Welcome to our first Newsletter of 2004.

As you will see we have included articles on Scapular Fixation, research into FSH, and the report and pictures from our 2003 Annual Conference - the first to be held in the spring. There are also articles and letters from our members.

Our Newsletter is for some of you the only means of communication with the group, and we really need your input. By writing articles based on your experiences in coping with life you can really help others with the condition, and at the end of the day that is the whole ethos of the support group.

Our membership is spread all over the UK. Unfortunately not everyone can manage to attend our annual get togethers, which always enable us to catch up with one another.

This year our Annual Conference will be on May 15th and, for the first time will form part of the MDC Northern Conference. We hope that you will be able to attend what we are sure will be a very interesting day.

We have received £684 in donations in the year ending October 2003. Anything that you can do to help the Support Group financially will be appreciated.

I am happy to report that we are receiving enquiries from various local medical authorities who will now hold details of our group on their databases, ensuring that their information is up to date.

We hope that this will spread the word about us and help educate those in the medical profession and enable us to increase the support that we offer to those newly diagnosed with FSH. Our website has been completely re-designed and is continually being updated and maintained. As a result more people are now using our message board, so we are truly becoming a “global group”

Lorraine Jonas

You will see that the 2004 conference is now being arranged for Saturday May 15th. at the Jarvis Hotel in Leeds and I look forward to seeing as many of you there as possible.

I realise that I have now been Chairman for nearly four years and it is time to give someone else an opportunity to take on what is a rewarding position. I remember Gordon Nutter, our previous chairman, saying that three years was enough so I am past my sell by date!

Fortunately the support group does not have any financial worries due to Norman’s excellent stewardship and Lorraine is a tour de force in running the Secretariat and developing membership. The chairman’s duties are pretty simple, firstly to liase with Lorraine and Norman about the annual conference, keeping in touch by phone and Email. It is essential that the chairman has internet (Email) facilities.

Secondly, the most important, and pleasurable, part of the job is to phone all new members a few weeks after they have registered (Norman sends you notice of all new members). I find this very rewarding, by sharing experiences of life with FSH, I find that I learn a lot about how our members adapt to daily life and share lots of amusing stories. Having a good laugh on the phone with someone you have never met certainly cheers you up and makes you realise just how people adapt and make the best of their personal situation. Membership seems to grow by about 12 per year so that there aren’t too many phone calls to make.

Please contact Lorraine if you would like to know more, by all means contact me if you wish, but don’t be shy. A good knowledge of living with FSH is important.

Finally, it really is a rewarding experience being Chairman.

Needless to say I shall keep an active interest in the group.

Martin Fielden.

Scapularfixation
Members have asked several times for information on the scapularfixation operation which by anchoring the scapular (shoulder blade) to the ribcage of the back can improve raising the arm and other movements. Several of our members have had the operation with differing degrees of success. Because the operation is comparatively rare it is difficult to get advice based on many examples. The medical profession produces assessments of such procedures in a series called the Cochrane reviews which are accessible via the Internet. I have reproduced a resume of the latest review for this operation below. A full version of the review can be ordered on the internet for a small charge (£10). Unfortunately it is not very definitive due to the rarity of the procedure.

Cochrane scapularfixation resume 2003
Cochrane reviews are regularly checked and updated if necessary.

Background:
Winging of the scapula is caused by weakness of the thoracoscapular muscles, which allows the scapula to lift off the chest wall during shoulder movements. In facioscapulohumeral muscular dystrophy (and occasionally in other muscular dystrophies) there is selective weakness of the thoracoscapular muscles which may spare other shoulder muscles such as the deltoid muscle. This imbalance results in significant winging and loss of shoulder function. Historically, a number of different surgical and non-surgical interventions have been used to achieve scapular stability. This review examines the evidence available for the use of all scapular fixation techniques in muscular dystrophy, especially facioscapulohumeral muscular dystrophy.

Objectives:
To examine the evidence for the relative efficacy of scapular fixation techniques in muscular dystrophy (especially facioscapulohumeral muscular dystrophy) in improving upper limb function.

Search strategy:
We searched the Cochrane Neuromuscular Disease Group trials register (search updated March 2003) for randomised trials and other reports, and made enquiries from authors of trials and other experts in the field.

Selection criteria:
All reports of scapular fixation for muscular dystrophy, including quasi-randomised or randomised controlled trials, comparing any form of scapular fixation (surgical and non-surgical) in people (of all ages and of all severity) with scapular winging due to muscular dystrophy. Our primary outcome measure was objective improvement in shoulder abduction. Our secondary outcome measures were: patient-perceived improvement in performance of activities of daily living, cosmetic results, subjective improvement in pain and proportion of patients with significant postoperative complications.

Data collection and analysis:
We collated and summarised studies on the treatment of scapular winging in muscular dystrophy.

Main results:
No randomised trials were identified. We therefore present a review of the non-randomised literature available.

Reviewers' conclusions:
Operative interventions appear to produce significant benefits, though these have to be balanced against postoperative immobilisation, need for physiotherapy and potential complications. We conclude that a randomised trial would be difficult, but a register of cases and the use of a standardised assessment protocol would allow more accurate comparison of the disparate techniques.

Citation:
Mummery CJ, Copeland SA, Rose MR. Scapular fixation in muscular dystrophy (Cochrane Methodology Review). In: The Cochrane Library, Issue 4, 2003. Chichester, UK: John Wiley & Sons, Ltd.

This is an abstract of a regularly updated, systematic review prepared and maintained by the Cochrane Collaboration. The full text of the review is available in The Cochrane Library (ISSN 1464-780X).

The Cochrane Library is designed and produced by Update Software Ltd, and published by John Wiley & Sons Ltd.

Martin Fielden

Dr. Kemp, one of two new research fellows, has contributed the following article to help our readers understand more about his work on FSHD.

By Graham Kemp, M.D., University of Liverpool

I and my three colleagues in Liverpool, are pleased to have been awarded the first FSH Society Roberts Foundation Nutrition Research Grant (FSHS-SMRF-01), for a project entitled “Muscle damage by reactive oxygen species, muscle atrophy and effects of creatine supplementation in FSH muscular dystrophy.”

A port city on the northwest coast of England, Liverpool was the European end of the transatlantic sea route to the USA, and may be known to some readers for its contributions to pop music in the 1960s. It is home to a large civic university with a thriving medical school, and to some of the country’s biggest and busiest hospitals. Three of the research team are based in the Faculty of Medicine of the University of Liverpool. I am in the Department of Musculoskeletal Science. Having trained in medicine at Oxford and specialized in clinical biochemistry, I held research positions at the Universities of Sheffield and Oxford before moving to Liverpool in 1996, where I am also Faculty Director of Postgraduate Research. My main research interests are in muscle biochemistry and muscle diseases and their study by non-invasive magnetic resonance techniques, and I take part in the outpatient clinical care of patients with muscular dystrophies.

Malcolm Jackson is Professor of Cellular Pathophysiology in the Department of Medicine. After training in biochemistry in Surrey and London, he held research positions in London before moving to Liverpool in 1984, where he is a former Dean of the Faculty of Medicine. Professor Jackson has a longstanding research interest in mechanisms of cell damage in muscle disease and ageing, particularly in the role of “reactive oxygen species” (of which more below).

Neil Roberts is Director of the University’s Magnetic Resonance and Image Analysis Research Centre (MARIARC), which applies magnetic resonance methods to research mainly in neuroscience. After training in physics in Liverpool, Cardiff and Aberdeen, Professor Roberts held research positions at the University of Durham and the University of California at Santa Barbara before moving to Liverpool in 1991, where one of his research interests is the mathematics of measuring body composition and structure. He is currently overseeing the expansion of the facilities at MARIARC with the purchase of two new magnetic resonance scanners.

The fourth member of the team is Bryan Lecky, Consultant Neurologist at our specialist neuroscience hospital, the Walton Centre for Neurology & Neurosurgery, Liverpool. After medical training at Cambridge and London, Dr Lecky held clinical and research positions in London before moving to Liverpool in 1987. His particular clinical expertise is in neuromuscular diseases, and he has extensive experience in clinical trials. This eighteen-month project, which started on 1st January this year, combines three of our main research interests: quantitative magnetic resonance methods, mechanisms of muscle damage, and the therapy of muscle disease. It is a pilot study designed to investigate two questions: how tissue damage occurs in the muscle of patients with FSHD, and whether adding a naturally-occurring substance called creatine to the diet can improve muscle strength and reduce tissue damage.

The project arose from two lines of evidence. The first was work on creatine supplementation. Creatine is found mainly in the muscle, where it plays a role in energy storage and use, and this makes it a popular dietary supplement among athletes. There has been a lot of interest in possible use in therapy and it is currently under trial in a number of different diseases.

Studies in mixed groups of dystrophies (including some patients with FSHD) have found short-term improvements in strength and daily-life activities However, this is not well established for FSHD itself and there is a lot of argument about how it might work.

The second line of evidence is from recent work, funded by the FSH Society, showing that muscle cells in FSHD are particularly susceptible to a kind of damage called “oxidative stress,” which is caused by chemicals, produced in the body, called reactive oxygen species (Barrett, Tawil, Griggs & Figlewicz, ‘FSHD myoblasts possess reduced resistance to oxidative stress’ in FSH Watch, Spring 2001, pg. 70). The two lines of evidence are connected, as there is evidence that creatine supplementation protects against damage by reactive oxygen species, although how this might work is also controversial.

We are recruiting patients with FSHD from our clinic. To make sure that any effects we discover are specific, we will also study a smaller number of patients with myotonic dystrophy, an unrelated muscle condition. Each patient takes creatine for three months, and at the start and end we make three kinds of measurement: magnetic resonance imaging to measure effects on muscle mass and body composition; a muscle biopsy for laboratory measurements of both the tissue damage by reactive oxygen species and the muscle’s defenses against this; and assessment of effects on muscle strength and the clinical symptoms.

We think this project will be useful in several ways: it will provide evidence of the usefulness of creatine therapy over a longer time span than earlier studies; it should throw light on mechanisms of muscle damage in FSHD; it may suggest that other substance that reduce oxidative stress in muscle may also be useful as therapy; and lastly, the results will help in the design of future placebo-control trials. We are very grateful for the Society’s support, and look forward to reporting our results in due course.

Title: “Muscle damage by reactive oxygen species, muscle atrophy and effects of creatine supplementation in facioscapulohumeral muscular dystrophy.”

Investigator: Graham J Kemp, M.D. Institution: University of Liverpool This project is the first FSH Society Sam E. and Mary F. Roberts Foundation grant for research on FSHD and nutrition. “This is a pilot study designed to test the following hypotheses:

• (1) that muscle in FSHD shows evidence of damage by reactive oxygen species (ROS) in vivo;
• (2) that this is at least due partly to reduced anti-ROS protection;
• (3) that this is ameliorated by six months of creatine treatment;
• (4) that this also partially alleviates muscle atrophy, even in the absence of training; and
• (5) that this results in an increase in muscle strength and clinical indices. This is an open label pre-post protocol examining the effects of creatine supplementation in 10 patients with proven FSHD (and disease controls and unaffected controls). ROS protection and damage will be studied in conchotome biopsies of biceps.

Muscle atrophy and its effect on body composition will be measured by whole body quantitative magnetic resonance imaging (MRI).”

The Society is pleased to have Dr. Kemp and his colleagues at the University of Liverpool, one of the foremost institutions in the world in state-of-the-art MRI and Magnetic Resonance Spectroscopy (MRS), working on FSHD and applying these new methods to examine and quantify critical areas of research.

Re-printed from FSH Watch - the journal of the FSH Society of America

I dont really know much about them but i can tell u how my dad found out about them.

My dad was the first to find out about the splints when he went to his muscualr dystropy AGM,and he met these two brothers who had these foot splints.

So at our next specialist appointment my dad mentioned these to the doctor and he put us in touch with another doctor,who then put us in touch with the orthoctics depart in our area, phew.

We attended our appointment with this great guy called Jim who first ran an overall check,testing our arms and leg strength,and then ordered me a small splint and my dad with a medium,drew around out feet and sent them off.

About a week later we had our next appointment, fitted with the splints and away we go.

I took to them straight away and Jim was quite supprised at the speed of my adjustment. My dad however being alot worse than myself took abit more time and being in this condition they splints didnt work as effectively.

I have a toeoff splint for each leg as wearing one with my right leg didn't feel comfortable and I still dragged my left leg.

I've been wearing these for about 6 weeks now and my boyfriend is supprised at how fast I walk and a lot more effortlessly.

It is so much easier and less tiring as you don't have to control your walking by picking up for feet.

I hope this will be of help to other members.

You can find out more information at www.campscandinavia.se

Hello my name is Josie and I have just been diagnosed with fsh and it has taken a year for them to finally tell me what has been going on with my body.

I don't know of any history of this in my family and I have been in so much pain for the last year.

Unfortunatly I have a HMO insurance and they have to authorize everything and everything takes so long. The day I went to see the neorologist that gave me the diagnosis he told me well you have muscular dystrophy and at that time my fiance asked him if there were some steroids that I could take and he said nope that won't help and then we asked about creatine and he said nope that is not going to help me and we asked about pysical theapy and he said listen do you know Jerry Lewis and we said yeah and he said well he has been doing those telethons for 30 years don't you think if those things helped they would have done them.

And then I asked so that means that I will get weaker and weaker he said how old are you and I said 43 and he told me well figure that by the time you are 80 you will be twice as weak. I asked him so there is nothing that can be done and he told me that the letter behind his name said MD not G-D!!

I have never in my life been so upset as I was that it is something that I will never forget it was the worst day of my life and this so called Doctor spoke to me in that way (sniff sniff) So it has been really hard on me and I read as much as I can about all the research and medical data that I can find so I would like to hear from anyone that has been through what I am going through. And thanks for reading my e-mail

G-d Bless and many Prayers to all.

Josie

Our Conference 2003 was held on May 30 at The Orbital Centre, Garston.

We had an attendance of 50 of our members on what turned out to be the hottest day in May on record! It has been decided to hold our annual meetings earlier in the year, rather than October.

Martin Fielden our Chairman welcomed all. Jenny Versnel who is the MDC Research Manager was due to be our first speaker but unfortunately was unable to attend due as her father was taken ill. MF has been re-elected as a National Trustee of the MDC. Over the last year there have been major changes to the MDC constitution resulting in decreasing the number of Trustees. MF is on the Medical Research Committee. The MDC hope to raise their profile with regard to fund-raising and have elected people onto the MDC with experience in this area.

MF thanked Norman Jonas for our newly formatted newsletter.

Some of the questions sent in prior to the meeting were as follows:-
Q. Why is there no FSH funded research?
A. Because no one has applied for a grant over the last two years.

MF then explained the process for applying for a grant. The application is copied and two referees are selected for each application who then report to the medical research committee. The committee then discuss each application fully before grading them. In 2003 the committee had to review 23 proposals and they could only fund half the projects, Six clinical projects were among the ones funded. MF would like the committee to review their strategy and to encourage proposals for condititions where there is a lack of research eg FSH. This will not happen overnight however.

There is research into FSH being carried out in other countries. Colin Lucas (one of our members, present at the meeting) has been in touch with the FSH Society (USA). He had extracted a list from their newsletter of the list of researchers around the world and brought copies to the conference. He also brought some copies of the FSH Society Newsletter.

Q. Is Royal Jelly helpful to anyone with FSH?
A. We have not heard of any research to prove that it is. There are a few trials being offered to people but they have to pay to participate, all agreed that this was really a “sham”, any genuine research would not be charged.

Colin asked about a supplement, L Carnitine which he had tried for a month. MF said that he had tried it for three months but did not find it helped. This was based on research carried out at an Institute in California. The cost of the drug is £15 for two months supply.

There is also a treatment for FSH proposed using calcium blockers but we do not know the results. Any new drugs are best in properly controlled trials.

Treasurers Report – given to all attendees. No questions were raised.

MF introduced our first speaker Dr. Richard Orrell BSc MD FRCP, Senior Lecturer and Consultant Neurologist, Royal Free Hospital Hampstead.

RO thanked our members for volunteering for his research project, which is based on taking a needle biopsy from each leg, to enable them to look at the string quadriceps over the next couple of months.. This is a pilot study and the minimum of six is required.

He then proceeded with a power point presentation on FSH

One in 20,000 have FSH. It is considered to be one of the less severe Dystrophies. It is an autosomal dominant inheritance. Men and women are equally affected. Onset can be from infancy to in middle age. Clinical signs in 95% of affected by age of 20 years. It has a slow progression, around 20% may eventually require a wheelchair.

Diagnostic criteria: Onset in face or shoulder girdle. Spaning of extraocular, pharyngeal, ingual and myocardum. Facial weakness in more than 50% of affected family members. Autosomal dominant inheritance in familial cases. Evidence of myopathy in EMG and muscle biopsy. This is based on research carried out in Cardiff.

Other clinical features:
Asymentry, progression, contractures may be present. Hearing loss – less frequent in FSH. Retinal disease – retinal vasculopathy (Coats disease) affects the blood vessels at the back of the eye.

RO then explained how they carried out a nerve conducting study and DNA blood tests. The laboratory findings and the FSHD genetics and showed graphs showing the genetic changes. in FSHD. In 1990 the FSHD chromosome was found. They found the same information on Chromosome 4 and 10.

Common symptoms – foot drop, knee pain or effusion, low back pain, scapular fixator weakness. There can be pain associated with posture, also joint and muscle strain.

A large Abuterol trial was carried out in the USA but was unsuccesfull.

He explained about the Mechano Growth Factor (MGF), formed in muscles in response to exercise. As part of a trial, when MGF was introduced into mice there was a 25% increase in the muscle cross sectional area over a two-week period.

Questions were asked of Dr. Orrell

Q. Can it skip a generation?
A.The gene cannot. If the gene is not present it will not reappear.

Q. Can FSH affect the heart?
A. No known link with FSH and heart problems.

Q. Are digestive and breathing problems associated with FSH?
A. Not a common problem but constipation can develop due to lack of exercise if people are chair bound.

MF thanked RO for his presentation.

NJ mentioned that Nordic Care Services had set up a stand and had kindly brought along some of their equipment, he thanked them and asked all present to take time to have a look at the equipment.

NM also mentioned that he would like to produce another newsletter, however in order to do so he needs further articles.

After lunch MF introduced Terry Robinson and Martin Chianani who are Family Care Officers employed by the Muscular Dystrophy Campaign.

They explained that they are part of a team of 15 FCO’s spread over the UK.

MC works from Guys Hospital and his area covers S.Essex, Herts, Kent, North,South West central London.

TR works from Addenbrook Hospital in Cambridge and covers Bedfordshire, North Herts

They offer an information service and can provide practical and emotional support for FSH patients and their carers. They try and build relationships with families and professionals. They have around 800+ clients to look after. They educate the professionals and deal with anything including non-medical issues. They work closely with Neurologists. There are three muscle centres (1) Oxford (2) Newcastle (3) Hammersmith; together with a Scottish and Welsh network.

Q. How can we find our nearest FCO?
A. Call the MDC in London or alternatively the FSH support group sends out a list with each welcome pack.

The MDC are shortly to produce a new fact sheet on Counselling.

They discussed the Disability grant and the new rules for Local Authority grants. By July each Local Authority must have a definite policy. TR said each applicant should allow between eighteen months and two years after applying before a decision is given.

They discussed the psycological affect on newly diagnosed patients, from shock, anger, denial, all negative emotions, it is important that patients move on, look for help to bring them back to a normal frame of mind.

Equipment – ask for help with the purchase.

The Patrick Memorial Trust is a subsidiary charity of the MDC that will look at contributing to the purchase of equipment not covered by Statutory Services.

Local Rotary Clubs and Lion Clubs may also assist with part funding.

Disability living allowance – seek professional advice before completing the form.

Everyone should be aware of the Access To Work Scheme.

House adaptations – there is a list of approved Architects across the UK, the list is available from the MDC.

MF thanked TR and MC for the informative session

MF introduced Kevin Asheton who explained The Alexander Technique

KA explained how Frederick Mathias Alexander sought to address his own problems, he had a persistent vocal problem. He was an actor who kept losing his voice.

For seven years he kept minute observations of himself with mirrors.

The technique is based on a free relationship of balance. KA got everyone to stand and explained not a miracle but dealt with by function and design.

KA gave a demonstration using a volunteer of how by learning how to sit and stand it is possible to feel better.

MF thanked KA.

MF closed the meeting by asking for attendees to look for a community centre for next years meeting. The requirements are disabled facilities, including toilets.

The meeting ended at 16.00

To view the photographs of the Conference please click the "Annual Conferences" button on the main menu.

HISTORY OF THE SOCIETY AND IT’S FOUNDERS
Daniel Perez, Carol Perez (his mother) and Dr Stephen Jacobsen founded the FSH Society in 1991. Daniel and Carol Perez both suffer from FSH and are confined to wheelchairs.

Daniel studied cell biology at Harvard University in Massachusetts and spent two years working on the Human Genome project.

By the end of summer 1993 a board of Directors had been set up and accounts had been established.

In October/November 1993 the Society started to make contact with medical and charitable organisations and research groups. They also contacted other FSH support groups in Europe.

Daniel Perez is the Society President and is the only full time paid member. Carol Perez is an executive director.

FUND RAISING FOR RESEARCH
During 2001 and 2002 the FSH Society gave presentations to the US senate and the National Institute of Health to press the case for FSH research funding.

In January 2001 the National Institute of Health set aside 5-15 million dollars for muscular dystrophy research. The latest FSH Society publication quotes that at the end of 2001 $4 million had been allocated solely to FSH research.

The MD care act was also passed at the end of 2001. This requires their government health agencies (i.e. the NIH) to place more emphasis on muscular dystrophy research. Co-ordinating and advisory committees were also required to be set up.

Daniel Perez has recently been invited to serve on the co-ordinating committee.

The Society also receives various grants from corporate and private sources, which they allocate to FSH research projects. These are listed in their latest publication.

WEBSITE
They have a website, which includes electronic copies of their publication called FSH Watch going back to spring 1994.

The website has a link to their Bulletin Board and chatroom. The chatroom contributors are mainly from the U.S, but you see postings from other countries including Australia , Canada , The UK and even Turkey.

Types of Postings include:
Latest interesting releases from around the world, often with a hyperlink to the related site or publication. Stem cell therapy and gene therapy articles seem to come up regularly.

Experiences with supplements. Creatine , Carnosine , Q10 & Carnotine are mentioned a lot.

Experiences of life with FSH.

Tips for getting over problems, frustrations with FSH i.e. falling over, access problems and even people letting of steam etc etc.

The web address is www.fshsociety.org

This is also listed on the FSH support group website under “Links to Other Sites”.

PUBLICATIONS
They put out a publication called FSH Watch approximately every 18 months, plus smaller newsletters once or twice year.

FSH Watch is a comprehensive publication giving an update on all research projects (US and International) plus a list of current researchers and the subject they are studying. It also gives information on funding and other FSH support groups around the world, including Australia, Canada, the Netherlands, France, South Africa and the FSH Support Group here in the UK.

Some of the articles in this publication were also featured in the January newsletter of the FSH Support Group.

MEMBERSHIP
Membership fee is $35.00 (approximately £21-£22).

Colin Lucas

At the conference in May, I promised Lorraine an article for the next FSHD Newsletter, so I thought I had better keep my promise. It's 7 pm and I'm sat outside a pub in a neighbouring village with a pint by my side and the sun shining on me - can't be bad can it? Then again I think back to a few minutes earlier when I walked into this pub (that I hadn't been to before) on my crutches, and found out that it was not in the slightest bit disabled friendly.

There were six steep steps down to the bar from the entrance area and then of course, six steps back up again. The pub was quite busy and I really hoped that the barman or one of the customers would offer to carry my drink up the steps for me but nobody did.

Normally, I would ask someone for help but everyone seemed to be talking and enjoying themselves and as I was a stranger to the place I didn't speak up - more fool me! Anyway, I just about made it back up the steps without falling down and only spilt a few drops of my beer. Sitting here now in the warm evening sunshine I am glad that I made the effort.

My first inclination when I walked in the pub was to walk straight out again and even now I am psyching myself up to go back in and get a second drink.

All the above brought my disability sharply to the front of my mind. Normally I try to lead my life in disabled friendly environments, but of course, it is not possible to do that all the time. Overall, I really do take my disability `in my stride', so to speak, but there are just occasions, like this evening, when I dwell on the fact that I will be disabled for the rest of my life and possibly to a greater degree than I am now.

Mind you, when your hospital Consultant tells you that if you take regular exercise and keep the weight off you shouldn't eventually become wheelchair dependent, it is a great motivator.

So it is to the gym and swimming pool I go twice a week and I gave up cakes, crisps, sweets and desserts a long time ago.

Luckily, I don't miss them, even biscuits which I used to eat by the truck load; I have managed to do without. Instead, of these goodies I eat fresh fruit and one low fat bio yoghurt a day.

Mind you, I don't skimp on my main courses as I have always had a healthy appetite. Luckily for me, cutting out the sweet stuff has done the trick.

The regular exercise has not been so straightforward. As you will all probably know with FSHD it is good to do some regular exercise but not too much. Just as much harm can be done by doing too much exercise as too little. Getting the balance right I have found really difficult, I have always been someone who has pushed myself to the limit in whatever I have done and it has been so hard for me to change my mental attitude to this. My wife was forever nagging me, poor thing, not to overdo it but more often than not I ignored what she said. Matters came to a head after she was involved in a road traffic accident (not her fault) and was left with serious permanent disabilities.

Even though my wife relied on me for most of her care I still couldn't change my `push myself to the limit' mindset and in the end I had to have a course of counselling to help me, and more importantly, to help my wife.

The counselling did make a difference although sometimes my wife might not agree. One thing that came out of the counselling was that I really missed being employed; I took ill health retirement in 1998. I now do voluntary bereavement counselling which has helped me feel more useful. Perhaps earlier voluntary work I had done hadn't been enough for me.

The worst part of having FSHD, for me, is getting tired so easily. I feel I manage to cope pretty well with the physical disabilities; I just 'hate' feeling tired all the time. 'The spirit is willing but the flesh is weak.'

This means taking afternoon naps, something very alien to me, and going to bed early at night. However, if this means that I don't get so tired and have a bit more energy then it is something I will have to do. I reckon, that 95% of the time my wife and I are positive about our disabilities, it is only very rarely that we get down, and then we manage to jolly each other up again.

Just a couple more points, before I go and collect my wife from her computer class.

Whilst I have been sat outside the pub, a middle aged lady and a young man possibly her son came out, he clearly had some mental difficulties and it puts things into perspective really. Yes, I had difficulty getting down to the bar, but at least I am able to sit here and write this piece without needing help from someone else.

And finally there were a nice looking couple going into the pub a short while ago so I plucked up the courage, explained I was disabled and asked them if they would mind fetching a drink for me as the steps to the bar were difficult for me. They didn't seem to mind at all.

The moral of this story is, don't be afraid to speak up and ask for help when you need it, the vast majority of people will help you.

Geoff Manning

I am from the United States and came across your website while scouring the internet for information on FSH.

I recently have experienced problems consistent with FSH, and after seeing a neurologist, have had a blood sample sent off for the DNA test that confirms this disease.

This is a long process, and I was thankful to have found your on-line information brochure. It is much more in-depth than most other articles for the lay-person that I have found... I wanted to thank you for posting it.

Again thank you for the website!

Chris

We planned to hold our 2004 Conference in May in the North of England, and when the MDC chose to hold it's Northern Conference and ACCM in Leeds on Saturday May 15th it was a good opportunity hold a joint meeting. This is not a permanent arrangement, as no doubt other condition specific groups will be involved in future.

The Conference programme was included with this newsletter as well as a booking form.

The programme includes both FSH and other MD topics, with opportunities for questions, as well as a short business meeting of the FSH SG and MDC.

The venue is an excellent one with facilities for the disabled, also overnight stays at a special rate can be booked directly with the hotel. Lunch is included with registration and there is to be a programme of children's activities, something that the FSH group has not been able to provide in the past.

We do hope that all FSH members and their families will join in on this special occasion. This is a good opportunity for FSH members who are not MDC members to learn more about the MDC and vice versa.