Report on Workshop & AGM, Sunday, 16 August 2009
Northcote Town Hall, Melbourne, Victoria
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More than 40 people attended the HSPRF AGM & Workshop, held at the Northcote Town Hall in Melbourne in mid-August.
President Robin Bligh commented that this was a very encouraging turnout and thanked all who helped make it happen, especially John Crowe, the convener.
At the Annual General Meeting, the following were elected:
President: Robin Bligh
Vice-President: Con Xiros
Secretary: Judy Hoole
Treasurer: Steve Lunn
Committee: Tim Xiros
Workshop
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After a Welcome by Convener John Crowe, there were five Workshop sessions followed by a general group discussion where participants could raise issues and ideas of their choosing.
Workshop Sessions:
HSP Update – Dr. Michael Fahey, Neurogeneticist
Living with HSP – Nicole Battisson, HSPer & Netballer
Transport Issues for the Disabled – Rosslyn Pickhaver, Disabled Motorists Association
Stem Cell Pilot Study – Robin Bligh, HSPRF President
Everyday Hero – Tim Xiros, HSPRF Fundraising Coordinator
HSP Update – Dr. Michael Fahey, Neurogeneticist
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Michael talked about differential diagnosis in the clinic. This is used to determine and differentiate between FALS (motor neurone diseases), dystonias, ataxias, and the HSPs.
Michael showed ‘before and after’ video footage of a boy with HSP taken at the gait lab in Melbourne where a study of the HSP gait is being undertaken by physiotherapist Brooke Adair. The boy underwent surgery known as SEMLS, where the femur and tibia are cut and twisted, and the muscles lengthened. The improvement in gait was visibly dramatic.
Michael gave a PowerPoint presentation with his talk.
Living with HSP – Nicole Battisson, HSPer & Netballer
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Hi, I’m Nikki Battisson. I’m 20 years old and I was born with a genetic disorder called Spastic Diaplegia which basically causes me to walk with my feet turned inwards which affects my balance and can lead to me tripping over myself. I find it difficult to walk long distance and get tired very easily, which has been limiting on what I can do.
Over my life I have undergone several different treatments to try and correct or at least minimize the effect of my disorder. I have had Botox in my hips, calves and thighs and about 4 years ago I had an operation called Rotational Osteotomy to try and straighten my feet, which involved the surgeons putting in plates and screws to keep my legs the right way.
Despite my disorder throughout school I always tried to do activities that everyone else was doing. I participated in P.E. classes and in year 6 played for the school basketball team. It was always hard trying to keep up with everyone but I was always invited to join in. Although wheelchair basketball is my passion now I have tried and participated in several different activities; swimming, scouts and able bodied basketball.
Through primary school the other kids weren’t fully able to understand why I walked different but most were accepting and were able to understand that I was limited in what I was able to do. When I reached high school I was more confident about my disability and myself. I found it a lot easier to settle in and my mates really understood which made it a lot easier on me. Even though I am more confident in who I am I still find it hard when random people stare at me and try to discern why I walk the way I do.
My whole family has always been involved in basketball and I am very lucky that I am able to play wheelchair basketball. I got involved in wheelchair basketball when I was 13. I went to go watch a basketball game and the Australian Gliders coach, Gerry Hewson approached me and asked if I had played wheelchair basketball before. He then gave me all the details for a Tuesday night local competition at Knox Basketball stadium for me to attend. Starting basketball has been one of the best things I have done and it has really stuck with me as a sport. Everyone tells me how much I have improved as a player, and occasionally I believe it. I am currently playing twice a week and attend regional tournaments where we play against different teams from different regions in Victoria. I now play for the Victorian Dandenong Rangers wheelchair basketball team which allows me to further develop as a player.
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Wheelchair basketball is a great way to socialize with different people of different abilities and have many different stories to tell you of their experiences. But its more than that, it is a community where I don’t have to try hard to hide my disability or act like my legs feel okay if they are about to fall off. I find that talking with people who have various disabilities is a great way to realise how lucky I am to only have Spastic Diaplegia and be appreciative of what I can do.
Also I am currently studying Children’s Services at Holmesglen Institute of TAFE, completing my final year of my Diploma. After this year I hope to go and work with a family becoming an aupair in Australia. Through my degree I have been out to childcare centres for placement. These placements have just strengthened my love working with children but I have found it hard with my mobility, the job requiring me sitting on the floor for long periods of time and constantly getting down and back up again.
I hope what I have said, very briefly, can help you understand the smallest sliver of what my disability is and how it affects the most basic levels on a daily basis. Although I know people with much more severe conditions, the thing with disability is it is about how it affects you personally, because no matter the severity, you live with it daily. It can be hard to explain to others, but I am so lucky to have amazing friends and family around that even when they can’t understand they still love me.
In response to a question from the audience, Nicole said that she had done strength work on hips, calves and thighs under physio direction.
Transport Issues for the Disabled – Rosslyn Pickhaver, Disabled Motorists Association
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Rosslyn shared the history of the DMA – outlining its humble beginnings in Victoria up to its current status as the peak body representing, educating and advocating for disabled motorists. Their charter is to support all motorists with disability Australia-wide. The DMA is now a national organization having changed its constitution to cater for the expanded scope.
The issues and areas covered by the DMA are as broad as the issues that affect disabled motorists.
- For example issues to do with construction and infrastructure are not always designed and implemented with due consideration for the needs of disabled motorists. The DMA has been instrumental in getting emergency freeway phones installed at wheelchair height.
- Parking is another infrastructure issue where the DMA is fostering uniformity and consistency across Australia. Parking bay design is constantly being raised by the DMA especially in regard to private parking, for example in shopping centres, where commonly parking bays are wide on just one side, rather than sufficient to allow entry and exit from either side of a vehicle.
- The DMA has also been a driving force in the establishment of the Victorian Vehicle Modification Subsidy Scheme piloted in 2008, where up to $40,000 is available to disabled motorists for vehicle modification, including wheelchair lifts and racks. Having hand controls fitted is also eligible for subsidy to $1,500.
- In another win, the RACV have guaranteed support for disabled motorists in breakdown situations such as flat batteries, changing tyres, etc.
- Public transport is also a focus of the DMA. Melbourne now has some ‘Smart Buses’ which are much more disabled-friendly. Progress is also being made on improving accessibility to the public transport network, including trams and trains.
There is now a National Disability Insurance Scheme that grew out of the 20/20 Vision initiative of the Federal Government. Currently a feasibility study is being conducted with the prospect of the scheme being similar to Medicare in concept. This could change how people with disability access services.
Questions from the floor included the issue of right and desire to drive balanced against capability and safety issues. On a show of hands most HSPers in the room indicated that they currently drive. Rosslyn shared with the group that an individual can ask for a review of where their capability is not currently adequate if they have been refused a licence renewal.
Another question related to taxi subsidy. At least in New South Wales and Victoria, anyone who has been granted a disabled sticker is entitled to get 50% off taxi fares. Rosslyn suggested people ask their local doctor about the half-price taxi scheme. Rosslyn surmised that this scheme may be Australia wide, but was not entirely sure.
Contact details for the DMA:
National Office
2A Station St
Coburg VIC 3058
Phone: (03) 9386 0413
e-mail: [email protected]
website: http://mc2.vicnet.net.au/home/motordma
Stem Cell Pilot Study – Robin Bligh, HSPRF President
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Who is conducting the Pilot Study?
Professor Alan Mackay-Sim
Director, National Centre for Adult Stem Cell Research
Griffith University, Brisbane
Dr Carolyn Sue,
Associate Professor,
Director of Neurogenetics
Kolling Institute,
Dr Gregor Abrahamsen, Researcher,
Dr Stephen Wood, Researcher,
Pilot Study Purpose
How do SPG4 mutations affect cell functions?
Pilot Study Progress
What we are finding – taken from a review done in August, 2009, halfway through the 12 month study period.
*We now have a bank of stem cell cultures from 25 HSPers, 14 of whom have a mutation in SPG4
*Some frozen down for later use; others actively growing and generating data for the researchers
*Human stem cell model of disease is revealing alterations from normal
*Differences in gene expression already found between normal and HSP cells are “dramatic and highly significant”
*This means there are good signposts as to targets, i.e. which impaired cell functions are going to be aimed at for drug screening.
Pilot Study leads to Research
Using human stem cells reveals alterations in HSP vs normal cells.
1. Find differences in cell biology — HSP vs normal
2. Identify target cell functions for drug candidate screening
3. Determine assay for each target
4. Set up the screening
5. Validate with high throughput screening (HTS) of 1,000 compounds
(pilot study finishes here)
6. If unsuccessful, state of the art robotic process for HTS of 2 more libraries of compounds
Research
Disease specific alterations can be identified and lead to therapeutic drug targets. (new technology awaiting publication)
‘Towards A Cure’ Research Program Timeline
Purpose: “Restore cellular functions altered in HSPers”
*Pilot Study conducted by NCASCR 2/’09-2/’10
*Proposed research 2010-11:
- finalize drug screen targets
- develop assays for targets, then
- screening preparation
*Research 2012:
- validate assays
- screen 1,000 CNS candidate drugs – ‘proof of principle’ step before screening
*Research 2013-2016:
- drug discovery andclinical trials 201?
- funding, preferably by NHMRC and or ARC.
Research Participation
*Are you interested in participating in the research program?
*Have you been clinically diagnosed with HSP?
*To join the stem cell bank, call
Wendy Welsh, clinic and research nurse on
02 9926 6491
or email her at [email protected]
Everyday Hero – Tim Xiros, HSPRF Fundraising Coordinator
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Tim spoke about the need for creative and concerted fundraising to be able to fund the stem cell based research described by Robin in his session. Tim gave a PowerPoint presentation that can be viewed here.
Community Discussion
Here is a summary of the output of the Community Discussion session:
- Hydrotherapy definitely helped walking. When hydrotherapy was stopped, HSP symptoms returned to where they were before (note – this is identical to the experience reported previously by an HSPer in NSW)
- Functional Electrical Stimulation (FES) is a technique whereby tiny electrical impulses are provided by a device fitted to the wearer to help the foot dorsiflex (foot comes upwards) during walking and prevent the toes from dragging or catching. At least one member of the HSP community in Victoria has trialled this and it seems to help a bit; however the devices are costly. These devices are available through Allied Appliances in Fitzroy – Lynn or Greg (03) 9419 2499. (Note – FES is much more well-known and widely used in the UK. The devices have been found to be beneficial for about 20% of those who try it)
- Baclofen pump – one user reported that it did not make much difference; the dosage was difficult to get right; however there was one success story. Other snippets of anecdotal evidence about the pump were not positive about its effectiveness.
- Botox injections have been tried by several people present, however nobody had found them to be much help.
- There was a report of the tendon transfer operation to make the feet point straight ahead and go straight versus inwards when walking. It was reported as being worthwhile, but it should be understood that this is a major operation. In this case both legs were done at the same time. (Note – there have been other reports by people who have had this or similar operations and all report that it has been worthwhile. Suitability for such an operation needs to be assessed by a relevant specialist).
- Regular exercise is generally recognized as being extremely important in managing and maintaining mobility for HSP. It was mentioned that a neurological physiotherapist is available at both Cheltenham and Caulfield hospitals in the Melbourne area. Someone else reported significant success as a result of working with an exercise physiologist.
- Swimming, together with hydrotherapy was reported as being highly beneficial to fitness, flexibility and spasticity management. Facilities mentioned included Melbourne Sports & Aquatic Centre (MSAC) Albert Park; Maribyrnong Aquatic Centre; and Monash Aquatic and Recreation Centre.
- Feldenkrais therapy was reported as having a positive outcome, with the person who does it twice a week feeling a lot freer after a session and with enduring effect. Feldenkrais can be undertaken as a regular home exercise routine once the techniques have been learned.
- Horseriding as an activity was reported as having a significant beneficial effect for someone with HSP. Cycling also got the thumbs up.
- The benefits of using a bedstick to pull yourself up out of bed was discussed. These can be bought at pharmacies.
- Another device known as a Handibar, designed to fit in a car door latch, helps get out of a car from the sitting position. This device was also commented on favourably. It can also be bought from pharmacies (costs $49).
- Tips for preventing falls were offered:
- watch the ground
- think about lifting the feet
- fall safely – learn to roll so that you don’t fall flat
- watch the ground
- Finally, there was general agreement that meeting others with the same condition is both useful and positive.
- Feldenkrais therapy was reported as having a positive outcome, with the person who does it twice a week feeling a lot freer after a session and with enduring effect. Feldenkrais can be undertaken as a regular home exercise routine once the techniques have been learned.