I am delighted to welcome all our FSH members to our very first newsletter. It has taken years to get to the publishing stage. I must firstly thank Norman our editor and publisher for all the many hours he has spent on this our first issue, it has been well worth all his efforts.

I would like to thank all those that have contributed and to say to those of you who have not, please do send any information that you think might be useful to other members, including useful hints and tips.

It is always very difficult to keep in touch with our members as we are all scattered around the country, but through regular editions of our newsletter we will be able to reach everyone.

We held our annual get together in October in Oxford, this was a very informal meeting and we were very fortunate to have as our guest speaker Dr Peter Lunt who reported on the progress being made on the research into FSH, and introduced us to Dr Mark Rogers who has recently commenced a FSH research project at Cardiff.

Our next meeting is planned this coming October in Oxford, we will be sending our information nearer the time.

Lorraine Jonas

Financial help is available for essential equipment.

The Joseph Patrick Memorial Trust (JPMT) gives financial grants to people with neuromuscular conditions who could not otherwise afford to buy essential items of equipment for daily living and mobility that are not provided by the NHS, social services or education authorities.

The majority of applications are for help to purchase powered wheelchairs but JPMT can also assist with other equipment.

The JPMT is a charitable Trust established by the Muscular Dystrophy Group through the generous support of the Patrick family.

JPMT staff will be happy to deal with any queries you have about equipment and if, for any reason, the Trust is not able to help in a particular case, they will try to put you in touch with someone else who may be able to do so.

For further information the JPMT can be contacted at:

7-11 Prescott Place,
London
SW4 6BS

There is nothing the matter with me,
I'm as healthy as can be,
I have arthritis in both my knees
And when I talk, I talk with a wheeze.

My pulse is weak, and my blood is thin
But I'm awfully well for the shape I'm in.

Arch supports I have for my feet,
Or, I wouldn't be able to be on the street.

Sleep is denied me night after night
But every morning I find I'm alright
My memory is failing, my head's in a swim
But I'm awfully well for the shape that I'm in.

The moral is this as my tale I unfold
That for you and me who are growing old
It's better to say "I'm fine" with a grin
Than to let folks know the shape we are in.

How do I know that my youth is all spent?
Well, my "Get up and go" has got up and went.

But really I don't mind when I think with a grin
Of all the grand places my "Get up" has bin.

Old age is golden I've heard it said
But, sometimes I wonder when I get into bed
With my ears in the drawer, with my teeth in a cup,
My eyes on the table until I wake up.

E're sleep overtakes me, I say to myself
"Is there anything else I could lay on the shelf?"

When I was young my slippers were red
I could kick my heels over my head
When I was older my slippers were blue,
But I could still dance the whole night through.

Now I am old my slippers are black,
I walk to the store and puff my way back.

I get up each morning and dust off my wits,
And pick up the paper and read the "obits",
If my name is still missing I know I'm not dead,
So I have a good breakfast and go back to bed.

Author Unknown

For most people who begin considering whether to start a family or not, there are a number of questions that they ought to ask themselves, e.g., can we afford it? Are we mature enough yet? Are we ready for all those nights in?

But for the majority of the above they do not have to consider the reaction that other people will have! They will not have hoards of so called "experts" quoting theirs and others opinions on the morality and responsibility of disabled people having children. They will not have ignorant libertarians offering them abortions at every turn. These are some of the attitudes we had to deal with when deciding that we wanted to start a family.

For those of you reading this who do not know us, Tracy has FSH and Robin is a full-time wheelchair user, so whilst we were able to counter all of the above with the knowledge that we would love and cherish any child we had, there were practical aspects that had to be considered.

We began by consulting the medical experts on the possible side effects of a pregnancy to Tracy's health. Most of the people we spoke to were of the opinion that it would be extremely tiring and would probably lead to months of bed rest, which would inevitably lead to muscle deterioration, possibly leaving her unable to walk after the birth.

Despite all these negative predictions we decided to go ahead in our attempts, and we must say that once we made the decision the medical team were very supportive, if not too much, with fortnightly ante-natal appointments, twice weekly hydrotherapy sessions and so many scans that we think Joshua had more pictures taken of him before he was born than most babies have taken after.

So how was the pregnancy then?

Tracy was delighted with the first sign of morning sickness, and mourning by the time it was into its second week. This and hourly night time wees were the only bad part of the pregnancy, the rest was just dandy, and this even included getting away for a fortnight to Cyprus at 16 weeks.

The pregnancy progressed as per normal and as each week passed we passed another deadline for needing to use a wheelchair all the time, the Doctors became more surprised, and we grew more confident.

By the time Tracy reached 20 weeks she was actually beginning to walk better than ever, this continued right through to the birth and the Doctors were so astonished they kept a video record for using as part of a future paper.

We had hoped, along with all our ante-natal team that we would have a natural childbirth, but by 39 weeks baby decided it didn't fancy the hard route so we elected for caesarean and baby Joshua arrived 18th August 1995.

We had reservations about invasive spinal anaesthesia, because we had heard some horror stories about the after effects of epidurals and spinal taps but with much reassurance from the senior anaesthetist who would perform the injection, we chose a spinal tap, which proved both painless, very effective, and without prolonged after effect.

Eleven weeks on from Josh's birth, although the days are hectic we are managing, every day we find different and better ways of doing things, and activities which seemed almost impossible a few weeks ago are becoming second nature, however if anyone had any ideas or helpful tips on lifting and transporting baby around the house, then they would be welcomed.

Please forward these through the FSH support group.

Robin & Tracy Surgeoner

Doing your hair
One of the irritants I've found over the years is raising both arms on high, independently.

I can raise one with the help of the other, but not one on its' own. So when it comes to doing my hair I've chosen the easiest route. However a few years ago my hairdresser styled my hair differently using rollers. Not ordinary rollers but Velcro ones and you can actually put them in your hair one handed. These can be purchased at chemists etc.

Bras
Bras, or bra straps in particular, are another nuisance, as they tend to fall off my shoulders. I've found over a period of time that a sports bra or a cropped bra top are the best buys and the most comfortable yet.

Dry eyes
Another problem that FSH sufferers have are their eyes not closing properly, so they can suffer with dry eyes. I've found for myself, after trial and error, that "Tears Naturelle" suit me.

It's worth trying different brands to find the best one for yourself. it may help to use a sleep mask. This is something I've still to find, let alone try.

NB:
These are just a few tips that I've found to help me, but you may have found others, so it would be helpful if you can write in with your experiences and remedies for us all to make use of.

Margot Keats

Dear All,

The 1st edition of the FSHMD Newsletter.

I have been asked as Chair to write a few words.

I was diagnosed with FSHMD in 1961 at the age of 18 by Professor Simpson who at that time was based at the Northern General Hospital in Edinburgh.

I had blood tests taken and a muscle biopsy and various electrical tests were done by passing electrical pulses through my muscles. I had facial weakness in the cheeks and eye lids and an inability to blow my cheeks out whilst having my lips closed. My left foot which was also a flat foot had dropped and my right foot which was clubbed at birth had been operated on late between the ages of 6 and 9.

When I left school at 16 I had become an electrical engineering apprentice.

Professor Simpson came out to my work and after speaking to the chief engineer advised me to resign my apprenticeship as the work would become to heavy for me as the FSHMD progressed. I was sent to an Industrial Rehabilitation Centre for six weeks to be reassessed work-wise. I was then sent to college where I obtained a Diploma in Commerce and embarked on a career in Accountancy.

The doctors wrote to my parents and said that I had a 50-50 chance of life and that I shouldn't get married as any children from that union had a 50% chance of inheriting the FSHMD. In other words dig myself a grave and step into it. As a result of the operation on my left foot I had had no primary education and only four years of secondary education. I had missed out on sports at school and on leaving school at 16 had joined the golf and tennis club only to be told two years on that these pursuits would become too arduous. I became very angry and after going through a period of self-pity and "why me" I commenced a course of self education in "mind over matter".

I studied psychology, parapsychology, occult philosophy and all the sciences to try to come to terms with my condition.

It was through voluntary youth and social work that I met my first wife Avril, and in 1965 we were married. A year later in 1966 my daughter Shuna was born. In 1967 I had an operation to strengthen the muscles in my left wrist at the Royal National Orthopaedic Hospital in London which was carried out by Dr Brooks. Being left-handed I was experiencing increased weakness in the wrist and fingers which was affecting my work. I had also developed a ghost vision in my left eye which was very short-sighted. I had warts on my hands and other parts of my body. I was told by the doctors that these other conditions were unrelated to the FSHMD.

The FSHMD had now affected my left foot which had "dropped" and I had to learn to throw the foot forward or trip and land on my face.

It wasn't until the age of 40 that I started to slow down a little and draw on the social services. In 1971 I divorced Avril and in 1983 I married my second wife Claire.

She soon afterwards became disabled with bone cancer, arthritis and diabetes.

In 1990 I was forced to take early retirement due to the stress that my Accountancy business was placing on my bodily condition.

I use various aids at home supplied by the social services, including a manga bath seat, an electrically operated chair to help me get to my feet and we will soon be having our flat converted for wheelchair access for us both.

I have a spring-loaded calliper on my left shoe to lift my left foot and have special shoes made for me at the National Hospital in Queen's Square.

I use a computer to write my letters and use the Internet to chat to other disabled people throughout the world.

As a result of the excellent services provided at the Abbey Centre at Abingdon near Oxford we propose holding this year's annual meeting there.

Our guest speakers will be Dr Sarah Yates and Dr Peter Lunt and there will also be exhibitors.

Initial dates proposed for this meeting are: 31st August, 21st September and the 19th or 26th October.

A final date and the usual letter and forms will be sent out in due course nearer the time.

As we are a voluntary support group for those with FSHMD the only time we request donations from our members is to cover our running costs and refreshments at the annual meeting. This is our only source of income.

I would like to thank our Treasurer Norman Jonas and our Secretary Lorraine Jonas for the many unstinting hours of work they have both carried out in ensuring that this support group continues to maintain its high standard both administratively and more recently in publishing this newsletter.

Robin A. Brown

"In a few families and in some isolated cases, hearing loss and specific problems with blood vessels at the back of the eye have been found" (Source: FSH Fact Sheet FSH1 Nov 93 1/6)

In December 1992 I had two dizzy spells and developed blurred vision in my left eye. the Consultant Ophthalmic Surgeon diagnosed the problem as a "Central Retinal Vein Occlusion". Despite laser treatment the eye has not recovered and now allows only 50% of normal vision.

It was suggested that the C.R.V.O. (Haemorrhage) could have been caused by raised blood sugar, blood pressure, ocular hypertension or a combination of all three. A careful diet soon had the blood sugar in order and daily pills control the blood pressure. However this action was too late as the eye has not recovered.

My Consultant Neurologist confirms that there is potentially a connection with FSH, although there is a lot of dispute about how real this connection is. Apparently Robyn Fitzsimons, when working for Professor Dubovitz at the Hammersmith Hospital, reported that about two-thirds of patients with FSH had abnormal blood vessels in the retina. Quite a few other workers have looked very carefully for these abnormalities and have not found them in anything like such numbers.

With hindsight I feel that the problem could have been avoided if I had asked my GP to regularly check my blood pressure, blood sugar and cholesterol. I recommend that anyone with FSH should take this simple precaution before it's too late.

Gordon Nutter

I wonder how many people can say that discovering that your partner has a degenerating condition has saved your marriage?

We can!

After 20 odd years of marriage it gets beyond a joke when your husband seems so lazy that he won't even wash his hair or cut his own toe-nails.

But to begin at the beginning:

We married and had two children straight away, so by the time I was 20 I was at home bringing up two toddlers. My husband had to work long hours just to pay the bills, so it fell to me to decorate the home, do the garden and various other bits and pieces normally expected of the husband in a relationship.

Once the children had started school and I was able to go back to work, it gradually began to register that it was falling on me to do all these things as well as working. Over the years resentment built up, particularly when another child, and two major operations came along and I was still expected to carry on as before.

Why couldn't my husband be like others and do more maintenance around the house? Why didn't he have what I considered enough baths? Why wouldn't he wash his own hair, cut his own toe-nails? etc, etc. I suppose some of the things that were annoying me were only minor, but it is the little things that build up large resentment and put a strain on a relationship.

About eight years ago we decided enough was enough. My husbands' job was changing and in order to protect it we decided to get his lack of arm movement, etc. investigated so that perhaps he would qualify for a "green card".

Off he went for various tests at the local hospital. He was then sent for a muscle biopsy. We went back for the results to be told what we regarded at the time to be devastating news that it was Muscular Dystrophy. Like most people who have only read about it in passing, it was like receiving a death sentence. We were then referred to genetic counselling because we have three children. Here we found out slightly more about the condition and the fact that FSH isn't the same as Duchennes, that it can effect either sex, and that my husband's brother and sisters should be tested. This is when we first met Dr Lunt in Manchester. The FSH was traced back to my mother-in-law and possibly to her father.

Once we knew what was wrong with my husband it explained so much. At last I could understand why he wouldn't do things, not through laziness, but because he knew he couldn't do them. Now we can work together and work around things.

He could not lift himself in and out of a bath because of lack of strength in his arms. Solution - install a shower. His toes did not bend, and his ankles dropped so that explained the difficulty in cutting toe-nails. No strength in arms means tiredness when gardening. Solution - concrete the gardens and fill with tubs of perennial plants. Can't paint the ceiling. Solution - either get a decorator in or paint the skirting boards and lower wall while I paint the ceiling and upper walls.

So far none of our children have shown signs of FSH, and as our eldest two are now 30 and 29, we are hopeful they are clear, although our daughter does have Myasthenia Gravis, and is on medication for this. Our youngest at 15 is not showing signs, but, because he has minor Spina Bifida, which has resulted in a small problem with his shoulders, we cannot rule out FSH altogether.

We are however, hopeful.

Anonymous

About five years ago Lorraine suggested to me that she would really like to publish a newsletter for the Group so that members could write about their experiences and how they cope day by day. Other members could then find strength and benefit from the ideas put forward; and thus the members would be supporting each other, which was the whole ideal behind the formation of the group.

We accordingly sought out and purchased the most appropriate Desk Top Publishing software package and I learned how to use it. Unfortunately, it has taken all this time to eventually publish and I am very grateful indeed to those who have taken the time to write articles and send them to me.

We do hope that you enjoy this first issue of our FSH News-Sheet and we want very much to make it a regular publication; at least twice a year, if not more.

We can only do this with your support - so please write to me and let me know what do you think of this first issue - did you find it interesting and helpful? Are there any points you would like discussed in future issues? Do you have any ideas you would like to pass on to other members?

Please support the news sheet so that the news sheet can support you! Thank you.

Norman Jonas

MAY 94
I started to feel unwell at work. I felt a lot of numbness, and lack of feeling in my upper body. This caused me to have a nervous breakdown as I thought I was losing my mind, because I didn't know what was causing it.

Things gradually got worse, and I had to give up my job.

One of the things which added to the problem was that I always had protruding shoulders, and have always felt very self conscious about my appearance so I decided to take matters further and see if there was anything that could be done to improve things.

MAY 94
Went to see the family doctor who examined me, and said that there might be weaknesses, because I have always had difficulty lifting my arms out, and above my head. My father then decided that I should go and see a private consultant in Burnley where I live.

JULY 94
Went to see a Doctor Chatterjee. He also examined me, and took some x-rays. I was then told that I had small clavicles (collar bones) which was causing me to have difficulties. He then decided he was referring me to a specialist he knew of. I then received an appointment to go and see a Professor Wallance at the Harlow Wood Hospital in Nottingham.

JAN 95
Went to see Professor Wallance.

I had some further examinations and was told to perform simple exercises like lifting my arms out in front of me, to my sides at shoulder height and above my head.

The team who examined me then went away to confer, I was told to wait.

I was called back into the examination room, and was told that something could be done for me, but first of all Professor Wallace suspected that I had several sets of muscles which were not working or had never worked at all, and that I was being referred to his associate at Liverpool Royal University Hospital. He also told me that he would get one of his colleagues interested in my case.

MARCH 95
Went to see Professor Edwards at his clinic in Liverpool. I underwent a series of tests i.e. Cardiovascular, e.c.g., breathing, and x-rays. I was told to come back for the results, and have a muscle biopsy performed.

AUGUST 95
I was finally going to find out what was wrong with me.

Professor Edwards had the results of the tests, and I was told that I was suffering from a form of Muscular Dystrophy known as Facio Scapulo Humeral, that there was no cure or treatment for, and was told to get on with my life and make the best of what I had got.

Professor Edwards then hinted that I might be able to have an operation performed which is known as Scapula Fixation.

OCTOBER 95
Went to see a Professor Frostick at Liverpool's R. and S. Dept. Went through a series of strength tests, on my arms, which rated me on a scale of about 10. I was in the range 4 to 4.5.

I was told which muscles weren't working, and what he was going to do to improve things.

Professor Frostick then said he was going to perform two operations on my left and right sides. This involved collapsing my lung to get into the chest wall and fix my scapula to it with stainless steel wires and bone grafting.

To do this he would have to take a bone from my thigh and graft it over the wires so that they would fuse.

I was told that I would be in hospital for 10 days and then in plaster for 3 months.

Following physiotherapy I would then wait a further five months before the second operation.

NOVEMBER 95
Went into hospital on the 5th and had the operation performed on the 6th. Was in ten days then came home with the cast bandaged to me. (My god it's heavy - I weigh 5kg more than usual.)

Went back the following week to have it reinforced, and was told to go back in 3 weeks for a check up.

DECEMBER 95
Went for a check up and was placed in a lighter fibre glass cast. This is 3.5kg lighter than the plaster cast.

This is as far as I've got so far. The next time I go back is in January and I will find out if fusing is taking place and whether the cast can be removed.

I hope this surgery has bloody well worked as it's been damn painful and I have yet to have the left side done.

I will keep the group informed of my progress.

Nicholas Haworth