heads......... I wanted to see faces!
It was six long months in the planning, and after many phone calls, emails, and hours spent planning the programe of events the great day dawned.

The weather wasn't bad an almost barmy spring Saturday, the setting was the Rowheath Pavillion, Bourneville home of Cadbury's chocolate.
The day was planned to encourage members to chat and get to know each other so for this reason I decided to use large round tables, and along with an all day tea/coffee station the stage was set for everyone to chat and get to know each other.
The theme of the day was "Keeping Well" with FSH. I wanted to offer as much infomation as possible on the subject. So our guest speakers hopefully reflected this.

Nick Emery head of Physiotherapy at the Neuromuscular Centre in Cheshire covered the benefits of physio in maintaining mobility, he also included orthotics, scapular fixation and alternative therapies in his presentation.

Carolyn Evans is a specialist neuromuscular nurse from the Royal Orthopaedic Hospital Oswestry. Carolyn talked about her roll as a specialist nurse and what that entails when looking after her patients. Carolyn was able to give lots of practical advice on living with FSH, and both she and Nick did workshops in the afternoon sessions where members could ask questions on a one to one basis, which proved really popular.

Our third guest was Jane Stein who is a regional care advisor from the MDC and is based at the John Radcliffe Hospital Oxfordshire.
Jane is co author of the new "Adult Self management" pack recently produced by the MDC for adults with one of the many adult onset neuromuscular conditions.
The packs are designed to cover all aspects of living with a neuromuscular condition, all those questions you wanted to ask but never knew were to go to get the answers.
The pack contains an A4 size folder packed with fact sheets and information and also includes a diary for you to keep records of hospital appointments etc so you can always reffer back to it when needed. Unfortunately it wasn't possible logistically to have them available on the day but there were cards in the welcome pack that everyone received so you could send off for your own copy.

We were also pleased to welcome Stuart Watt a group member who has a special interest in FSH he is also a member of the steering group FSHD Europe. Stuart informed us of the progress being made on the international front with regards research, treatment and support for FSHer's across europe.

Members also had the opportunity to try out the Thera range of passive exercise cycles demonstrated by Shukri and Sonia from Medicotech Ltd. They proved to be very popular, I've definately put one on my wish list.

The day also included a two course buffet lunch which seemed to go down well with everyone, and I must admit the caterer's did a splendid job especially with the dessert table.

The comments so far have been very positive with members enjoying the opportunity of chatting with fellow fsher's.
I really enjoyed the day too even though we had a few hiccups with the projector and sound system.
I must also thank our three "Here to help" volunteers Louise, Laura and Alison who were on hand to help everyone, and also kept the day running smoothly.

It was really good to make new friends and I hope to see everyone again soon.

I've been asked to take on the role of chair of the group and I've accepted

Friday 22nd May 09
Splat! to A&E on a spinal board......badly bruised back, leg & discharged, then collapsed and ended up in resuss.......spent night in hospital........think this is one fall to many may have to spend admit defeat and use my wheelchair around the house:(

Thursday 18th June 09
Pride come before a........
I've never really thought much about the falls I've had over the years, I suppose I've been fairly lucky, no broken bones, only knocked out once and lots of bruises. It usually takes me a week or so to recover.
My last fall even by my standards was pretty spectatcular. I was washing the dogs feeding bowls out in my laundry. I remember putting them on the drainer, I dried my hands and turned towards the kitchen door, and that's what caused the problem my feet stayed firmly stuck to the floor while my upper body twisted round. I remember feeling a searing pain shoot up my back and then crashing to the floor in a somewhat unnatural position with both hands tucked underneath me. I took a few seconds to grasp what had happened, okay I may need a little help here so I tried to free a hand, bad mistake the pain returned making me instinctivly scream out. Luckily my daughter was home and on finding me in a heap crying out in pain promptly dialled 999. I was so relieved when the paramedics arrived truly knights in shining armour. To cut a long story short I ended up on a spinal board with a surgical collar and my head imobilized with copious amounts of sticky tape. Once in A&E I was examined with the utmost care and finally given the all clear nothing broken, but I had badly bruised my back and hips, they gave me some pain relief and said I could go home. Standing to get into my wheelchair was going to hurt, but it had to be done, okay lets take it slowly try sitting up first, I remember sitting then someone switched the lights off...
I had a searing pain under my ribs and across my abdomen, and feeling really hot and nauseaus, I can remember thinking that the ceiling was moving above me as I was whisked into resuss voices were asking where the pain was, but I couldn't reply I just felt as though I was trapped in a vice which was getting ever tighter. My daughters tell me that all hell broke out with theatre's put on alert, crash trolleys and scans, and I became the proverbiable pin cushion.
I remember coming round the pain was gone, the Consultant came to me and said they thought I'd had an internal bleed but the scan hadn't showed anything so next thought was an heart attack as my ECG's had been all over the shop and my heart rate had dropped to around 40. They decided to keep me in over night and treated me for a heart attack but the blood test that they did twelve hours later came back negative. So I was allowed home the next day none the wiser as to what had happened or why.
My recovery has been some what slow I'm unable to do the few things that I could do before, I'm in pain most of the time and reconciled to useing my wheelchair full time, though I've have graduated to using a walking frame for the short walk from my bed to the bathroom.
I've always fought fiercely for my independence and my right to remain upright no matter what, thats why there is no one else to blame for my fall or the position I find myself in. I've always pushed the boundries when the dr's said use the walking frame I refused, when they said you'll be safer in the wheelchair I chose to ignore their advice because I knew better or thought I did. I've now had to come to terms with the fact that this may have been a fall to far, I've done so much damage to my back that I may never go back to what little mobilty that I had previously and I may have to learn to live with the pain.
I really wish I'd been using the walking frame that day because I wouldn't be in the pain that I'm in today, I wouldn't have had all the trauma of A&E and above all I wouldn't have caused my family so much worry and upset. I can still remember the shocked frightended look on my daughters face when I came round in resuss.
I suppose I've learnt the hard way Pride really does come before a fall!

Sorry that the blog hasn't been updated recently, it's been a bit hectic lately, I'm off to Glasgow this weekend but when I get back I'll tell you about my recent trips to Liverpool, Bradford and Glasgow, and about my ongoing battle to get my care service's in place, oh and about my new drive from wheelchair car :)

Wednesday 18th November 2009

The last few months have been very busy. In August I went with friends to Liverpool for the Mathew Street Festival. It was the first time in ages that I’d felt brave enough to venture far from home and stay in a hotel. Since my fall I’ve become very reliant on my wheelchair and was very worried about coping in a different environment
We had booked into the Staybridge Suites close to the Albert docks, the room was large and spacious, king size bed, kitchen area with microwave, full size oven and hob and dish washer, and a nice lounge area with a very comfy sofa and flat screen tv, and the bathroom was huge with a level access shower. The whole hotel was really spacious with easy access to all the public areas.
Liverpool was some what chilly and windy unlike the locals who were very friendly and accommodating, there were several of us in wheelchairs but everywhere in the dock area was really accessible. We found a fab restaurant called Gusto’s they served the best Italian food ever and they had no problem moving the table and chairs to accommodate us; we were also made very welcome in the Blue Bar where we hung out well into the early hours. It was a great weekend and I’d definitely be up for a return visit.

Bradford & Glasgow.....
In September I attended the Muscular Dystrophy Campaign’s annual conferences held in Bradford and Glasgow. I’ve recently become quite involved with the FSH-MD Support Group UK and became their events and publicity coordinator. It was decided at one of the committee meeting that the group really needed to raise its profile and the best way to achieve this was to have a presence at the MDC conferences. Again this involved travelling long distances and staying in hotels but I found that the best hotels to stay in as a wheelchair user is the Holiday Inn/ Express by Holiday Inn chain. Their wheelchair accessible rooms are very spacious and bathrooms well thought out, and best of all if you have p.a’s or carers you can have an interconnecting room free of charge so it makes a weekend away more affordable.
It was really good to meet fellow FSHer’s and we had lots of positive feed back. We also showed off our new display boards and banner promoting the group.

On Saturday 17th October the FSH-MD Support Group held their “Autumn Get Together” it was well attended with over 85 members and guest speakers, as well as representatives from some of the leading companies showing their latest ranges of equipment promoting independence and mobility.
There will be a full write up in the group’s newsletter which is due out now and it will also be available on the new website which will be launched shortly.
We also held the groups annual general meeting, Sheila Hawkins the group's chairman stepped down from office after five years and I was elected the new chairman. I’m very honoured to be asked to take on the role but also very aware how much work is involved.
I chaired my first meeting a few days ago, I think it went okay, no complaints as yet and we have already made plans for 2010 when we will be celebrating our 25th Anniversary.

Sunday 22nd November 09

Direct Payments: Finally things seem to be getting sorted, after 12 months of waiting I've been given direct payments to fund my personal care, though I will be a lot happier when it finally reaches my bank account so that I can actally pay my p.a's.
Motability: I'm now in the system to have a drive from wheelchair vehicle from Motobility, I've been told to expect my Kia Sedona Pilot sometime in March/April. It wasn't too long a process to go through, the paperwork and driver assessment were straight forward it's just the waiting between stages that is so frustrating it will have taken eleven months from my first enquiries to actually getting my car. In the mean time I've got a car that I can no longer get into unless I have someone to help me get in and out.
House Adaptions: After a visit to see my consultant who wrote a letter to Occupational Therapy Services I'm hoping that my house will be adapted so that I will have an accessible bathroom and bedroom with room to move around now that I'm spending all day in my wheelchair. It hasn't been without its stresses I was initially told that it was quite acceptable to be stripped washed and that access to a shower or bath was not a priority........I had quite an argument to persuade them other wise. I've passed the initial assessment but will still probably have to jump through hoops to get what I need.
Wheelchairs: I've finally received a new wheelchair from Birmingham Wheelchair Services after waiting nine months for a an assessment plus another three months till it was delivered. It's a Invacare Spectra Comfort Plus, not exactly my first choice, but it was the best they offered. It does have a rise and tilt in space feature which is great for transfers, but no reclining back or leg lifters which would have been very welcome given my problems with my back and swollen legs caused by my fall in May.
The chair has not been set up correctly the speed is set to factory settings which is basically for indoor use. My old chair was an outdoor/indoor chair and was able to get up pavements, hills and would maintain a brisk walking pace when I went out with my dogs for a walk. This one however is an indoor/outdoor and moves at a snails pace it dosen't matter if its set on number one or the top number five it stays the same speed and is unable to do the smallest of inclines. I did ask why I'd been given an indoor/outdoor chair and asked for it to be looked at by someone from the wheelchair services, the guy that came out said it wasn't his job to change the settings if I required anything other than the original factory setting this would have to be done by the assessment department. Despite contant phone calls I've not yet managed to get this done.
I'm now going to resort to making a total nuisance of myself by contacting the senior management and writing letters sent by recorded delivery so that they can't say they not received my complaints which is what they keep telling me when I phone or email them.
Sunday 25th February 2011

My Eye Surgery......

Ever since I was diagnosed with fsh-md I’ve had problems with my eyes firstly dry eye caused by my eyes being partially open when sleeping and more recently droopy eyelids. I suppose as with most symptoms of fsh-md it slowly crept up on me. I’d always had one eye which seemed lower than the other but given that I’ve one arm stronger than the other and the same with my legs I just accepted that it was all part of having md. Although I’d practically plucked away all my eyebrows because for some reason they always looked like two hairy caterpillar’s sitting on my eyelids rather than being on my brows where they belonged.
My optician pointed out at my routine eye check that my droopy eyelids had become worse since my last check up. When he lifted my eye lids at the sides I realised that my world had indeed become a lot darker almost like wearing a peaked cap that protects your eyes from the sun but in this case it was making my world much darker when I was indoors and my field of vision from the side was also affected, a bit worrying as I’m still driving
At my next appointment with my neuromuscular consultant I mentioned my droopy eyelids and asked if anything could be done, he referred me to a consultant plastic surgeon at the Queen Elizabeth Hospital, Birmingham.

At my first appointment I had photographs taken and after a thorough examination of my eyes and a history of my fsh and how this affected my face it was decided a brow lift would be the answer.
I wasn’t keen on the idea of a general anaesthetic so my consultant said he would do it as a day patient under a local.

On November 1st I arrived at the hospital for my operation a bit nervous, not about the actual surgery but more how I would manage transferring from the bed to trolley and back again.....the usual worries when you’re unable to do these things unaided.
My surgeon was brilliant he explained that he would make the whole of my forehead numb and that I shouldn’t feel anything at all. Everyone in the theatre was really helpful and reassuring I suppose they were used to their patients being asleep during surgery

A cold compress was placed across my eyes to help keep them closed, and to the strain of some very laid back jazz music my operation commenced.
I had two slices of skin each the size of an orange segment removed from above each eye on my brow bone then the muscle above my brow was lifted and attached to tissue under my fore head the cuts were then stitched back together so that my eyebrows were once again on my brow bone I was in theatre around two hours. The time seemed to fly by and next thing I knew it was all over and I was sitting up in recovery. About half an hour later I was back on the ward where I finally had the opportunity to get my mirror and have a look at what had been done........

............Apart from looking like I’d been dragged through a hedge backwards I was really pleased with the result and I noticed how bright everything now seemed.

Over the next few days the bruising came out, I did have a lot of swelling around my eyes and forehead my daughter remarked that I looked like a cross between Herman Monster and a
It was quite painful for the first week and I took the recommended pain relief, but gradually over the weeks the pain lessened and the swelling went down. I’ve not had any permanent numbness to any part of my forehead which is possible when undergoing this type of surgery.
I went back to the outpatient’s eye clinic to have the stitches removed ten days later and my surgeon was pleased with the result. My next appointment was in December where it was decided that I may have further surgery on my eyelid to get rid of the last of the loose skin as the lids are still slightly hooded, but the final decision won’t be made till April when I return for a check up.

I’m really pleased with the result and glad that I had it done. For the first time in years I actually have eyebrows in the right place and they’re not drawn on with an eyebrow pencil.

30th June 2014

Well its been quite awhile since I've done any blogging in fact its been years so much so its taken me a couple of days to work out how to do it as its all changed while I've been away.
Such has happened that it would probably take me a few months to write it up so here's a very brief update..............

My fsh has progressed and I'm now no longer able to drive as my neck muscles are now very weak. This was also made worse by a bad dose of shingles on my face in April 2013 which caused me to have to resign my position as trustee at NMC Midlands. Which I found very difficult. As one of the original founders of the charity I'd put a lot of hard work into it and having to walk away because of the stress it had caused me which in turn triggered the shingles I found difficult to come to terms with.

How ever life goes on 2013 wasn't one of my best but 2014 has been much more enjoyable. We have had three family weddings in 72 days.......

My son Steven married the Lovely Sarah on the 10th of April, My eldest daughter Laura married the equally lovely Neal on 10th May 2014, and my youngest daughter Louise married her long time partner Darren who is also very lovely on the beautiful island of Grenada on the 24th June...........I now have an extended family including a grandson. I'm very proud of my new family and love them all to bits.

Now that life is returning to "normal" I hope to keep my blog updated so I hopefully post again soon!!

Please click on comments to leave your thoughts or a message on my blog


Anonymous said...

That was an excellent read and I've bookmarked this site in anticipation of some of your stories about living with our common enemy!

And I had two of those muscle biopsies and can totally relate when you say you felt everything ... :^(

traceyanne said...

What can I say other than it is virtually a mirror image of my life even down to the violin playing. :-)

I really enjoyed reading your journey but I have to admit to shedding a tear.

I'm just happy I now have people to share these tears with, who know exactly how I feel and what I'm going through. The future suddenly seems a little brighter :-D

Anonymous said...

Oh, how I love your story. Especially, the PE part in your intro. I won an award in PE-new teacher decided to hand out these wooden letters in 7th grade. I won an E for effort and she didn't understand why I didn't smile more when receiving. My best friend I met that year. She was an athlete and had won several letters. We became friends because she would catch up with me at the end of the line when running. Her lap with me was her resting lap. She always told me there was some strange reason why I was always last and she was always first. I quess a lot of us would like to know the answer to that. I found out I had FSH at 25. I am 36 now and married one daughter (5) and still a dental hygienist 15 yrs now. I fall down some and drop things and etc., etc. I'm still able to get around pretty well. Hope that continues.

fishingdog said...

Helps finding stuff like this. The medical info sites are useful, but they miss out so much. Like what is actually like.
You mention West Midlands Group. I live in Northamptonshire. Will that group be my nearest or is there another one?

Ann Pink said...

Read most of your story, it so matches mine. I might add that we even have some of the same facial features. In fact you bear a passing resemblance to one of my younger sisters.

I too find it infuriating that I have to rely on my independance on someone elses decisions. I applied for a grant for bathroom and kitchen adaptions and because my husband works they want 10K towards any works. If I had £10K why would I need a grant.

My husband bless him is very good, but having worked all my life I find it difficult staying at home now. I walk with a stick or a rollator walker. I try and do most things but its a struggle sometimes.

I'm 54 but act like a little old lady at times - its frustrating to say the least.

Shona said...

Hi Karen,

It was really interesting to read your blog. What a shame you didn't get diagnosed earlier in life it might have helped. You are doing great work with raising awareness and helping other people to hopefully have an easier time than you did.

I have Myotonic dystrophy, I'm trying to raise awareness of PGD and so have started writing a blog about my story. I believe PGD can be used to help people with FSH have a baby without passing on their condition too. Have a read if you are interested.

Best of luck with the future and running your support group. If you think it is a good idea perhaps you can tell your support group members about my blog. I already have a couple of followers who have fsh.


Shona said...

I've just given you a blog award. Head over to my blog for the details. Shona

Chevalier Mal Fet said...

Thank you so much for posting this. I have been desperately searching for information the last two years, trying to find people with similar issues.

I have only recently started my own blogging, or in my case randomly jotting down my thoughts trying to organize them!