
	Events & Activities

	Events & Activities MDANSW conducts or is associated with a range of activities to assist in lifting the profile and / or raising much-needed funds. National Electric Wheelchair Sports (NEWS) The National Electric Wheelchair Sports (NEWS) is specifically for people who use electric wheelchairs for their everyday mobility and this is the only competition available for people with neuromuscular disorders to compete on an "equal playing field" throughout Australia and New Zealand. NEWS was first held in Melbourne in 1986 and has been held annually since. NEWS is conducted under the auspices of the Muscular Dystrophy Associations of Australia Inc. NEWS is the showcase for electric wheelchair sports in Australia. It is a unique annual event which displays the all round skills of each athlete in the three sports of electric wheelchair hockey, balloon soccer and rugby league. 21st NEWS Tournament April 2006 The NSW Colts dominated the 21st National Electric Wheelchair Sports, held in Perth, April 17-23, 2006. The team: Chris Suffield (Captain), Peter Dalrymple, Stephen Webb, Hamish Armitage (on debut), John Shepherd, Dean Crane and myself as Coach. This was to be my first NEWS where I would not actually play, as last year I was a player and coach. I quite enjoy being part of the game from the sideline. Tuesday: the games commenced with the Opening Ceremony and the round robin competition for Soccer. The Colts dominated the day, winning all of their matches. We qualified for the final against the Western Wasps. Wednesday: the round robin in Rugby League. Again the Colts were undefeated. The hardest game was the traditional State of Origin clash with the Queensland Gladiators. Once again we would meet the Wasps in a final. Hockey was held on the Thursday with Victorian Vipers as the favourites. After defeating the other teams, the Colts gave their southern rivals a wake-up call by winning 7-2. The Vipers qualified for the final against the Colts. After the round robin competition, the Colts secured the 3rd consecutive Roger Melnyk Trophy as the overall best team. What was also amazing was that we were the first team to complete the round robin by winning every game. Thursday night: the All Australian Team was announced. NSW Captain, Chris Suffield was selected along with Stephen Webb. I had the privilege of being selected as the All Australian Coach. The All Australian team were to take on the All Stars, comprised of the next best six players in each sport, in exhibition matches the following day. Friday night: All Australian vs. All Stars. Australia easily won Hockey and Soccer, but only just won the Rugby League, due to some great All Star performances. Saturday: the Finals. First up was Soccer against the Western Wasps; a tight and tense game. We thought we had the lead until what we felt was a fair goal was disallowed. The same linesman gave Western Australia a free hit a minute later which led to a goal. The last three minutes we threw everything at them but just could not get that equaliser. The Western Wasps won 1-0. We regrouped and took revenge in the Rugby League final. After trailing 6-0, we won 16-6 in a high quality game with very few errors by either side. Our defence was too good, as we claimed our first Rugby League title in 9 years. Then, the amazing Hockey final – the Colts showed great maturity, systematically playing Victoria out of the game. The Colts won 5-3 and won Hockey for the first time and this was only the second time that Victoria have been beaten in a Hockey final – they have played in all 21 finals! After a lovely Presentation Night dinner, the Best & Fairest winners were announced. For NSW, this honour went to Stephen Webb, also awarded the Overall Best and Fairest. The successful Colts hope to do it all again this year in Sydney. Anybody interested in getting involved with NEWS is welcome to any of the training sessions and can get more information by contacting Michael Baptiste on 02 9643 1429, or by contacting us. The King and Us - A Beautiful Partnership! December 2007 In planning our final 50th anniversary celebratory fund-raising event of the year, we came across a coincidence that we thought was worth promoting. We shared our "birth" with the launch of the original hip-swiveller himself, Elvis Presley! Did we need any other excuse to put together what turned out to be a very successful and fun night for all concerned? No way! We called the event "The King and Us" (with no offence intended to Yul Brynner or the English language!) and we held it at HMAS Penguin, Mosman, courtesy of the Senior Sailors’ Mess Committee headed by its President Warrant Officer Ken McPherson on Saturday 1 December 2007. Over 160 fun-seekers joined us for the night, many of whom would have made Elvis overjoyed and proud of their attempts to fashion themselves in his likeness. There were big wigs, jump suits, hair dye, gold sunglasses, chains, bangles, glitter, etc. There was even a cigar! Go figure! And the guys were even more spectacular! We raised over $55,000 net on the night and are very grateful to HMAS Penguin, chef WO Colin McKenzie, the Mess Manager Jane Slieker and her staff and John Kinsella of Bilbergia for having underwritten our costs. We also received magnificent support from over 50 very generous donors. Our sincere thanks to everyone who helped put the night together and those who attended, as well as our new President Rob Ferguson for all his hard work in making it happen. The accompanying photos of a few of the attendees will confirm that the meat that was included in the excellent meal for the night was not the only "ham" present! Our Favourite Trekker Readers may recall that last year one of our wonderful supporters, Mandy Smith, raised over $5000 in sponsorships competing in the Sydney Marathon. Mandy has a very good friend whose eight year old son has been recently diagnosed with muscular dystrophy. I spoke to Mandy earlier this year and told her of the fantastic experience I had had walking the Kokoda Track with two of my daughters in 2005. I also showed her my photos of the eight days we spent in New Guinea. To put it mildly, Mandy's interest was aroused. Great, another challenge and an opportunity to raise some more money! So, armed with the tips and information she had gathered and with a lot of kilometres of training behind her, Mandy set off for the Kokoda Track in July. Being the super athlete that she is, she took the whole 96 kilometres of it in her stride(s). In fact when we were comparing notes after she returned I was sort of hoping that she would declare it the toughest and most gruelling physical experience of her life. This would make me at least feel that my experience had been nothing exceptional. I was not therefore quite prepared for her answer when I asked her what the toughest part of the trek was for her. Her reply? Her tent, followed by the awful food! Enough said, so we moved on to check out her photos! Mandy managed to raise nearly $10,900 on our behalf from her Kokoda efforts and hopes to repeat the experience in a few years time with her husband. Maybe I should show her some photos of Mt Everest to see if there is any spark of interest there! I was delighted to present Mandy with a framed certificate of appreciation for her outstanding efforts on our behalf. A fantastic supporter and a real champion. Well done, Mandy. Ian Dear		Community Champions We continue to be inspired by the fundraising efforts and enthusiasm of our members and supporters in helping to raise awareness of neuromuscular disorders. Below are some of our Community Champions Streetrides “cruise” from Weston Creek to Nowra Angela Kettlewell and her family are well known for raising the profile of Muscular Dystrophy in the Shoalhaven district, and on October 15 a lot of hard work came to fruition. Angela was revved right up for three months organising the event which involved Streetrides, a major car club based in Canberra and others, driving from Weston Creek ACT to Nowra. Despite the cool misty weather, over 100 cars registered for the event which included: A drive via Braidwood down Clyde Mountain to Bateman’s Bay, followed by a coastal cruise up to Gray’s Beach beside the Shoalhaven River A “show ‘n’ shine” of some beautifully modified vehicles A tasty barbecue provided by Nowra Lions Club Presentation of awards provided by local auto parts businesses and the organisers Presentation of a thank you plaque from MDANSW (Rob Ferguson) to Angela for all her hard work Angela was assisted by her grandmother June Ivers, and Robyn and Arthur Kirby helped on the day. Many drivers and passengers commented that they thoroughly enjoyed the event, and supporting our cause. Angela was pleased with the event, which raised over $1,500 for MDANSW, and spoke about doing it again next year. The day concluded around 4pm when roaring exhausts signalled the return of drivers to the ACT via Kangaroo Pass and the Hume Highway. NEUROMUSCULAR DISORDERS IN THE 21ST CENTURY - THE HOPE AND THE HYPE - Here is a brief summary of Professor North's talk which covered three broad topics: Gene discovery and genetic diagnosis Understanding disease mechanism Better symptomatic and specific therapies 1. Gene discovery and genetic diagnosis The identification of the underlying genetic cause of a particular neuromuscular disorder allows researchers to begin to understand the mechanism of disease, ie how a mistake in a particular gene causes the disease. This then enables us to develop therapies that target the specific disease process, and therapies that aim to replace the faulty gene or correct the mistake. Knowing the specific genetic change in a particular individual allows us to provide that person and their family members with accurate counseling about genetic risk, and the option of prevention or prenatal diagnosis. The genetic basis of an enormous number of neuromuscular disorders has been identified in the last ten years. For example, in 1996 there were four known genes for limb-girdle muscular dystrophy (LGMD) - alpha-, beta- and gamma-sarcoglycan in addition to dystrophin (for Duchenne and Becker MD). Now, ten years later, there are at least 23!! Despite this there are still a significant number of LGMD in which the genetic basis remains a mystery. 2. Understanding disease mechanism Researchers are now studying all the genes known to be associated with muscle disorders to understand the complex process involved in the disease and the factors that lead to muscle wasting and muscle weakness. For example, dystrophin is known to be an important structural protein involved in protecting the muscle membrane from the forces generated when muscle cells contract. However, it is becoming clear that dystrophin’s role is much more than just structural, and it is involved in the complex signaling pathways in the muscle cell itself. 3. Management of muscular dystrophy: Better symptomatic and specific therapies Medical Therapies: Although the focus in most people's minds at the moment is very much on the possibility of future curative therapies, it is important not to forget that excellent medical care continues to make a major impact on the quality of life and survival of children and adults with neuromuscular conditions. For example, the introduction of spinal surgery for scoliosis and respiratory support has significantly improved survival of boys with Duchenne MD (DMD) Drug therapy: Corticosteroids have been used for some years now in DMD and in some boys result in rapidly improved strength. This is usually measurable in 10 days, and the maximum benefit is reached by about three months. Steroids slow the loss of function for up to three years, and can prolong the ability to walk for a number of years. The exact mechanism of action of steroids is not well understood, and we know that they have an effect on many different processes within the muscle eg. They promote muscle regeneration, stabilize the muscle fibre membrane, have an anti-inflammatory effect and decrease muscle degeneration. Gene therapy: Most of these therapies aim to convert the disease to a milder form eg. from Duchenne MD to a milder disease like Becker MD by producing at least some dystrophin that is partially functional. The biggest challenge for these therapies is to deliver enough of the therapy to the muscle cells themselves. Many of these therapies are successful in mice - but a whole mouse only weighs 25g and a single mouse muscle weights a lot less than 1g! Cell replacement: Myoblast transfer therapy aims to introduce immature muscle cells with a normal or functioning gene into a patient's muscle - these normal cells can then differentiate into normal muscle tissue. Gene replacement: Directly replacing the faulty gene using a carrier called a 'vector'. The aim is that the new gene can be put into an altered virus and given to the patient where it will multiply and spread into the muscle. Gene repair - which aims to fix the patient's own faulty gene: Drugs that cause 'read through' of early stop signals (called 'nonsense mutations'): In some patients, the fault in the gene results in an early stop signal, so that the gene is not read all the way to the end, and the protein is not formed normally. PTC124 is an example of one of these drugs, and is in an early trial currently starting in the UK. Compounds that provide a 'gene band-aid' - and allow the muscle cell to 'skip' over the part of the gene where the mistake is (called 'exon skipping'). At least some functional protein will be formed. Gene up-regulation or inhibition: We know that although there is degeneration of muscle cells in muscular dystrophy, there is also a lot of regeneration. This regeneration is not enough to keep up with the muscle damage. Therefore, rather than just trying to replace the missing or faulty protein, we can try to boost other pathways of muscle regeneration or dampen the degenerative pathways. For example blockade of the protein myostatin results in loss of the normal inhibition of muscle growth in mature muscle and results in muscle hypertrophy. Also there are other proteins that may be capable of taking over at least some of the function of the missing protein and we may be able to increase the production or activity (up-regulate) of these eg. Utrophin is an immature version of dystrophin that can replace some of its functions in skeletal muscle. There is a huge amount of exciting research happening, and although progress seems slow, it is important that above all we do no harm. Side effects of experimental therapies can be severe, and therefore they need to be tested thoroughly before they become widely available. A catastrophic side effect will be a dramatic set back to the process, a set back that we cannot afford. Beginnings "Pathways to a Cure" had the phones running hot within my first few weeks at MDA as we had 150 acceptances. This proved again a great opportunity to meet and speak with some members of MDA. Many had travelled from afar (Dubbo) and spent many hours travelling to the venue in quite a heat! The only 'hiccup' was the unforseen need for 'coins', necessary for the use of the outside car park. That will definitely be an added reminder for other events we might have at the Novotel Sydney Olympic Park. The Conference opened with a retrospective on Muscular Dystrophy over the past 40 years followed with the outcomes of the recent research over only the past decade. Topics discussed by the speakers focussed on trials and therapies for MD, studies of stem cell transplantation with possible restoration/cell growth and the ethics involved in such research. The focus was on the 'lack of the protein Dystrophin' in DMD, with great detailed diagrams (most informative) and discussion on the various therapies that will hopefully enhance the muscle regrowth for future clinical application. Stem cell research with therapeutic cloning was explained with the hope of future application resulting in cell regrowth. Speaking with some Members and visitors during the breaks, the majority agreed "the conference was a unique opportunity to gain knowledge and more insight of the research into MD over the past decade". Here I would like to take the opportunity to thank Professor Graeme Morgan for his introduction, and Professor Kathryn North, Professor Anne Cunningham and Dr Bernadette Tobin for their expert knowledge on research in MD and ethical issues. My further thanks to Ms Louise Suffield (MDA member), Ms Sophie Scott (ABC medical reporter) and the exceptional moderator Robyn Williams (ABC Science presenter) who joined our experts to form the creative, Hypothetical Panel! They addressed a fascinating scenario of a wealthy philanthropist wishing to donate a large sum of money to a researcher who was claiming success in the treatment of muscular dystrophy in mice. The philanthropist's five year old son had recently been diagnosed with Duchenne MD.