September 2013 - Extra Reward for Extra Effort

Each year Flying Scholarships for Disabled People (FSDP) awards a special trophy, the George Stewart Memorial Trust Award, to recognise one scholar's particular efforts. Every scholar works hard during the flight training process and the benefits soon reveal themselves in each one.
Several years ago the FSDP scholar selection process took place at Biggin Hill Airport.
George Stewart was an air traffic controller at Biggin Hill at the time, and was greatly impressed by the applicants and the aims of the Charity. He left FSDP a small legacy in his will and the George Stewart Memorial Trust award was born.
However, it’s often obvious that someone in particular has come that bit further in their personal journey. During the training Trustees visit the scholars at their various flying schools and sometimes the positive change in an individual is immediately obvious, which is additionally backed by their instructor's observations.
Mark Tomlinson is one such student. He told us that prior to learning to fly, he felt invisible especially at work amongst his colleagues and bosses. Flying endows a pilot with a very special form of confidence, most adults can drive a car, but not many can fly! His determination to overcome his fears, from his HSP condition and the way it impinges on his everyday life, to finding that initial special courage to take to the air did not go unnoticed.
And in recognition of Mark's enormous mental as well as physical efforts, he was awarded the George Stewart Memorial Trust award. Mark is not longer invisible!

Susie Dunbar FSDP Trustee

Chairman’s Column

I seem to regularly begin my column with an apology for the delay in producing each particular issue of our newsletter. On this occasion I’m going to use school holidays, family visitors and a gorgeous summer as my feeble excuse. Just as I was about to put pen to paper, along came the Bournemouth Air Festival where I thoroughly enjoyed three consecutive days in the sunshine watching amazing air displays highlighted by the Red Arrows and the Battle of Britain Memorial flight. All of this was at no cost. The disabled parking facilities are very good and for anyone who hasn’t been I would strongly recommend this event. I don’t believe there is a better venue for an air show in the UK.
Our AGM back in June now seems a fair time ago but as always it was brilliant seeing so many of our members enjoying the day. The minutes of the AGM can be found in this Newslink as can a summary of all four presentations. I’d like to thank Adam Lawrence for providing us with such accurate summaries of these presentations. Physio Function also attended the AGM and offered free assessments where members had the opportunity to try different FES systems.
On the subject of the AGM, nobody came forward to offer help or was nominated to join the committee. One of the main purposes of the AGM is to structure the committee for the year ahead and we currently seem to be very stagnant at committee level. I believe that for any organisation to move forward, new blood is needed from time to time. I for one could certainly do with some assistance as I don’t have the stamina I had when I first got involved 10 years ago. If any of you would be interested in helping out the committee would be very keen to hear from you.
I’m looking forward to the next meeting in Ashburton, Devon on October 26th. Professor Jon Marsden will be joining us to update us on the work he is doing at Plymouth University regarding the effects of hot and cold temperatures on individuals with HSP. I’ll be emailing invites to all members in the South West nearer the time. Another meeting for our members in the North West has been organised on November 16th. We’ve also organised a meeting in Norwich on October 19th for members in Region 5 to get together. Details of both can be found on page 8.
Later in this issue you’ll read about Peter Bateman’s Ukulele concert in Porthcawl. I hear that Peter is a bit of a star on the ukulele and I thank him for organising this fun event and raising over £300 for the Group.
Further thanks to everyone else who has raised funds for the HSP Support Group over recent months. To name just a couple, LP jumped out of an aeroplane and raised in excess of £100 and the Enfield Grammar School Lodge have very generously donated £750.
I visit our Facebook group (Hereditary Spastic Paraplegia’s Unite) regularly and I notice that it now has more members than our Support Group. It’s certainly a great resource for sharing information and seeking advice. If you haven’t yet discovered it, please sign up and make the most of all it has to offer.
Please keep the newsletter material rolling in and I look forward to catching up with many of you at forthcoming meetings and events.

Ian Bennett

The search for greater knowledge and more effective drug treatments for HSP

In the neurogenetics clinic in London we diagnose and manage the treatment of many patients with HSP. Regardless of the genetic cause, one of the commonest questions asked is why are there not more effective drug treatments for HSP that stop progression or reverse this disorder? This is quickly followed by how rapidly will the disorder advance and what will the future hold?
A major bottleneck in the improved understanding, discovery of promising treatments and biomarkers of HSP progression is the development of a detailed patient registry and blood sample biobank. A registry would allow patients to be grouped by the gene that causes their HSP and therefore, provide a greater understanding of how HSP progresses and attract research funding and pharmaceutical companies with specific therapies to investigate. The registry of HSP patients that are willing to be contacted and informed about trials to see if they are interested in participating will be essential for future UK drug trials.
A second, and equally important question is how quickly will each genetic type progress and what disabilities will patients develop. This is known for some genes but little is known for others. In addition, as part of the registry we would have all blood results and MRI scans to follow progression and also blood samples as part of a biobank that would allow biomarkers to be tested by researchers and pharmaceutical companies. A further benefit to patients of the registry is to make sure they have had the correct investigations carried out.
The development of a registry and biobank is an important task and will depend greatly on the help from patients and the HSP society.
The HSP registry and biobank would consist of:
1.Details of how each patient’s HSP started and progressed with a yearly rating scale.
2.MRI, other scan imaging results, along with the results of other tests
3.Results of genetic tests and detailed family tree
4.A blood sample from each patient for DNA, serum and cells. This would be done for an accurate diagnosis (particularly important) and every three years to develop markers of progression.
The registry would be administered at the National Hospital for Neurology and Neurosurgery and we would aim to enrol as many patients in the UK as possible. Patients would have a unique number and collaborators and other researchers would only be able to see this number, whereas we would have full details to enable contact and follow up. The UK HSP registry would also be important for collaboration with other HSP societies such as the European SPATAX organisation.
Ideally we would enrol everyone in the UK with HSP in the registry and in the next few months we will be trying to spread the word as I think the registry will be of great benefit to HSP patients and the development of treatments and markers of HSP progression.

Henry Houlden
Professor of Neurology and Neurogenetics,
The National Hospital for Neurology and Neurosurgery,
Box 12, Queen Square,
London, WC1N 3BG, UK.
Email: This email address is being protected from spambots. You need JavaScript enabled to view it.

Useful Information

Sportability

Using sport and other challenging pursuits, Sportability seeks to put the purpose back into lives shattered by paralysis; to get the adrenalin rush back into a lifestyle coralled by a wheelchair or confined by crutches; to take the ‘dis’ out of disability and focus on ability. And to inspire the thoughts “ If I can do this what else can I achieve ? There is life after paralysis.”
What It Is!
“I’m not a sports fanatic or anything, but proving to myself just what I’m capable of has helped in every facet of daily living. There really is no better way to build a life worth living after paralysis. I can tell you that from the heart.”
That quote sums up Sportability. The Charity has one aim – to provide sport and challenging pursuits for people with paralysis.
It’s not about creating elite sportsmen and women. It is about ordinary people getting off their crutches or out of their wheelchairs and enjoying the fun, the challenge, the rush, the social chit-chat, the whole Sportability Experience and putting the buzz back into life!
Starting modestly in 1989 and we are now active in 13 regions around the UK, and still growing! Young, old, male, female, makes no difference – there’s a sport or activity out there that can fire your imagination, get your pulse racing, lift your spirits high and put a smile on your face.
Activities include archery, sand yachting, quad biking, microliting, gliding, sailing, clay pigeon shooting, kayaking and more! Visit our website for further information.
.Just Do It
If you, or someone you know, would like to participate in our activity programme, then please feel free contact us.
And talking of “free” that’s the cost – our events are available free of charge. We don’t want any barriers to participation.
Why do we do it? If we can help just one person, perhaps someone currently lying in a hospital bed thinking life has to come to an end, to turn back on to a life worth living it’s job done !
Full details can be found online at:
www.sportability.org.uk
email: This email address is being protected from spambots. You need JavaScript enabled to view it.
Sportability is dedicated to re-building those lives shattered by paralysis.

HSP Group Funds

The HSP Support Group is now in a position to support research projects with a maximum grant of £10 000. Applications for such support are welcome. Please apply in writing.
Funds are also available for members to apply for financial assistance with the purchase of mobility aids or equipment that may improve quality of life or simply make life easier. If interested, please request a grant application form from a committee member.

Survey for People with HSP

Readers may remember my article a few editions ago about my HSP blog. For a new phase in the blog I’ve set up a survey to capture information about people with HSP. If this is successful I’ll repeat each year with a different topic. I would be pleased if you’d consider spending a few minutes to answer my questions. I aim to report survey findings on my blog in the New Year.
http://www.surveygizmo.com/s3/1360676/HSP-Symptoms-and-Mis-diagnoses

Adam Lawrence

Fergus Palmer goes “Through the Keyhole”

Turning 70 years of age can be an eventful time in ones life. One can look back and reminisce. In my case, I reflect on my condition. HSP was diagnosed quite late in my life. I often think of that television programme, “Through the Keyhole” the clues are there; as we go through the keyhole. Going through the keyhole of my life, the clues were there!!
Three months before my 17th birthday the family sent me on an outbound course to Aberdovey, Wales, in November 1959 for a holiday. I was very gullible in those days! Holiday it was not!! Character building it was! In the last week, when pushing my canoe, I stumbled, was duly knocked unconscious and was rushed to sick bay by motor launch. I returned home with severe back pain. My sister, Judy claimed the wimp returns!!
United States
In March 1962, I emigrated to the United States of America, and in June 1964, I was duly drafted into the US Army. I could write a book about my experiences. After all the tests of the day, I was diagnosed as suffering from dyslexia, which could explain my poor school results. The drill sergeant claimed, no one could march behind me with my gait. Why did I not join the Royal Navy and not his Army? I was then posted to Fort Knox Kentucky, to become a cavalryman i.e. tanks. This meant I no longer marched. I was then posted to South Korea, spending 13 months patrolling the Korean Demilitarised Zone.
Back to England
During the next 25 years my condition deteriorated, but not to the extent I could not work. I returned to England in 1976. My gait to all concerned was abnormal. My brother, Hamish, said ”You walk like a blob of jelly”. It was not until I met my wife, Anne, at the age of 50 who suggested I should visit my doctor, who in turn thought I could be suffering from cerebral palsy. The neurologist diagnosed FSP, and wondered why this had not been picked up when I attended Outward Bound. The US army he claimed would take any one!!
The next 10 years I basically spent in denial. I drove an average of 800 miles a week as a dry cleaning technician. Most of my customers thought I suffered from arthritis, until one day my collapsible walking stick, which I folded up before entering a shop, sprung out of my wax jacket and frightened the customers, as well as me. The manageress said, “Don’t worry that’s just Fergus, pretending he does not need a walking stick”. I learned something that day, whom am I kidding. Be Yourself!!
There is Help Out There
I can remember our first meeting with Ian Bennett, Region 4, it was in an hotel outside Exeter. Ian suggested I contact Odstock in Salisbury and try the Functional Electrical Stimulation FES. I did, and this week made my tenth annual visit. It is a godsend for me. The FSP Information Booklet is most helpful. I also use the Neuro 4 Trophic Stimulator, issued by the Lindens Clinic. This helps to strengthen the quad muscles and was introduced to me by a neuro-physiotherapist. May not be everyone’s cup of tea. Hydrotherapy is also of great help
Conclusion
I consider myself very lucky. I have made many disabled friends over the past years and they all have one thing in common, it is called a positive attitude. I must admit, some days I say “Thank you God, but why me” Then I remember attitude, have I got it right today?

FP Region 4

Members’ Letters

Dear All, I remember writing early in 2011 how I thought we should try whenever possible to seek rich experience, as the indelible memory of it can well be such a fillip when life’s otherwise proving irksome; further, that, when pushing the boat that bit further out, we are likely to need some assurance that we are not being simply reckless, that peace of mind that is beyond our comprehension and God-given.
Since then, having steadily become more dextrous with my elbow crutches, I have, with that peace that puzzles a good few people, ventured into unusual more challenging environments and safely returned marvelling at so much I’ve seen, more than likely once in my lifetime. Having for a number of years wanted first-hand three-dimensional experience of the Northern Lights, I went earlier this year to arctic Norway, armed with optional spikes over the ferrules of my crutches, only then to totally fail my ambition! They are ellusive, after all, but the impression of them that I bought in Svolvaer from a local photographer and the awe-inspiring spectacles of brilliant sunshine playing on the snowclad environment admirably compensated. I took a tumble or two, but no lasting damage! The last night was spent quite extraordinarily ‘simmering’ at minus 5°C in the Igloo ice hotel, where it is possible, oddly enough, to sleep complete with wall-to-wall ice around you.
Let alone the personal fillip that I certainly got from all this, any notion among those unafflicted that our disability may render us ‘less alive’ is most certainly dispelled by that sort of revelation and the lively conversation that usually follows. While there can be no denying our weakness, the strength left in our upper bodies unaffected by the paraplegia, can, I’ve found, gradually be made to achieve so much more, tethered only, I hope, by our Maker.

JM

Della’s thoughts on Fatigue

Fatigue can be an all consuming condition. It's not visible to others, except, maybe the drawn look and dark shadows under one's eyes. Many, who do not understand, think the afflicted person is being lazy! This is not so and can be very distressing for the individual, being accused of not bothering or being selfish. Two states of fatigue that I have witnessed
1 - Depression Fatigue
I have, in the past, suffered from Depression fatigue. It's an all consuming feeling of wanting to hide away. To not face up to realities of life. Doing the minimum daily chores, then back to bed. Same with work. Go to work. Do the necessary, then home. Because, so exhausted with the worry of getting from A-B, whilst at work or home. Making the effort to put one foot in front of the other. Worrying that people were thinking bad things about you. The feeling that life did not have a place for you. It's more an insular thing than physical. The only way I could get out of it, was concentrating on something positive. I did not know it at the time, because I was concentrating on my illness. HSP etc. People were trying to tell me this, but I could not see it. A case of being at the bottom of the pit and not trying to get myself out. It is the individual that has to, with help, decide to change their outlook on life. Having others to care for you, whilst in this pit, is essential. It is hard for them also, because when we are like this, we tend to push the one's that love us the most, away. Yes. I have been back there a few times since then, because of major upheavals in my life. But, nowhere near the first time. It's more like a typical reaction to events in life. Necessary to go through, to move on in life.
2 - Extreme Fatigue
I have and do suffer from Extreme fatigue. Fatigue is a big part of my life, due to HSP. Do not get me wrong! I am, in no way depressed, mentally. It's the extreme effort of trying to do normal activities in everyday life. It takes so much more energy to do simple things, that used to be effortless. I used to do the housework, go to work, do the gardening and shopping in a day. Now it takes most of one day, to do anyone of those listed. I have had to slow down immensely as HSP has progressed. This, at times, has made me drop into (Depression fatigue) at certain points. Mainly, when so fatigued, that one feels useless. I forget, when feeling this way, that it will soon pass. I am getting used to it now. I know, to rest when I can't physically do life activities, without an extreme effort. It's a case of having to just leave stuff and rest. Be it sleeping, or just resting and doing the minimum. It is totally different from depression fatigue. it's mostly physical, total drainage of energy. Legs feel heavy. Balance is way off. Falling around, stopping my fall on furniture or walls. I now have a tray trolley walker, to stop me falling and hurting myself, also, a walker in the garden. When extreme fatigue hits, I cannot think properly or talk without slurring. It is hard to concentrate, when someone is talking, taking in information, let alone remembering anything more than a few minutes. Clonus is more noticeable when like this. It feels like someone has turned on a tap or zapped all the stuffing out of my body. This, I am sure, could be described as being drunk. The symptoms are similar, or the same. I can assure you, no alcohol is consumed at this time! I call it "Drunk with fatigue".
In my opinion, I feel that Extreme fatigue can produce Depression fatigue. As, when Extreme fatigue hits, we feel useless and life is oh so difficult. If you are not in the right frame of mind, it can fool your mind that things will not get back to "normal". As discussed recently with a fellow HSP’er. I now know my body and mind. That Extreme fatigue will not last. I enjoy myself when I can, knowing full well that Fatigue will hit. So, I do, when I can and don't, when I can't. I'm not saying that I'm happy with it. I hate it, when it is bad. But, I DO know it will not last. It's a case of listening to one's body.
A positive outlook is essential to enjoying life, despite life's lows. Always good to have things to look forward to and friends and family to talk through any worries in life. I am in a happy place. Yes, life could be better, but that is the same with most lives. Could be a lot worse.
I hope I have not DEPRESSED anyone, with my thoughts? Sorry! Be nice to hear others thoughts on this subject. I know, everyone see's things differently.

Della Brookman Region 3

Regional News

Region 6

Ukulele Concert

Held on 8th June 2013 at the Grand Pavillion in Porthcawl, South Wales.
The Porthcawl Ukulele Band were in concert with our very own Peter Bateman in the line up. What a great evening was had by all who attended, enjoying lots of singing and great entertainment. The room was full of lots of HSP Members and friends and guests to support us. It was fantastic! At the interval ice creams and drinks from the bar were available. In the second half there was lots of audience participation. After the concert we sat outside the Pavillion to enjoy a drink and catch up in the lovely summer sunshine. Well done Peter.

AB Region 6