November 2006 - New European HSP Website

Let me introduce you to some of the very hard working and enthusiastic Europeans who have made all this possible:

Rudi Kleinsorge - Germany

Rudi has been HSP affected for 20 years and was diagnosed 6 years ago.  His twin sisters are also HSP affected.  He used to be a civil engineer, a job that he loved, but unfortunately he had to retire due to his disability.    
Rudi decided to “work” in HSP.  There are two groups in Germany.  The first is the Tom-Wahlig-Foundation (=TWS).  This organisation has been in existence for over 8 years now and Rudi is an active member of this group.  The TWS works successfully in initiating and financing HSP-research projects.  Today projects of the TWS run in Germany, England, France and in the USA.
The second group in Germany is the “HSP-Selbsthilfegruppe Deutschland” (HSP-Support Group Germany). Rudi is a founder member of this group.  Incredibly after only two years of existence they already have more than 400 members (“Where are we going wrong Rudi?”).  This group offers support to the sufferers and information about the disease.
Rudi says the following words about HSP-Europe:
“In the HSP Group Europe we see the potential of getting and giving good information concerning both parts: a) Living with HSP b) Information about HSP. We hope that this group will grow in years to come so that a lot of other countries will follow this idea”.

Philippe Grammont – France

Philippe inherited the condition from his father and one of his brothers is also affected.  Not much was known about HSP in France at the time when Philippe was diagnosed and this caused feelings of isolation.  This is why Philippe decided to create ASL (the Association for Strümpell-Lorrain) in 1992, to bring patients together (I believe that the French generally refer to HSP as Strümpell-Lorrain Syndrome).  Philippe is also active in other organizations associated with rare diseases and is very keen to promote European cooperation.

Gunnar Flåtten – Norway

Gunnar is 58 years old and lives in Oslo.  The first signs of his HSP occurred about 6 – 8 years ago when he was aware he couldn’t run to catch a bus, but initially thought little of it.  During his childhood both his mother and brother had walking difficulties but their diagnosis was unclear as not a lot was known about HSP in Norway during those times.
The Norwegian Group is called NASPA and one of the founders was Dr Sverre Flåtten, Gunnar’s brother.  Sverre was the original “leader” of the group mainly because of his competence as a doctor, but also of course because of his HSP experience within his own family.  Gunnar was elected leader of NASPA when his brother Sverre had to temporarily move to Stockholm.  I get the impression from Gunnar that it is early days in Norway in terms of HSP Support Groups, and any help that can be obtained from European friends will be greatly appreciated.

Fernando Gonzalez - Spain  

Fernando is not HSP affected himself, but his wife, son and brother in law are affected.  He used to be a reserve Spanish Navy Captain and was in the Navy from 1960 until 1992.  Similar to Norway and indeed the UK, HSP was a bit of a mystery a generation ago and it wasn’t until Fernando’s son was 12 that the condition was correctly diagnosed.  Via the internet, Fernando discovered the French ASL (run by Philippe), and after joining up, received a lot of useful information which was unavailable in Spain at the time.  Some years later when a number of Spaniards were associated with the French organisation, Fernando decide to form a Spanish HSP group.  In 2002 AEPEF was established meaning Association Espanola de Paraparesia Espastica Familiar.  The main goal of the group was to expand knowledge of the condition to both patients and the medical profession.   
Interestingly, because Spanish is spoken worldwide, AEPEF attract people not only from Spain, but also from Portugal, South America and Argentina.
Anyone can now access this website at:
www.hsp-info.eu
The website is still under development and the information available will become far more comprehensive in coming months.  You’ll notice that there are links to each of the individual European groups, and I must emphasise that there is no intention to amalgamate these groups.  We are all going to remain individual, but now begin to help each other more by sharing information.
Certain European countries are conducting comprehensive research programmes and it is hoped that we’ll all now have better access to information concerning this.  Those of you without internet access, please do not worry, as any breaking news will always be published in our newsletter.
As with our own UK website, there is also a forum facility within the site, but the advantage of using this new one is that the audience is potentially much larger.  It is estimated that throughout Europe, members of all individual groups total over 1500, and this number is expanding rapidly.  The French currently have 650 members, Germany has 400 members, Spain has 175 members and Norway has 60 members. With this in mind, once members have become aware of the new European forum, when messages are posted asking for help or advice, many interesting replies could be received.  
You do not have to register to use the forums, but I would recommend that you register so that you become known to other users.  Registered users can also send private communications to other registered users.
I’m sure you’ll all agree that opening up Europe to the HSP community, has to be of benefit to all of us.  One thing I am very confident of is that we’ll now have more news from FSP research organisations to report within future editions of this newsletter.

Ian Bennett

From the Chair

Hello everyone
No sooner has the sun disappeared then its time to dig out the heavy jumpers and turn on the central heating.  Are the seasons almost levelling out or is it just a child’s memories that recall a good old white Christmas?
I do hope you’re all keeping well, which brings me to say a few words on Mike Fawcett, our treasurer.  He is feeling a bit out of sorts at the moment and would definitely welcome a call or a card from friends.  If you would like to get in touch, Ian or myself we will gladly give you his contact details.
I have to thank Dave Harris for his work on updating our Information brochure, mailed out to everyone last week.   I do recommend you take time to read it front to back.   We all have snippets of information and to have it all available in one document is a great benefit, especially if you are new in confronting HSP.  Please let Dave know if you haven’t received one.  In a similar vein, thank you so much for those of you who have returned your transport survey cards.  Could I ask you to especially participate if you generally attend meetings?  This will ensure we have a truer picture on how we might help.
Our 2007 AGM is well on plan for the third Saturday in June, again at Warwick, with just the third speaker to confirm. Focusing on our awareness programme, you might like to know a mail shot to 80 or so Neurology units nationwide is imminent, hopefully including a poster for display in out-patients departments.  Perhaps we could all ask our hospitals to display one when attending clinic? I will happily give advice on best practice here. Amongst our aims and objectives for the coming year is a need to look at the coordinator role.  A summary of events to date and some observations for the future is shown on page 4.  Perhaps you all have a better feel on how it might operate more effectively in certain regions? Have a read, and let’s all work together on this project.
By the time you read this I will have enjoyed another Southern area social with Jane and the gang.  I’m really looking forward to seeing everyone again.  As usual please feel free to contact me at any time.

Keep well      
         David

The Secretary’s Spot

Lots of you have said how much benefit you have got from talking to others at meetings.  We thought we would try and find out if there were ways we could help more people to get to meetings.  We also recognise that others have said that meetings are not for them.  These were the main reasons behind the questionnaire form we sent out recently with the Information Booklet.  We wanted to “quantify” these various views.
So let me share some Survey Results:  Firstly over 30% of our members responded.  That is a very good response - many questionnaires don’t even get a 10% response.  Of those who responded:
15% said they were not interested in meetings
40% felt meetings were too far away from them
46% felt a local meeting would be easier
14% said that getting a lift might help them attend
So we will be spending some time and effort to see how we can help more local meetings to take place and to investigate means of assisting those who would like some help with getting to meetings.
On to other matters: the second version of the Information Booklet has been well received.  We still want to collect other tips and hints for general publication - we can put them into the Newslink, and add them to the booklet next time we issue it - so don’t be shy - let us know what you find out.
One topic we’ve not mentioned is that of making the most of our personal finances.  There is a very good web site run by Martin Lewis - a journalist who specialises in helping people get the most for their money.  If you have not yet heard of it, I recommend a visit to: www.moneysavingexpert.com.  The site contains lots and lots of information about ways to spend less and still have the same quality of life. (Example: we saved £200 per year on House Insurance).
It really is worthwhile registering for the free weekly email which contains the current saving topics.
Well I’ve probably gone on for way too long now...

Dave Harris

From the Helpline

Hi Everyone,
I hope everyone is ok in the cold weather, it makes most of us who get pain, an awful lot worse, so do what you can to keep yourselves warm.  Those of you in wheelchairs, get yourselves a hot water bottle and put it on your lap or between your legs, it does really help from getting the cold in your legs and then not being able to get warm once you get home.
This also helps with those of us that get back ache, place the hot water bottle behind your back when you are sitting down.  An electric blanket is a wonderful investment; put it on your Christmas list.  Please give me a call if you have any problems.  Warm regards to all for Christmas, may you all have a happy one.

Stephanie xxx

FSP Regional Coordinators:

The Story so far:
The coordinator role was introduced some four years ago, its prime raison d’etre being to assist the integration of new members into the group. Hand in hand, came the sharing of pertinent support information via telephone and of course at socials as appropriate.  Hopefully it also offered a convenient doorway through which both negative and positive feedback could be conveyed to the committee.
It seemed logical to simply allocate counties to regions and then seek an active member within it.  There it seems to fall down.  Clearly members’ proximity or otherwise creates transport problems and of course not everyone necessarily wants to meet others with their condition.  Membership density within a territory must play a significant part in attendee numbers at meetings.
I have to thank all current coordinators for their time and positive endeavours through out this time, in particular Liz & Ronnie Ferguson, Jane Bennett, Ray Exley and Ian Bennett.
And so to moving on:
Can I suggest we consider a system driven by yourselves and not necessarily a scheme we have imposed; exclusively on those areas not covered by the above?  Your information brochure will make this a little clearer.
There may simply be a case for us to get in touch with anyone nearby.  If you personally would like to learn or share experiences with your colleagues we can happily provide details.  This short paper will resist the temptation of banging the meetings drum; other than to say they are mutually beneficial.  The territories currently standing can be reduced in size; though of course a meeting can always be arranged without a coordinator necessarily attending (distance).  Territories may develop better naturally and therefore be sustainable.

David Pearce

Help Needed

It is our belief that there could be several thousand people with HSP in the UK and we’d like to let them all know about our group.  We plan to send out a letter to phsio departments and hospital neurology departments and any other similar places.  We will send a small amount of publicity material with the letter (a poster and some membership forms).  This will only be really effective if we can contact a named individual in these places.  Can you each let me (Dave Harris) have contact details for the neurological specialists and institutions you attend?  You can send by email (This email address is being protected from spambots. You need JavaScript enabled to view it.) or snail mail (18 Lodmoor Avenue, Weymouth DT3 5AF).  What I need is name, title, department address.
We can then build up a mailing list from your information and information from other contacts.
Also if you could put a poster up during your next visit, please contact us and we'll send you some material.
Many thanks:           

Dave Harris

Italian Mice

Approximately 30% of HSP cases are inherited in as autosomal- recessive traits.  One of these recessive forms is due to loss of function mutations in the SPG7 gene, encoding paraplegin.
Researchers from the National Neurological Institute in Milan, Italy have used gene therapy to delay onset of HSP in mice affected by mutations in the SPG7 gene.
Mice that were bred to have little or no paraplegin developed progressive degeneration in their axons.  I have not read this in the journals, but perhaps these mice walked with a scissor gait.
Injections of paraplegin were shown to stop the degeneration and improved peripheral nerve cell activity.
One single injection of paraplegin before onset of symptoms was able to save axons from degeneration and consequently improve the mobility of paraplegin deficient mice for up to 10 months.
This study provides evidence that gene transfer may be an effective therapeutic option for HSP patients affected by mutations in the SPG7 gene.
For more detailed information on this study, visit:
http://www.ncbi.nlm.nih.gov/entrez/query.fcgi?cmd=Retrieve&db=PubMed&list_uids=16647881
Please don’t get over excited by this research at this stage.  It is not relevant to most of us and certainly not relevant to the most common form of HSP which comprises mutations in the SPG4 gene (spastin).  Apparently French researchers have a spastin mouse, but no news on this has been published yet.

Christmas Cards

A very late effort has been made to produce a limited quantity of FSP Christmas Cards to raise funds for the Group.
£24-00 was generated for the group by selling Christmas cards at the recent Region 1 & 2 afternoon tea session at Milford.  A big thank you to all who purchased cards at that meeting.
Cards will again be on sale at the Region 4 meeting on November 25th and a few are available by post on a first come first served basis due to the limited supplies.  Each card is 20 pence, but by post they’ll be 9 cards for £2.  It may appear your being short changed, but it helps to cover postage costs.
Maybe next year we can be more organised with this project and set about it a little earlier.  If anyone can offer some assistance or ideas with this, it would be much appreciated.  It appears to be a great way to raise funds for our organisation as many members are keen to support in this way.

Useful Information

Can’t make the loo in time?
Try URIBag
Anyplace - Anytime the URIBag takes virtually no space until you need it
Then you have over 2 average bladders full of capacity at your disposal!
Folds away into a hard plastic case (8 x 4cm male version, 8 x 6 x 4cm for female version) which is small enough to fit into a shirt pocket, or handbag so its massive 1100ml capacity is instantly available for use wherever you need it.
Ideal for use in the car, plane or coach. Also for use in a wheelchair as its small dimensions mean it is easy to get into place.
For further information, visit:
http://www.legbags.com/urinals.asp
Holiday Recommendation
Alan Walters from Region 4 regularly stays at a self catering cottage in the New Forest which is fully accessible and wheelchair friendly.  Alan says that lots of disability equipment is provided free of charge, including a wheelchair and tri-walker.  The cottage is located in Pennington, on the edge of Lymington, two miles from the open forest and close to the coast.
For bookings or further information contact:
Roger or Mary Lewis
9, Lodge Road,
Pennington,
Lymington,
SO41 8HH
Telephone: 01590 673141
www.ourbench.co.uk

Members Letters

FSP & I.B.S. or Coeliac Disease
Having been treated for Irritable Bowel Syndrome for 10 years, I have recently been diagnosed as Coeliac.  Following years of embarrassing and painful symptoms such as diarrhoea, abdominal discomfort and general fatigue, it is such a relief to find one solution to my on-going health problems.
When I was 18 I was referred to a gastroenterologist and after ruling out other causes, was told it was I.B.S.  I have tried everything from hypnotherapy and Aloe Vera to anti spasmodic drugs.  Nothing proved effective.  It was not until I returned to my doctor about some other complaint, and mentioned I was still having problems with my stomach that things began to change.
He sent me for more blood tests and despite testing negative once for celiac disease, this time my tests came back positive.  Although rare to have a negative followed by a positive result, it can happen and can only be fully confirmed by an endoscopy.
Since being referred back to my gastroenterologist and starting on a wheat gluten free diet my symptoms have improved considerably.  Although at times the diet gets me down (no bread, biscuits, cake etc) the benefits of being pain free are definitely worth it.
Associated problems with coeliacs disease can be severe anaemia and in long undiagnosed cases, pins and needles.
A link between celiac disease and M.S has been suggested and although further factors and medical examination must be substantiated, why not a connection with other neurological conditions such as FSP?
I wonder if this applies to any other members?  If so perhaps other people could write in to the newsletter sharing their experiences and who knows?  We may have established a further significant development in the many forms of FSP
Information on celiac disease can be found at www.coeliac.org.uk

G C - Region 3

 The following letter was submitted to our email group:
Firstly, a thank you to those members who replied to my initial e-mail enquiring as to their experience(s) in connection with genetic counselling/testing.
My husband and I attended at Royal Blackburn Hospital earlier this month for an appointment at the genetics clinic.  Blackburn is one of the hospitals that Central Manchester and Manchester Children's University Hospitals and Trust Regional Genetic Service use for seeing patients.
We saw a Doctor H Kingston who is a consultant clinical geneticist.  Unfortunately, Philip Greene the genetic counsellor was off on paternity leave so it was not possible for us to see him during this appointment.  Dr Kingston was really lovely and began by taking details of my family and tree and personal (health) history.  She explained in a fairly easy to understand way about genes and how they worked and how it would be possible that I'm affected when no other family member can be traced as having the problem.
Rather a lot of blood was taken from me (I thought I had a lot taken before, but this was certainly the most - large syringe and a tube to take the blood from the needle in my arm to the syringe - quite impressive) for two initial gene tests - they usually start with the most 'common' gene and work back from there.  My blood was to go to Sheffield for testing and the first result should be back within a couple of weeks but the other test will take several months.  If nothing is revealed from those then further tests will be done.
Dr Kingston did advise that it may be that it would not be possible to find out the gene in question as not all of them were able to be tested for.  Apparently developments are made all the time in respect of gene testing and it may be that somewhere in Europe would discover something and my blood might get sent there.
I was given her telephone number at the Regional Genetic Service of St Mary's Hospital in Manchester and can ring if I have any questions or enquiries in the future.  When all the possible tests have been done, my file is kept in the hospital records and is reviewed every couple of years to see if any developments have been made in connection with relevant genetic testing which we were very impressed with - you are not just seen, tested and then forgotten about.  The only reason is it every couple of years is because the clinic deals with many conditions which are gene related but Dr Kingston said that it specifically relevant progress was made for a certain condition which they discovered, then certain files would be reviewed at that time.
It was a most interesting appointment and was so nice to actually be given a telephone number and told to ring with any questions etc which I may have in the future and to meet a doctor who was actually very interested in me and my problems.  Hopefully, if a 'bad' gene is found, and hopefully if not, we may get to see the actual genetic counsellor when he returns from paternity leave.
I have also, in the last few months, had awful trouble with my back in that it has 'gone' twice and for no apparent reason.  I have had trouble before with twinges which have then developed into a bad back but usually after sitting or a night's sleep, it has felt easier.  But the last two times when my back has just 'gone' nothing has relieved it which has been very difficult to cope with.  The pain initially is usually in my lower back but this last week or so it has move to the lower right and has affected movement in my leg, due to severe pain in my back.
Does anyone else suffer with back pain?  How do you cope with it?  I already take pain killers which are suppose to help with back pain and my family think I should create more to my doctor etc but the last time I went I was just given yet more pain killers which didn't seem to make any significant difference and referred me to physio, but by the time my appointment came, it had got easier and my physio said everything felt ok, even though I was still getting twinges at times.  The trouble is I am not really a complaining kind of girl!
Thank you in anticipation of replies/assistance to try and help me cope!

Z G – Region 9

Dear Dave (Harris)
Thank you for the excellent information booklet.
You asked us to let you know about any useful information, so with reference to the section about the RADAR key, we were able to purchase a key from our local Abelworld shop for £2.99. More importantly, during an outing, we wanted to visit a cafe within an enclosed area of children's playground, etc. but struggled to get a scooter into the area because of special gates. Once in, the proprietor of the cafe said he thought that the additional gate had a special key fitted for disabled. We tried the RADAR key and it worked! Obviously, the RADAR locks are used for more than just disabled toilets, a fact that may be useful to other members.
A possible topic for future publication could be on scooters and scooter insurance? The Disabled Living Foundation has some useful fact sheets on all sorts of topics (we have the fact sheets on scooters and powered wheelchairs).
We enjoy and find it useful to meet with other members at group meetings/AGM. From our point of view, being in group 6, it is just as easy to travel to group 9 or group 7 area as it would be to travel to the south of group 6. Therefore, would it be possible to "belong to" group 9 and 7 for information and any group meetings, even if this meant a supplement to the membership costs. Please put this idea to the committee.
Kind regards

P and T R

Dave’s reply
Thank you for your recent letter - I’m really glad that you like the booklet.
The extra information about the RADAR key is very useful, and we will try and get it included in the next issue of the booklet.
I have talked with our Membership Secretary, Ian Bennett about the “attendance at meetings” point.  Ian has associated you with Region 9 because that is nearest to you.  To confirm this I enclose (with Ian’s permission) a list of the members in Region 9.  Carol McGowan is the Co-ordinator for Region 9.  For completeness there is also a list of members in Region 6 on the back.
Irrespective of Regions, we want members to feel free to attend any of the Group’s meetings.  We would ask that members check beforehand with the meeting organiser - just to ensure the right space is available for the expected number.
Having said all that, Ian and I were discussing how we could best get this information to our members.  One idea was to publish your letter, together with a reply, in a future issue of Newslink.  Before we do that however we would want to be sure you had no objections.  Please let me know if you are happy with this idea.  We will not do anything unless we hear from you.
Thanks again for the information and your comments.

Dave Harris

Dear All
Very much prompted by a Newsletter article by Ian Smith 3-4 years ago, I worried that my ability to drive my (Automatic) car by foot-controls was beginning to become “sluggish”….and, indeed, 2 years ago I made the decision to convert my car to Hand Controls.  I can assure you all that it was one of the wisest moves of my life!  
After the rather difficult initial “settling-in” (where, particularly, parking is a little difficult to adjust to) I now drive far more efficiently and safely than ever before.  So I can only recommend this to anyone who has any fears as to their own diminishing ability with foot controls; but please don’t be “turned-off” by my comments about the initial difficulty: you can obtain lots of advice and even professional guidance.
There are a number of firms who produce the equipment, and also plenty who fit the adaptions.  There is, I believe, quite a variety in cost.  It is not my position to advertise, but I can at least tell you the firms whom I have used.  The range of equipment may also vary according to the physical needs of each person.
I use:
a) A throttle and brake hand-control.  This is a simple push/pull design, where you pull the control handle towards the steering-wheel to accelerate, and push it away from the steering-wheel to brake.
b) A steering ball, which is a mushroom-shaped knob which is firmly fixed to the steering-wheel: It can turn on its own axis, enabling the driver to hold it in the left hand and perform all of the steering operations while the right hand is in absolute control of the throttle & brake handle.
When I first had these adaptations, I also had a Pedal Guard, which protected me against accidental slippage of my feet onto the foot pedals.  (The foot pedals always remain in place after the adaptations, because long rods are fixed between the pedals and the push/pull handle of the hand-control.)  Anyway, I gave up the use of the Pedal Guard, as my lower limbs are so rigid when I’m sitting in the car, and when I changed my car under the Motability Scheme at the end of April, I did not seek a new fixing when a) and b) were re-fitted.
The re-fitting of my hand-control unit and the transfer of my steering ball cost me £367, but I heard of someone recently being charged £600 for very similar equipment.  I guess that mine, with inclusion of a new steering ball, might have been chargeable at a little over £400.  So get some quotes and compare for value.
Let me add one very important comment:
If and when you are purchasing or leasing a new car under the Motability Scheme it has recently become possible for hand controls to be installed at the time of its purchase, with the cost being borne by Motability (up to certain limits).  You would have to ask your dealer at the time.
There is a range of other items which are available as optional extras, according to the driver’s needs, such as an Integral Indicator Switch, an Integral Horn Switch, a High/Low Beam Switch…and some others, I believe.
 My equipment is manufactured by Jeff Gosling Hand Controls Ltd (0161 430 2151) and the fitter was Philip Chapman Car Care of Southampton (02380 333 222).  I never needed to consult Goslings, as the fitter gave me all of the advice and supplied the equipment, based on his own physical assessment of me and the car.
If you want to assess your own needs, and you live too far away from Southampton, there are widespread operators who could deal with advice and fitting.  The best source of information that I can think of is Mobilise Organisation, which is the name of the now merged Disabled Drivers Association and Disabled Drivers Motor Club.  You could join the Mobilise Organisation (01508 489 449) at an annual charge of £14, and one of the benefits of joining will be the receipt of free monthly magazines in the name of Mobilise, which provide its readers with a wealth of knowledge for the disabled driver.
Finally, I have to say that you should not rely entirely upon my advice or my descriptions of the equipment; the only certain way of proceeding is to get professional advice, and see for yourself with the experts.

John Moore – Region 1

 Forthcoming Events

Aviation Opportunity
The November 2005 edition of the Newslink described Ian Bennett’s exciting introduction to flying.  Ian is now a member of The British Disabled Flying Association, and he regularly flies from Lasham airfield, Hampshire.
The British Disabled Flying Association is very keen to offer disabled people the opportunity to experience the thrill of flying in a light aircraft.  They have generously offered members of the FSP Group this opportunity.  
It is likely that flights would be for a duration of around 30 minutes and a charge of around £40 per front seat passenger would be implemented and a reduced rate for rear seat passengers.  This is approximately 1/3 of the rate one would usually pay at a flying club.  All flights would be operated with an experienced instructor as pilot in command.
Lasham airfield near Alton, Hampshire would probably be the venue mainly because of their experience in dealing with disabled aviators.  Lasham has excellent catering facilities and has disabled access and a disabled toilet.  The date of this event has not yet been allocated, but it’ll almost certainly be next Spring.  The success of this prospect will depend upon numbers.  
Please contact Ian Bennett if you are interested in this.