ISSN: 1366-0209 Summer 2017 No. 53 A-T Society News

This sparkling issue celebrates • The start of the Erydex trial • The International A-T registry • Achievements of people with A-T • Inspirational fundraisers 2 A-T Society News

Ataxia-telangiectasia is a rare, genetic, challenges than many, people with A-T have lives neurodegenerative disease. It starts in early to live, and the Society’s aim is to ensure they have childhood and affects many parts of the body causing the support they need to live them to the full. We do severe disability. this through funding research, providing information, practical support and financial assistance, working to The A-T Society was established in 1989 and is improve clinical management and raising awareness. committed to helping, supporting and advising families affected by A-T. While they may face more

Contents

3 Diary 13-22 People • William Davis, • Erin’s new wheelchair chief executive • Pride of Gwent award • My journey to employment 4-8 Research • Erydex trial underway 23-43 Fundraising • A-T Registry • Corporate fundraising Page 6 Page 22 • ATTeST trial • Chelsea Film • Diabetes and A-T Study • Global Make Some Noise • Inspirational Fundraisers 9-12 Support Services • BBC One Show Children in • Adult Activity Weekend Need Rickshaw Challenge • PIP assessments • Breast screening in women

Page 9 Page 34

Editor’s comments

Many thanks to all contributors. The copy date Phone: 01582 760733 for the next issue is 1st October 2017. Please Fax: 01582 760162 send comments, ideas, articles and pictures to: [email protected] www.atsociety.org.uk Beatrice Prokofiev Champagne 07841 485330 Registered Charity cycle ride [email protected] No.1105528

If you would like to receive this newsletter The opinions expressed in by email as a PDF file, please let us know. A-T Society News are those of the individual authors and A-T Society, Rothamsted not necessarily those of the Harpenden, Herts AL5 2JQ A-T Society.

A-T Society News Diary 3 Just William William Davis, chief executive of the A-T Society, reflects back on a busy few months Having returned a couple of weeks But alongside all these and many ago from cycling from St Albans other uplifting events, there to Champagne to raise money for have also been moments of real the Society I feel that the last six sadness, in particular the loss of a to nine months have something number of people who have been in common from riding across very influential for the Society. Normandy. You go riding across Many people will remember the sunny uplands for a while and Rupert Prokofiev, who died at the then it’s down into a deep valley beginning of the year. Apart from with a long climb out and then being a trustee of the Society, once again you’re rolling across Rupert had spoken at a number of beautiful flat countryside. Family Weekends, talking about We’ve had some great highs. For independent living and most a start, there was all the coverage recently leading a workshop on the we got on national Radio in the language used to talk about A-T. run-up to Global’s Make Some His engaging personality, positive “There was that Noise Day. A number of our young attitude to life and willingness to people and their families did a speak out were a huge inspiration incredible moment great job of talking about life with to me and to many other people. when the £26k cheque A-T on the various stations. We also lost Siân Watkins, another from Global magically There was Ebony Robinson, who person who had lived many years morphed into one for took part in BBC The One Show’s with A-T. Siân’s family were very a staggering £90k.” Rickshaw Challenge – the first closely involved in setting up the ever wheel-chair user to do so. A-T Society and her father Geoff Her enthusiasm and her openness was chairman for a number of people like Siân and Rupert to about the challenges of life with years. It is as a result of their living fulfilled and independent A-T moved and endeared her to efforts that the A-T clinic was first lives with A-T. people across the nation. Read established in Nottingham, where Our strategy will look to address more about this on page 34 and 35. the family lives. We publish an the psychological and emotional article by Siân on page 20. There was the touching film about needs of people with A-T and their the twins Brae and Broghan Sewell Another sad loss was that of families at the different stages of and their parents. While the film Mireille Gervasoni, Chair and their lives and in particular to focus highlighted the difficulties A-T driving force behind the French on adults with A-T and help them brings, it was lit up by the obvious A-T group APRAT. Mireille set up build the confidence and skills love between the brothers and APRAT when her son David was to take control of their lives and within the whole family. And this in diagnosed with A-T and it became manage them as they wish. turn brought nearly a whole page the most active and effective This is an ambitious programme, of coverage in the Sun as well as A-T organisations in continental but one that I am convinced is television features. Europe. Mireille admired and becoming more and more urgently worked very closely with the A-T And of course there was that needed given the advances in Society, translating our clinical incredible moment when the £26k care for people with A-T and guidance and other documents cheque from Global magically the increasing number of adults into French and jointly funding morphed into one for a staggering living with the condition. We will projects. £90k. There was the buzz of of course be working closely with cheering on our runners in the So it’s been very up and down, people with A-T and their families Brighton Marathon. And there was but then that reflects life with as we develop the strategy and in the fantastic experience of riding A-T. Looking ahead, though, the doing so we will be taking forward 350 miles with an amazing group Society is currently putting together the legacy of Sian, Rupert, Mireille of cyclists to raise funds for a strategy which is inspired by and many others – and that to me the Society. and rooted in the commitment of is really important. 4 Research A-T Society News

Erydex trial now underway The first major clinical trial of a treatment for the symptoms of A-T gets underway – but not yet in Nottingham After all the talking, planning to say exactly when recruitment their frustration and have been in and preparations, the trial of will start. regular touch with the Nottingham the Erydex System is finally team to offer any assistance we The issue is that Nottingham will underway. Now known as the can to help speed things up. We be one of the largest centres in ATTeST (A-T Treatment with the are also in constant contact with terms of numbers of participants. Erydex SysTem), this study is the the sponsors of the trial who are This, and the complexity of the first major international trial for fully engaged and committed to trial, involving as it does monthly a treatment for the neurological providing all the support needed visits to the centre and a range symptoms of A-T. As we go to to solve the issues encountered of tests and assessments to be press some 20 people with A-T in Nottingham. carried out, mean that they need to out of the 180 required have been recruit a research fellow. It is this However, while we don’t know recruited in 8 centres in Europe recruitment and the bureaucratic the exact start date, we are and the USA. processes of the NHS and nevertheless confident that the The A-T centre at Nottingham will University which are taking time. trial will go ahead and also that be participating in the trial and there is an excellent team in place Many families have been in aims to be recruiting participants in Nottingham to carry it out. contact with the A-T Society to later this year. The centre is As soon as we have any more find out what is happening, having receiving its final site-visit from the information about a start date, heard that the trial is underway in trial organisers in July. However, we will let people know. other countries. We understand at the moment it is not possible

A-T Registry The registry will contain clinical data uploaded by doctors and as such it will complement the data being collected by the Global A-T Family Data Platform which is provided by individuals with A-T and their families. In addition to data on people with A-T, it will also collect data on people with related conditions, such as A-T-like disorder, AOA1 and AOA2 and Nijmegen breakage syndrome, which are even rarer The A-T Registry has now been launched and is than A-T. starting to collect clinical data on people with A-T. As it was set up with a grant from the European Because A-T is so rare and patients so spread out, Commission as part of the Horizon 2020 grant which and also because symptoms vary from patient to is helping fund the ATTeST trial, data collection is patient, it is difficult to be able to answer with the therefore beginning with participants in the trial. certainty required by science, questions such as: how The Registry is managed by the A-T Society, with many people experience certain symptoms, at what much of the work being done by Registry Coordinator, age certain features appear or whether particular Jo Reader. symptoms are linked to others. By bringing together data from hundreds of patients, a registry will help We are in touch with all the centres that are answer these questions and in doing so help us participating in the study and are in the process of understand A-T better and thus help treat it better. translating the necessary information and consent forms into the different languages required. We are For more information, you can visit the Registry also working on ways to simplify the uploading of website www.atregistry.eu or email existing data. [email protected]. A-T Society News Research 5

About the ATTeST trial In a number of small-scale studies in Italy, steroids have been shown to improve the neurological symptoms of A-T. This means that children who have taken steroids have shown improvements in things like walking, balance, movements and speech. However, conventionally delivered However, for the treatment to be and one will get the placebo, steroids have significant side- approved as safe and effective, i.e. no active drug. effects, which rules out prolonged the licensing authorities require a use in people with A-T. The Erydex However, the trial is double- larger trial, involving 180 patients. system is a novel way of delivering blinded, which means that neither This is now being carried out in 24 the steroid dexamethasone, which the participants nor the doctors centres around the world, including seems to avoid these side-effects. running the trial will know which Nottingham. A small amount of the individual’s group they are in. blood is removed, the red cells To participate in the trial, you After six months, and again at are separated out and the drug must be at least six years old nine months, the number of is infused into these red cells. with a diagnosis of A-T, weigh participants in the placebo group They are then injected back into over 15 kilos and be able to walk will be reduced. After 12 months, the individual, where the drug independently or with occasional the formal trial will be over and is released slowly over the next use of a support. There are also a assuming that there have been few weeks. number of other criteria regarding no adverse events, anyone who health and immunological status. A previous smaller-scale trial wishes to will be able to continue showed that the treatment It must also be remembered that on the treatment. At this point was effective in improving the this study is placebo-controlled. everyone, including those who neurological symptoms in children This means that while all have remained in the placebo with A-T and had no major side- participants will undertake the group will get the active drug. effects. In a few children who have treatment, not all will be receiving More information is available been on the treatment for several the actual drug. Patients will be on the trial website attest-trial. years, it also appears that the divided at random into one of three com. If you would like to talk to us positive effect continues, and that equal-sized groups. One group about it, or express an interest in the treatment is well tolerated. will get the drug at a higher participating, please contact Anne dosage, one at a lower dosage or Kay on 01582 760733.

Diabetes and A-T: the Dundee RAMP study Laura McCreight gives an update response to certain medical A-T and Insulin Resistance on the RAMP study (Response treatments. They were particularly Case reports from the 70s had of Individuals with Ataxia interested in metformin – a previously linked A-T to an Telangiectasia to Metformin medication with glucose-lowering increased risk of diabetes and and Pioglitazone). effects, widely prescribed for type resistance to the action of insulin, 2 diabetes. A-T and Metformin so Prof Pearson teamed up with doctors at the A-T specialist In 2011, Prof Ewan Pearson Whilst investigating the body’s centre at Papworth, to investigate and his team of researchers at response to metformin, they found this further. the University of Dundee, were a surprising genetic link with a investigating how an individual’s region of DNA that includes the They studied ten adults with genetic code might affect their ATM gene, the gene which does A-T and ten adults without A-T not function in people with A-T. continued overleaf 6 Research A-T Society News of similar weight and age using oral glucose tolerance tests. The results confirmed, for the first time, that adults with A-T do have an impaired ability to handle sugar and are resistant to the effects of insulin. Mice lacking ATM Since then, a scientific paper has described the link between ATM deficiency and insulin resistance in mice. It demonstrated that mice lacking ATM tended to be insulin- resistant, have higher levels of fat One of our volunteers wearing the indirect calorimetry ‘hood”, which around their abdominal organs, measures the oxygen and carbon dioxide in the exhaled breath of the wearer. and increased liver fat content. However, they had less peripheral The main part of the study is Recruitment update fat. The paper also showed that a “dual tracer mixed meal” test, Recruitment for the RAMP study treatment with metformin can which involves the participant has now finished. In total, 9 improve the insulin sensitivity lying on a hospital bed for eight individuals with A-T, and 18 control of these mice, while another hours, with a glucose infusion participants were recruited. The diabetes drug, pioglitazone, can (drip) running in one arm. After final study visit will be at the end improve the fat distribution and two hours of this infusion, a liquid of August, and results will be insulin sensitivity. meal is given. Both the drip and published at the beginning of 2018. This mouse study identified the liquid meal have glucose tracer The team are hoping that they will potential mechanisms by which added – meaning these glucose gain new insights into the causes ATM deficiency causes “fatty liver”, sources are “labelled” and can be of diabetes and fatty liver in people something which is now being distinguished from the glucose with A-T and that the results will found in many people with A-T, already in the blood stream. During help improve the treatment of and insulin resistance. The the eight-hour study, multiple blood diabetes in A-T. tests are taken from the other arm, team felt this warranted further With thanks investigation in humans. and gas exchange is measured. This tracer study is repeated at The research team in Dundee The RAMP study each visit. have worked closely with the A-T Society throughout the study, The RAMP study investigates From the tracer study, and would like to pass on sincere how individuals with A-T respond measurement of blood glucose gratitude to all the staff. The help to metformin and pioglitazone, levels and hormones such as they provided with recruitment compared to “healthy” controls. insulin and glucagon, allow the and support of the study has Participants visit Dundee on three team to calculate the individual’s been indispensable and greatly occasions – once at baseline, insulin sensitivity and how this appreciated. the second visit after eight weeks changes in response to each of the of metformin treatment, and the diabetes medications. The research team would like final visit after eight weeks of to give special thanks to all the pioglitazone treatment. fantastic people who took part, At the initial visit, an abdominal along with their families and MRI is carried out to measure fat carers. Without your help, none distribution in the abdomen and of the research would be possible! a fat biopsy is taken. These It has been a pleasure to get images and tissue samples will to know all of you, and we look allow investigation into the link forward to catching up with all of between A-T and fat metabolism you at the A-T Family weekend. and turnover. Blood bottles ready for the eight hour (Photos used with permission tracer study – 55 blood tubes in total! of the volunteer). A-T Society News Research 7

A-T clinical research conference October 2014 saw many of world’s an international registry in order of inflammation and what the leading specialists in treating A-T to produce an accurate natural current evidence tells us about gathering in Warsaw for the 3rd history. this. While there were no concrete A-T Clinical Research Conference. conclusions, this is clearly an area It was also good to get the first Also present were a number of top that needs more work and data presentation of data from the research scientists with an interest from the A-T Registry will play an CATNAP imaging project in in understanding and treating A-T, important role in this. Nottingham. While there is still as well as representatives of the a lot of work to do to analyse A-T Society, A-T Children’s Project, Another major topic was bone- all the data from the scans, one BrAshA-T and patient groups from marrow transplant. Four operations interesting finding is that the Poland, France and Spain. have now been carried out in volume of a particular part of the people with A-T in Poland and Over three days sessions looked brain (the 4th ventricle) becomes Germany. The transplants were all key areas of A-T: immunology, enlarged as the cerebellum shrinks carried out for different reasons but neurology, respiratory care, and this appears to grow in parallel mainly to improve immune function cancer and metabolic disorders. with neurological deterioration, and reduce the risk of cancer. For each of these areas there were which means that there is a While all those who had the presentations both on the basic possibility that this could be used transplant seem to be doing well, it science and on clinical aspects. as an outcome measure, for is not clear what long term benefits Other sessions focused on issues example in clinical trials. there are and there seems to be such as next-generation research, little evidence of any major impact There was lots of discussion emerging treatments and on the neurological symptoms. around the biggest unanswered unusual cases. Significant questions remain about question in A-T: why does the loss the circumstances in which one As always with these clinical of ATM protein lead to the loss of would choose to transplant. research conferences, neurons in the cerebellum? There presentations are kept short to are many mechanisms that might The respiratory session ensure maximum time for group be responsible: the effects of emphasised the importance of the discussions. This is something unrepaired DNA damage, failure recommendations of the 2015 ERS which participants always value to deal with oxidative stress, statement on the management of very highly and discussions abnormal processes of autophagy lung disease in A-T for aggressive, often went on well into the and mitophagy, problems with proactive monitoring and treatment coffee-breaks. the network of cells that support of lung disease. There were two While there were sadly no neurons and so on. Different presentations on MRI studies of reports of major breakthroughs in researchers hold different views, the lungs. One from Rome showed understanding and treating A-T, but there was general agreement some extremely interesting images the main aim of conferences is that it would be beneficial to be and the other in Nottingham is due to share knowledge, encourage able to develop ATM-deficient to start shortly. There was also a debate and spark new ideas and neurons which could be studied report of an interesting study into there was a lot of this. in the laboratory, and researchers oxidative stress in lung disease are making good progress towards which is currently underway in The neurology session looked at doing this. Australia – though there was no data from Nottingham and Johns data as yet. Hopkins detailing neurological Immunology is a complex area deterioration over time in their and in A-T the pattern of which The cancer session contained patient groups. The Frankfurt team antibodies are low high or normal a lot of detailed discussion of had found a correlation between varies considerably between treatment of cancers in A-T which neurological deterioration and patients. Discussion focused on is difficult to summarise here. the presence of specific brain the significance of these variations Some key points though were that proteins. These findings underlined and what they can tell us about while some people respond well the importance of a standardized A-T and about the course of the to reduced-intensity treatment, scale for measuring neurological condition for individual patients. approach. This requires a detailed impairment, as well as the need for Delegates also debated the role pre-treatment assessment, which continued overleaf 8 Research A-T Society News should include a brain MRI and This would then aid the the next conference, to be held in consultation with A-T experts and development of more targeted Naples in November 2018, even a major supportive care package. therapies. better. The planning group led by For the future, we should be Cynthia Rothblun-Oviatt of the There were many other areas working to get more information A-T Children’s Project and William of discussion and presentation, on cancer in A-T with information Davis is working to incorporate and feedback from participants in registries and if possible the these into the programme for was overwhelmingly positive. collection of tissue samples. that event, which we are already Nevertheless, there were a number putting together. of ideas about how we can make

ATW 2017 in Milan William Davis reports on the latest A-T research conference April 2017 saw another conference They are currently available on on A-T research, this time the our website. A-T Workshop, which took place in Milan. This conference, which A personal highlight for me was takes place every two years is being asked to chair a bilingual much more focused on laboratory Q and A session for 20 or so research than the Clinical Italian families with doctors and Research Conference. Given that researchers, similar to the one this was only six months after the we have at our Family Weekend. Warsaw conference, there were Although it was complicated few clinicians present and the having to translate between Italian A-T Society was the only patient Italian families attending the and English, the families greatly organisation from outside Italy. Milan Conference appreciated this opportunity, which they don’t usually get, and there The conference was opened by was very positive feedback from Yossi Shiloh, whose team first The difficulty at the moment some of the professionals, too. identified the ATM protein back in though is telling who, if anyone is I was also able to have supper with 1995. He started by expressing his right. What was more encouraging and chat to the families at frustration that 22 years after the though was that compared to the a nearby restaurant, which was ATM gene was first discovered, first conferences I went to six years very enjoyable. we still don’t understand how loss ago, researchers are looking much of the gene and its ATM protein more at the bigger picture, and I also helped William Whitehouse leads to neurodegeneration – trying to see how these various (the Nottingham Neurologist) which makes it difficult to develop functions work together to cause run two workshops as part of targeted treatments. He underlined the symptoms of A-T. This certainly a project to identify some ‘Core the need for a holistic view of feels intuitively more sensible and Outcome Sets’ for A-T trials. This effects of ATM deficiency to is more in line with how people means a series of measures (for understand the neurodegeneration with A-T experience the condition. example how far you can walk in ATM. He sees DNA repair as the From the A-T Society’s perspective unaided or fatigue) which would be critical area of ATM activity but this it was good to be able to speak measured in every clinical trial for is a very complex area in which to researchers and to catch up A-T and would enable the results ATM interacts with hundreds of on where their research is. I was of different trials to be more easily other proteins. able to discuss an exciting and compared. The measures need to Most of the other presentations significant research project which be agreed by all parties involved, focused on cell processes that is currently being considered by people with the condition, doctors, were in some way related to ATM, the Society’s Scientific Advisory researchers and so on, so not and some speakers suggested Board. I was also able to film a simple process. However, it is that particular functions might be interviews with a number of something which if it can be done, responsible for neurodegeneration. researchers and put these on line. is potentially very useful. A-T Society News Support Services 9

Adult activity weekend 2017 Friday 8th September – Monday 11th September

Our adult activity weekends provide a great opportunity for adults with A-T to get away for a few days, take part a range of exciting activities, and relax in the evening with other people with A-T. We’re excited to announce that this year our adult activity weekend will take place in the Lake District at the Bendrigg Trust, an outdoor learning and activity centre especially designed for people with disabilities. The Bendrigg Trust provides the perfect place for people to try something new and face up to new challenges. From canoeing, indoor or outdoor climbing and abseiling, zip wire, sailing, orienteering to arts and first week of the booking letter being For a sneak preview of the crafts, film nights, disco and sent out last month. We’re currently facilities and the activities campfires – there’s so much to do. working with the team at the available please visit their website This three night experience has Bendrigg Trust to plan the agenda www.bendrigg.org.uk. proved very popular and all for the three days and will confirm 15 places were taken within the more details nearer the time.

Family weekend Saturday 14th October – Sunday 15th October 2017

visiting Paradise Wildlife Park on Jackson and Dr Anke Hensiek on the Saturday and will be visited by mild variant A-T. On Sunday we’ll ‘Dennis the Fire Engine’ as part be focusing on Psychological and of an Emergency Services and Mental Health issues and also our First Aid workshop on the Sunday. adults with A-T will be talking about With a magician, fancy dress their experiences. competition and disco there will be Of course we have allowed plenty plenty of entertainment to keep the of time in the schedule for people children busy over the weekend. to catch up. We know that this is For parents, carers and other one of the most appreciated parts adults the presentations on of these weekends as it gives Our annual family weekend will Saturday will include talks about a chance for families to socialise be held at the Radisson Blu Hotel using adapted computers and and support each other. Further near Stansted Airport, Essex on technology and the changes to details about the weekend will be 14th and 15th October. As usual, disability benefits. The focus of published on our website once an action-packed agenda has the afternoon will be on research they are finalised. been planned. The children will be including talks from Prof. Steve 10 Support Services A-T Society News

PIP assessments There has been talk in the media The A-T Society is very aware of In the meantime, though, if you recently about possible future these problems. We consider it are sent the forms, please do cuts to disability services and ridiculous that so many people with contact us before you fill them benefits. This is clearly a matter of A-T are being required to undergo in. Likewise, if you are invited concern to people living with A-T an assessment visit, for a condition to an assessment, please get in and to the A-T Society. It comes as severe as A-T, and when they touch, so that we can advise or at a time when the Department of have previously had a lifetime support you. You can either email Work and Pensions is rolling out award of DLA. We have spoken to [email protected] the change from Disability Living ATOS and to the DWP about this. or ring us on 01582 760733. Allowance (DLA) to Personal However, it is currently a matter And finally, try not to worry. A-T Independence Payments (PIP) of policy that everyone with a is a seriously disabling condition, across the country. Many families disability, regardless of what it is and with the support of the A-T are receiving complicated forms or its severity, has to go through Society, we will be able to make to fill in and being invited to an an assessment, unless a doctor them see that. But if you are assessment interview. This can states that they are unable to do worried, please do contact us. be very stressful, particularly as so. Trying to change this policy is the system seems to be operating not something we can in an environment where there is do on our own, but we will work pressure to find that people are with others to try and do so. And able to work or are not eligible for if you have a chance to raise the the allowance. issue with your MP, that can only be helpful.

Direct payments Have you considered applying Direct payments are available to at www.carersuk.org. to the government for direct people over 16 living in England, If you would like to apply for direct payments? Direct payments let you Scotland and Wales. You can only payments visit the government choose and buy the services you get direct payments if you’ve been website: www.gov.uk/apply- need yourself, or the person you assessed by social services as direct-payments. are caring for, instead of getting needing care and support services. them from your council. If you, or Direct payments can be made to: Benefits fact sheet someone you care for, receive help Our benefits fact sheet from social services then you can • disabled people aged 16 or over provides a comprehensive apply for direct payments. (with short or long-term needs) overview to the benefits • disabled parents for children’s A direct payment is the amount of system and the different types services money that the local council/trust of benefits available. Hard has to pay to meet the needs of • carers aged 16 or over copies are available from Kay you or the person you are looking (including people with parental and Anne and each family after, and which is given to enable responsibility for a disabled will receive a copy with this you/them to purchase services child) newsletter. To download the that will meet your/their needs • elderly people who need factsheet as a PDF visit www. (as assessed by the local council/ community care services atsociety.org.uk/factsheets. trust). Many of our adults, for Applying for direct payments is not example, use them to employ a compulsory and so, if you would personal assistant for a few hours rather, you can continue to have FACT SHEET a week to enable them to enjoy BENEFITS the local council/trust continue There are many different benefits that you may Motability Scheme social activities such as going out be entitled to claim. Some are for children and adults who have a disability and others are for The Motability Scheme enables disabled people to arrange your support. Further their parents/carers. to get mobile by exchanging their mobility allowance for the lease of a new car, scooter or to the cinema or shopping. Children powered wheelchair. If you or your child is detailed information can be found receiving the High rate of the Mobility Disabled Living Allowance (DLA) Component for DLA or PIP then you would ‘DLA’ is a benefit for children (under 16) for help qualify for this scheme. Please go to their with care and mobility costs. It is not means- website for more information. tested. There are 2 components: ‘Care www.motability.co.uk Component’ – Low, Middle or High (depending on the level of care that they need) and ‘Mobility Component’ - Low or High (depending on how Blue Badge much help they need in getting about). You can If you're disabled or have a health condition that apply for the ‘Care Component’ from any age affects your mobility, you can apply for a blue and for the ‘Mobility Component’ from the age badge. You can also apply for a badge if you of 3. Most children with A-T will qualify for at care for a child with a health condition. If you least the middle rate of the Care Component get certain benefits you'll automatically be able and the high rate of the Mobility Component. to get a blue badge. Most people with A-T will Adults have a blue badge to help with parking and access. For more information on how to apply Personal Independence Payment (PIP) please go to www.gov.uk/apply-blue-badge

‘PIP’ helps with some of the extra costs caused by long-term ill-health or a disability if you’re Employment and Support Allowance (ESA) aged 16 to 64. There are 2 components ‘Daily Living Component’ and ‘Mobility Component’. If you’re ill or disabled, Employment and Most adults with A-T will qualify for the Support Allowance (ESA) can help with enhanced rate of both components. financial support if you are unable to work. The 2 types of ESA are: If you are an adult still receiving DLA then the Department of Work and Pensions will write to  Contribution-based ESA – usually you you to tell you when your DLA will end and to get this if you’ve got enough National invite you to apply for PIP instead. Most adults Insurance Contributions with A-T will qualify for PIP. (Children will  Income-related ESA – usually you get eventually move onto PIP from DLA when they this on its own or on top of contribution- reach 16). based ESA, if you’re on a low income A-T Society News Support Services 11

Wheelchair maintenance advice from A-T clinic therapists This article aims to provide a Postural management tips: • Are the knees level when looking reminder to manual wheelchair Postural management refers from the front, the same length users, (and their families) about to how the body positions and and positioned in the centre the importance of maintenance maintains itself against gravity, in facing forwards? Can this be of the wheelchair regardless of all positions. In sitting, alignment maintained when moving in the how frequently the chair is used. in an upright position is optimum, wheelchair, if not self-propelling? It is based on our experience with good symmetry on the right If the knees sweep to one side from the A-T Clinic in Nottingham, and left at the hips. The trunk consult your local therapist. over the years. should be in midline with the • Footrests – are these the correct Physical maintenance tips: head aligned above as much as height? Is the knee bent at 90 • Wheelchairs last longer if possible. This position needs to be degrees in sitting as it should maintained correctly. maintained when still or moving in be? (If too long or too short these need adjusting). • Are the tyres correctly or fully the wheelchair and be sustainable inflated? If not this will make over time too. • Are the push handles at the it harder to push or propel the Good postural management is correct height for the carer? wheelchair and can affect the preventative of pain or muscle • Check the wheelchair is stable ability of the chair to stay straight contractures whilst enhancing when the individual is seated when moving. functional independence and in it. Check size and weight • On a monthly basis, check comfort. remains correct for the chair, the tyre treads: are they still • Observe the child/adult when as this can affect stability and visible? Or have they been seated in the wheelchair. What safety, especially on uneven worn smooth? If bald (smooth) does their positioning look like? surfaces. patches are present or the tyres If they cannot keep upright over If the individual uses a powered do not look the same all the way time, extra trunk support may wheelchair for a majority of round, you need to inform your be required. (It is important to the time, it is likely the manual local therapists and/or contact be seated as symmetrically as wheelchair only has occasional your local wheelchair services possible). use. This in turn means that for a review, replacement. NB: • Is the backrest offering enough postural review in the manual worn tyres do not grip effectively support? If it is a soft backrest, wheelchair is often overlooked or and can affect the performance is it fastened (velcro is common) infrequent. It is essential that all of the wheelchair when being as firmly as possible? wheelchairs be reviewed regularly. self-propelled or pushed. • Is the backrest too high or too (Keep a record of when this is • Do the brake handles move low, from a comfort perspective due and book in advance). A easily into place? Are they or when self-propelling? useful time scale would be 12-18 effective at keeping the • Seat width and depth; is this still months, depending on growth, wheelchair stationary when correct (i.e. no more than two usage and postural changes. A on a slope? If not the brakes finger widths from back of knee service on a wheelchair should be may need tightening or other to the seat)? Seat is not too undertaken by a suitably qualified adjustment. narrow or too wide. and experienced professional • Are the footplates level and do • Seat cushions; is the seat either via wheelchair services for they remain so when weight is cushion still keeping its shape NHS-provided equipment (no cost put onto them? and not going too saggy? incurred), or via an independent • If a lap strap is attached is it a) • For gel or other pressure-relieving provider for privately-purchased in place and b) still able to be cushions, ensure they are stored equipment, (which the individual or secured? It should fit snugly level not on their side and that the family would need to fund). but not be too tight. In winter gel has not become lumpy. When If in doubt about any aspect of the accommodation is needed for pressure is applied and released manual wheelchair, contact your coats etc. does the cushion bounce back local Occupational Therapist or • Is the wheelchair still the into shape? If not, this needs correct size for the child/adult? replacing. NB: old cushions can Physiotherapist. Growth or other physical increase the risk of pressure This article has been devised and issues including comfort problems, discomfort and written by the Nottingham A-T can influence this. postural asymmetry of the pelvis, Clinic Therapists, Janet Corderoy which impacts on spinal position. and Sarah Jessop. Please contact us through the A-T Society if you have any queries. 12 Support Services A-T Society News

Breast screening in women with A-T This article is based on an Screening by mammography is or aunts may be and can request information leaflet produced by the not recommended for women testing, by asking their GP to refer team at the Papworth A-T Centre. with A-T due to their increased them to their local Clinical Genetics sensitivity to X-rays, although Service. Cancer risks in A-T mammography may have a role in Current guidelines recommend that It is widely known that people assessing an abnormality found carriers of the A-T mutation should with A-T have an increased risk of on MRI. If a woman is unable to be offered a mammogram every 18 developing various types of cancer. tolerate MRI scanning, she should months from the age of 40 until the While in children the cancers are be given information about breast age of 50 years, after which they predominantly blood cancers – awareness and ask her GP to refer will get three-yearly mammograms lymphomas or leukaemias – in her on should she suspect any as part of the regular NHS Breast adult life the range is more varied changes. including breast cancer, blood Screening Programme. A full cancers, endocrine tumours, There are a number of ways assessment of the family history brain tumours, and hepatocellular to access screening of cancer will help guide whether carcinoma (primary liver cancer). You may wish to see a Clinical any additional screening above Geneticist or Genetic Counsellor to this baseline is required. Women While it is sensible to keep an eye discuss your genetic status (types carrying the c.7271T>G mutation on the health of a person with A-T of gene alterations), implications for may be at higher risk of breast and consult a doctor when there other family members (or planning cancer. Genetic counselling is are any unexplained symptoms a pregnancy) and cancer risks. This particularly important for families or unexplained ill-health, the only can be done either locally or when of A-T patients with this mutation. formal screening programme you attend Papworth: They may be offered additional currently recommended is for MRI screening based on their breast cancer. 1. Your GP can refer you to family history. your local genetics service, Breast cancer risk who will then arrange the Any of these routes should result Breast cancer affects about one breast screening after your in your being able to access in eight women in the general appointment at your local breast regular screening. Once again population (12%) most commonly unit. Be aware, though, that you though, your GP or local screening after the menopause. Women may have to inform them about service may not be familiar with with A-T have a substantially the guidelines for A-T as they the guidance. If you have any higher risk of developing breast may well not have come across problems please get in touch with cancer from age 25 years. It has them before. Kay or Anne at the A-T Society been estimated that nearly half all on 01582 760733. women with A-T will develop breast 2. Alternatively, you can see a cancer by the age of 50 years but Clinical Geneticist as part of this number is based on very small your regular A-T review at the studies so we do not know how National adult A-T service, accurate it is. The level of risk may Papworth. Your screening be different for women with milder can then be done either at forms of A-T, but again we cannot Addenbrooke’s Hospital in be sure about this. Cambridge during your visit to Papworth or through referral to If you, or anyone you know, Screening recommendations your local breast unit. has been affected by cancer NHS England guidelines If you live in Scotland, Wales or then you may wish to visit recommend screening using an Northern Ireland and wish to have the Macmillan cancer support MRI scan of the breasts once per breast screening done locally, your website. Macmillan offer year, starting when a woman is local genetics service will need to 25 years old. Breast screening advice on living with cancer make the referral. aims to detect any developing whether that be practical, breast cancers at an early stage Advice for other family members medical or financial, as well so that they can be treated more Women who are carriers of the A-T as help and support for the easily. However, it may also find gene mutation have a moderately patient and family and friends abnormalities which turn out to increased risk of breast cancer. who are supporting them. be non-cancerous and do not Mothers of people with A-T are www.macmillan.org.uk require treatment. always carriers, while other female family members such as sisters A-T Society News People 13

Rachel lands her first paid job Rachel Ferguson has classic A-T her resilience in completing her but she hasn’t let A-T get in her placements. They also recognised way of pursuing her dreams and, that Rachel has an amazing sense with much grit and determination, of humour which shone through she has overcome every hurdle when the wrong bus turned up to get to where she is today. without a wheelchair lift for a She achieved seven GCSEs at college trip. Determined to go school and has recently graduated Rachel told her friend and teachers from Telford College of Arts and to just ‘manhandle her up the Technology. Rachel passed her steps’ which they obligingly did! three year BTEC level 3 course in Health and Social Care with Since leaving college Rachel has flying colours. embarked on her next challenge - her first ever job. She is now Rachel was recently interviewed employed as a part time website for her college newsletter and had Finishing my course took a lot administrator working with the this to say: of effort and I couldn’t have Bridgnorth and Morville Parishes Team Ministry. “When I started college, the staff completed it without the support centred the whole course around of my tutors. As well as supporting Her mother Joy said: “We now me. They put me in a normal group me, they also became my friends.” have a website administrator in of students, gave me a reduced The staff at the college were the family! Hopefully this is just the timetable, let me have study time so impressed with the way that start of an exciting new chapter after lunch to get on with any work Rachel embraced the course, in her life – I’m so proud of my I had, and allowed me to go home her determination to be like all daughter for landing her first at 3pm. the other health students and paid job.”

Jordan’s flying high

Jordan has always had a sense for people with disabilities and archery. He had the time of his life of adventure and doesn’t believe believe it’s what a person can do and loved every minute. He did that his condition should prevent that counts! With so many activities however, give his mum a fright him from tackling a challenge or and new challenges on offer, when his wheelchair decided to do two; in fact he featured in the last Jordan had a hard time choosing a wheelie on the assault course newsletter describing his driving which activities to do and tried but Jordan wasn’t in the least bit experience riding in sports cars. to fit in as much as possible on phased and found that it added to his break. the adventure. He recently went on holiday with his family to the Calvert Trust His grit and sheer determination Jordan has now decided what he adventure centre in the Lake to try everything really shone really wants is his own crossbow! District. The Calvert Trust offer through as he tackled the assault We can’t wait to see what challenging outdoor adventures course, zip wire, climbing wall and adventure he has lined up next! 14 People A-T Society News

A new addition to the family

Congratulations to Bea and Dan Rupert Prokofiev’s twin sister, who have become proud parents a regular attendee at the family to baby Luca born in December weekend and wonderful editor of last year. Looking after Luca is this newsletter. Rupert was keeping Bea very busy but it’s the a very proud uncle who enjoyed best job in the world watching her his cuddles with Luca and a little man grow and explore the lovely Christmas together before world. Crawling and solid foods he very sadly passed away are next on his agenda. Many of in January. you will know Bea as she’s

A baby sister for Winston Flying the flag for Great Britain

On 7th June 2017 Spencer had the honour to be the GB flag bearer for the European Clay Pigeon Shooting Winston is the very proud brother Championships at Lulworth of baby sister Wren who was Castle. Spencer is a well- born in June last year. They are known shooting enthusiast, definitely keeping mum, Sara, so was asked to bear the flag on her toes and are all having a by Graham Brown who runs lot of fun together. They dressed Purbeck Shooting School up as skeletons for Bonfire night and led out the Great Britain and, judging by the smiles on team. Well done Spencer – a their faces, they had a fearfully much-deserved honour. good time! A-T Society News People 15

George’s colour run Jake’s heading George took part in his very first colour run in June organised by off to high his school. As you can see from the big grin on his face, he loved school in every minute of it. His wheelchair September was multi coloured by the end of it but luckily it didn’t take too long It only seemed like yesterday to clean. to Jake’s mum when Jake started primary school but in September he’s off to high school. His family are so proud of him as he’s Orla joins Rainbows making another big jump all by himself, going to a Orla joined Rainbows last summer new school with none of and made her Rainbow promise his friends! He’s so excited just before Christmas. Joining and has already had his first Rainbows has been a really taster day at his new school. positive experience for Orla – she’s made new friends, tried lots of new activities and challenges and her Rainbow unit have been very inclusive.

A new baby sister for Ibraheem Ibraheem was so excited to meet his new baby sister, Juwairiyah, born in December last year. As the photos prove it’s very hard to get everyone smiling at the same time! We hope they both have many fun adventures together. 16 People A-T Society News

Erin’s new pink wheelchair – Taylor Swift

Anne interviewed the delightful Erin recently whilst she was waiting at Nottingham A-T Clinic for her appointment. Anne found out all about school and Erin’s new wheelchair which she has named Taylor Swift. Here’s her story with little a bit extra from Erin’s wonderful school special needs assistant Áine. Anne: So Erin, I hear ‘Taylor Swift’ has come into your life recently, what’s the story?! Erin: Well, I’ve always loved the name Taylor and I also like Taylor Swift’s songs. So when I first saw my new pink wheelchair I said ‘That’s not a wheelchair, that’s Taylor Swift!’ And we’ve been together ever since.

Anne: What a brilliant idea. What kind of things do you get up to with Taylor then? Erin: Well sometimes I’ll say ‘Come on Taylor, lets go for a ride’ and off we go to the park, the shops or whatever. Sometimes I ride ‘IN’ Taylor. Get it?!!!

Anne: Yep I get it. I guess Taylor comes in handy when you get tired. Erin: Did you hear about my speeding fines in school?

Anne: Erm, no! Please tell me more. Erin: The head teacher was always telling me ‘Erin you need to be slowing down now’. But I kept going too fast, so he’s started giving me fines!

Anne: Really? I bet you’ve slowed down now then, to stop getting into trouble? Erin: No, I just hand the fines to Áine and tell her to pay them! Áine’s my special needs assistant in school and she helps me out when I need it.

Anne: Well she’s going above and beyond if she’s paying fines for you… I think I’d like to find Each speeding ticket costs 20 cents but according to out more about this story from Áine! Áine none of the fines have been paid yet and there are lots of them!!! On further investigation, it seems Erin can indeed be Áine also reported that Erin’s class mates are very seen speeding around with her walker, both inside caring towards her. They never leave her out of any and outside of school. No amount of telling her to activities, which is wonderful. slow down worked. So in an attempt to reduce her speed, her head teacher resorted to issuing Erin with As for Erin’s mobility equipment; the other kids don’t speeding tickets whenever he spotted her zooming even notice it! They see Erin, not her walking frame along too fast! or wheelchair (oops sorry, I mean Taylor Swift!) A-T Society News People 17

Annie’s dedication recognised by Pride of Gwent Award Judges Annie Wills, whose daughter do anything for my daughter to Alexandra has A-T, won the Carer make her life easier. My daughter Award in the Pride of Gwent enjoys going out so I take her out awards run by her local paper and arrange things like hospital South Wales Argus. Her family appointments.” are so proud of her and say she When she found out that she had always goes the extra mile for won the Carer Award, Annie said: her daughter Alexandra. Despite working part-time she visits and “I felt in the beginning that if cares for her daughter nearly every anyone should win the award it day. Annie’s other daughter, Terrie, should be my daughter, but some nominated her for the award as people say that I do wonders she felt her mum deserved to know and I have two other daughters just how amazing she was. Terrie who also say that I go all out for said: “I don’t know where she gets Alexandra.” The most important the energy or strength from. My thing about winning the award for mum is the most selfless, kind and Annie is realising how much she giving person I know.” Annie wasn’t is appreciated and how much her N.B This is an extract of the story expecting the award at all so it daughters think of her and for that printed by South Wales Argus was a lovely surprise. Annie said she is really thankful although paper – the full story can be found “I did think ‘why me?’ because as she still sees it as being part and online on their website. a mum you do what you do without parcel of a mum. questioning anything. I would

Toni-Jo’s Boccia victory Five year old Toni-Jo should be extremely proud of herself after being awarded a medal, certificate and receiving a very loud cheer from the whole of her school. In November Toni-Jo and her three team mates played in a Boccia tournament. (Boccia is a disability sport that tests muscle control and accuracy. Players propel balls to land close to a target ball). Toni-Jo’s team came first out of the primary schools and 7th overall (most were secondary schools). They won a trophy for their school and came away from the event full of excitement and pride. A big round of applause to such superstars!

End of an era for James Congratulations to James Ferguson who has just finished secondary school. Over the past few months he has been diligently studying for his GCSE’s, and had to sit a two-hour Physics exam on his 16th birthday! James’s hard work has been recognised and he has been awarded a special award called the ‘Carroll Bennett Prize for Endeavour’. James enjoyed his time at secondary school and he was fortunate to have some very supportive teaching assistants and teachers. We wish him the very best of luck with his results. 18 People A-T Society News

Becky receives the Inspire Scholarship Congratulations to Becky Simpkin Using the gym helps with the ataxic for being awarded the Inspire movements, helps keep her lungs Scholarship. 24 year old Becky working properly and aids general has an active and busy life and fitness and well-being. Also, works hard at keeping strong because she is in a wheelchair full and healthy. time, it is important to strengthen her arms and legs. In November 2016 she was awarded the Inspire Scholarship Becky said “I was doing pay as you by her local gym, Gym & Tonic. go at Gym & Tonic once a week The Inspire Scholarship gives free with trainer Kate and we did a gym access to an inspirational mixture of things including the bike, member of the community. Becky the arm bike, resistance machines impressed the team at Gym & and free weights. Kate has recently Tonic with her sheer determination introduced me to Boxing which I to work hard to achieve her love!!! Since joining the gym I have fitness goals and they awarded actually put on weight & I’m getting her the scholarship in recognition stronger which is great for me and of her efforts. something I need to retain!”

Rowing for charity On the 22nd May 2017, I visit to the Papworth Clinic, I completed a 10,000m (6 mile) found that I had improved my by Eleanor Hewitt row for Meningitis Research lung capacity which is one of the in memory of my friend, Vicki, body systems that deteriorates in who passed away from bacterial A-T. I believe this to be a result meningitis and would have been of my cardiovascular training I supportive of my challenges. I do at the gym. Like I say, I firmly decided to uphold the work of believe a healthy diet/lifestyle to the charity who can help prevent be instrumental in slowing the A-T the same tragedy happening to progression in my case and would others. I successfully raised £150 love there to be a holistic diet and for Meningitis Research and know lifestyle approach on prescription my friend would be proud of my for people with A-T to follow. achievement. Anyway, the media interviews After appearing in a local sporting earlier this year and my many paper, a local radio station became sporting challenges have led to aware of my fundraising challenge local status as an ‘inspiration’. and followed my training for my My advice to parents of children row. They even made a video clip with A-T or people with A-T of me which is on Facebook. themselves is to not give in. I believe my diet and lifestyle to Prevention is better than cure. be a large contributing factor in No matter how long it takes or how the explanation as to why my A-T small the benefit, keep persisting hasn’t deteriorated for years. as small steps make big changes. I have a very active life which includes going to the gym twice a week as well as fitting in other sporting events. On my recent A-T Society News People 19

A place to call home The whole process started Council Home Improvement Team, a couple of years ago. Balmoral & the A-T Society. Maldon District Council Home We are proud to have been able I will never be able to thank any of Improvement Team came in took to work closely with Maldon you enough ever. You have taken measurements and made plans for District Council and Balmoral my fears away and given me back the house and showed us how and Property Services to help a family my own pride to know that I will be why things were being done for desperately in need of major home able to care for my child. We may us. Even down to things I wouldn’t adaptation. It’s been a long and have a hard road ahead of us all, have ever thought of. Once plans stressful process, but finally the but being in this home I know it will were confirmed it all went ahead work is complete and the family be that bit easier now. are loving their ‘new’ home. So so quickly. We were very lucky to what was this mum’s dream? be decanted into the property next You have given us a home to Enough space for a dining table. door, which the Maldon District be happy in and make the most The adaptations have given Council Home Improvement Team precious memories. Thank you her that, so now her family can & the A-T Society sorted out for all so much from the bottom of enjoy mealtimes together, plus us. This meant that my children our hearts x the children have a place to play were able to see all the works done games and do their homework. and were able to get to know the Balmoral team, who have not only A new home Here’s excerpt of the letter that done a fantastic job with the house the family wrote to express their at last! but have also supported the family gratitude. through what could have been a After a lot of letters, reports, Dear Maldon District Council Home really very stressful time. They even telephone calls and sheer Improvement Team, Balmoral and took the time to tell the children unremitting determination, the A-T society, what was happening and why, our family support manager Kay was finally able to get I needed to write to you to describe which meant a lot to them and us. Neil and Steven Wells into how having the adaptations done Our home is now a safe, open new purpose built residential to our property has been and try to planned, welcoming place that I’m accommodation close to explain how life-changing it is. It is able to care for both the disabled their family in a brand new very difficult trying to express just people in my life. You have given Seeability development in how much you have all changed our me back the ability to be able to Aylesbury. They moved in lives… When you’re told that your care for my child in a way she in October 2016 and have child has a condition such as ataxia- deserves. I have the room to fully thoroughly enjoyed settling telangiectasia. (or A-T for short) that complete her physiotherapy and into their new home. They is so life changing and has no cure, give her a place to be able to rest even have neat little gardens it stops you dead in time. freely. I have the facilities to wash outside their rooms which When I started to see what the her safely and for her to be able they look forward to using future holds for my child it hit me to get around the whole property this summer. that the place I call home will from inside to outside, front to back Neil and Steven were at an become her prison, a place she without having to have someone event celebrating the opening can’t move around or be safe in. I doing it for her. This will allow of Waterside House to which knew that something had to be done her to be as independent as she Suzanne was invited. When to provide her with a house she possibly can be, especially when they had all stopped eating could feel safe and secure in and she is fully wheelchair bound. I cake, they popped outside for we could eventually all call home. know I am so very lucky to have had support from Maldon District a picture! 20 People A-T Society News

In memory of Usman, Rupert, Siân, Amy passes and Wayne her degree Rupert Prokofiev sadly died on course with 2nd January after two years of deteriorating health, during which flying colours time he carried on determinedly in his usual independent way. He was a trustee on the A-T Society board, a new role for him in which he was beginning to see how he could make his own contribution. He spoke on stage at the family weekends several times and was keen for others with Usman A-T to speak up too. He thought of A-T as an essential part of who It is with great sadness we he was. The A-T Society was very announce the passing of three of important to Rupert and his family, our adults with A-T; , Siân Watkins providing much support as well as and Congratulations to Amy Rupert Prokofiev Usman a sense of belonging to a warm . They will all be very much Cooper who has just been Khan community. Rupert especially missed by their family and friends offered a place on a Masters enjoyed having fun with his peers and have left a huge hole in their degree course in Social Work at the adult activity weekends. hearts. Our thoughts are with them at Swansea University. Amy He leaves behind a proud family, all at this sad time. had to participate in a 3 part many friends and a wonderful interview which she passed Usman Khan’s mum, Neelum, team of carers. with flying colours. Amy had said “Usman sadly passed away Siân Watkins died on 23rd January this to say: in January this year. He will be 2017. This June she would have remembered as a lovely young man “I am a fun, caring person been 37. Of course all her family with a smile that lit up a room.” and have a good sense of and friends miss her dreadfully. humour, I always say follow She was tough, stubborn, clever, your dreams and don’t let and funny. Above all else, she was anything stop you from doing extremely proud of living alone and what you love! I have been determined to be as independent told that I’m a fighter. I have as possible. She wrote a piece that been studying in Swansea she wanted to publish in the A-T for three year at university newsletter, but never got around to for degree in Health and sending it in. Social Care, I received some fantastic news that I passed. Her family say it is how she At the end of my second would want to be remembered, year, I was nominated by my so we publish it now, as they tutors and the Dean of faculty request, with her own spelling and for an award from Ede & punctuation. Ravenscroft for ‘best student March 08 of the year’. I’m now going I started looking for somewhere on to study my Masters in else to live about 6 years ago. I Social work.” was living with my Mum and Dad at this point and I didn’t want to move too far away, plus all my family live in Nottingham and I Rupert wanted to be close to them. A-T Society News People 21

June09 We also sadly announce the All the adaptation was finished passing of Wayne Bugby, the about 3 years ago, then I could beloved husband and carer of start getting things ready to move one of our adults with A-T, Kath. in. At first I only stayed for the Many of you will remember Wayne odd weekend and then I gradually from our family weekends. Wayne started to build it up to a week and attended all of the events with so on. Kath, including all of the adult activity weekends and was well July 09 known within the Society. There I have been living in my bungalow was nothing he liked better than for just over a year now and I watching Kath enjoying herself, love it. It’s been a lot of hard work particularly when she had the but it’s been worth it. So don’t let opportunity to try canoeing for anyone tell you, you can’t because the first time and loved it. They if you really want it; you can, laughed together the whole day! I did it!!! Wayne will be very much missed I have two carers (personal by Kath, all the children and family. assistants) who both do 24 hours a week, that’s 7 hours a day. They do 52 hours between them but sometimes they go early because they’ve done everything. They help Siân me with things such as cooking, In Nottingham we have a group cleaning and helping me to have a called the Independent Living shower. Team (Part of Nottingham city They come about 10.00 and leave council) and their job is to give help at 4.00. We also go out a lot. We and support to disabled people go shopping in town or if I need to who want to live independently. go shopping for food we go to the Someone told me about a system Co-op or Asda or we sometimes where you can bid for a property, a just walk up to the local shops. flat, a house or bungalow. I made There is a café there where we three bids for a bungalow and my sometimes go for lunch. They third bid was accepted, that was leave my tea when they go and in October 2004 so you can see a snack for later and a couple of it’s been a long process to get too drinks. where I am today. We have even been to the theatre Someone arranged with me to and we have been on day trips so go and view the bungalow, I had we have lots of fun as well. about a week to accept or decline, Nov 09 I accepted the offer. That was two There were times when I thought I Wayne and a half years ago. couldn’t do it but I did it!! I couldn’t The bungalow needed adapting for of done it without my family’s and my needs. The bathroom had too my P.A’s help and support and I’m Share your news be ripped out and know I’ve got glad I did because I love it. Our next newsletter will be a wet room with a shower, there Who knows maybe one day you’ll out before Christmas so was an airing cupboard that took do it too? please send any stories, up half the space, that’s gone. The updates or photos to kitchen also had to be dipped, new [email protected] units and lower work surfaces and by 1st October 2017 if an electric door opening system. It took over a year to get that done. you’d like to be included. 22 People A-T Society News

My journey to employment by Kayleigh Aris 31 year-old Kayleigh Aris was diagnosed with mild A-T when she was 26. She has never let her condition define her and has always been determined to live as normal a life as possible. Kayleigh wanted to share her journey to employment and the challenges she faced to find a job. My ambition was to be a Primary School Teacher, so I undertook a degree in Childcare and in 2009 I achieved a BA (Hons) in Education, but to be honest this put me off working with children full stop! So I decided to have a career Kris a bowl of water whilst I was supports me financially to have a change. I applied for lots of jobs sitting listening to various talks on taxi to and from work. and had a number of interviews but how the Trust operated – I knew at had the feeling that my disability I feel lucky that I was given a this point that I was in safe hands had a bit to do with me not getting chance when I started working and that this was the place of work the job. All I ever wanted was for at RJAH and my line manager, for us both. an employer to give me a chance Alison, has never judged me. and see that my condition only In terms of the practicalities, I Instead she encourages me in affected me physically; I did have a was given a bigger desk so that I all aspects of my work and I will brain and I could use it! I do firmly could get a dog bed under it and always be very grateful to her believe that this is all each person the Estates Department put up for that. Following a Leadership with a disability wants - to be seen a poo bin on the field for him – course that Alison undertook, she as a person who has a disability being an assistance dog Kris is had to develop a member of her and not a person who is disabled. so well-trained, but I still haven’t team who showed potential - she managed to train him to clean up decided to develop me, which I started hearing really good things after himself as of yet, or to input made me very happy. As a result about Robert Jones and Agnes comment cards, but believe me I of this, my role has changed Hunt Orthopaedic Hospital NHS am working on it! dramatically since I first started Foundation Trust (RJAH), what a working here; I now have much good employer they were so I was Kris is very well known in the more responsibility. determined to get a job at the Trust hospital; in fact the staff here quite too. I had many interviews here, often know his name over mine. I do really enjoy my job and but was always second in line to For example, I can be walking I believe this has helped me getting the job. Determination to down the corridor and people will in my achievements. I have work at RJAH saw me through acknowledge Kris first – it’s really become much more confident and I successfully got offered a quite funny actually, but talk about and developed many new skills. job in the Clinical Governance stealing the limelight! However, it hasn’t been all plain Department – beating 60 other sailing – for example, I have had The Trust has been so supportive candidates for the post! to learn to work as part of a team, of my disability, I have longer which has been a challenge at When I started working in the lunch-breaks so I can exercise times and is frustrating. department, Kris, my assistance Kris on the field, away from the dog, and I came as a team so Heli-pad I must add, and they are One day I would like to have my steps were taken to ensure that flexible with my days off. Also I am own consultant to look after, being he would be made welcome and allowed to have time-off for my his/her secretary as I do like to a part of the team too. During my annual trip to Papworth Hospital nurture people and take them induction day at the very beginning and for yearly breast-screening under my wing. of my employment, a member of too. I also have Access to Work, staff came up to me and offered which is where the government A-T Society News Fundraising 23 Fundraising

Goodness, a whole year has families asking for help with and enthusiasm to support the passed with barely a moment housing, education, medical care, wonderful people who fundraise to draw breath since the last equipment and mental health we with us. In the meantime, newsletter. There have been either have to take our fundraising Suzanne and Sophie are here for some staff changes, with Eve to a new level or make some anyone and everyone who wants leaving and Sophie Arnold joining difficult decisions about what to assist the A-T Society with the team to provide excellent we can fund in future. For this fundraising and donations, and admin and Trust support. reason we are widening our we would love to hear your great fundraising opportunities by taking ideas for events and raising funds As our responsibilities increase on a community and events in the coming year. with more A-T diagnoses and fundraiser to provide new zest

Corporate supporters

We are delighted to continue our It was very exciting to learn that Hello Baby is a fabulous corporate partnership with our a friend of the Goble family had online baby store based in friends at Pizza Hut Delivery. suggested the Houghton Regis St Albans. They recently They have been immensely division of Aggregate Industries, opened a retail space in the supportive over the last couple a national aggregates company, town and are planning various of years, including opening their support the A-T Society this year. fundraising events in support test kitchen for our young adults Suzanne went along to meet some of local charities including a and offering space for the trustees of the team to hear about their coffee morning for the A-T meetings. It’s great to know that exciting plans. She remembered Society. They have also made every time someone orders a pizza that this isn’t the first time some very helpful donations online from www.pizzahut.co.uk Aggregate Industries have offered of products to support our they have the option to make a their support for an A-T family – fundraising for which we are donation to Children in Need and back in 2012 the company donated incredibly grateful. the A-T Society. supplies to a DIY SoS building If you have a baby, or want project in Liverpool to extend The staff at the company’s head to shop for one, you can a home for a young man with office in St. Albans are planning find the store atwww. A-T…coincidentally, the lady who various fundraising activities hellobabydirect.com. organised that donation was none through the coming year and we other than Lisa Noscoe, whose hope to involve some A-T children eldest son Jake was diagnosed and young people in at least one with A-T that year. of them. 24 Fundraising A-T Society News

Another company providing Nisbet’s, Bristol Luke Harrison valuable assistance is the local organised a team cake sale branch of national Chartered in support of Simone Kelly’s Accountants Mercer and Hole. Champagne Tour fundraising. They have been generous with The company plan to support us M&S Bridgnorth raffle prizes, sponsorship of the again later in the year. ball and shared lots of cake on a The amazing team at Marks ‘dress down and eat cake day’. and Spencer’s Simply Food in beautiful Bridgnorth have gone above and beyond in their charity of the year support for the A-T Society. With Joy and Rachel Ferguson regularly invited to the store and carrier bag charge money added to a fabulous fundraising total, it’s been a bumper year. We are doubly delighted to have been The Internet Watch Foundation chosen for a second time with work internationally to make the the store and look forward to internet a safer place for everyone working with the team again who uses it. We are very honoured through to summer 2018. to have been selected as their Oxford Children’s Hospital had 2017/18 Charity of the Year and a dress down day and raffle for look forward to meeting staff us. The mouth-watering prizes on later in the summer. Their first offer were most distracting and the fundraising event took place in team raised an excellent amount June with many more to follow. for A-T.

Tarmac Wolverhampton’s staff are hard-core fundraisers, they don’t settle for being ‘middle of the road’ and their dress down days are great fundraisers for Convergys is a global customer the A-T Society! services company with a base in Derry, Northern Ireland. Staff there organised a raffle in honour of Brae Sewell and raised a splendid sum for the A-T Society. Brae and his twin Broghan went along to draw the raffle and make lots of new friends. A-T Society News Fundraising 25

2017 marks the completion of Costa Coffee Kings Lynn Local support have been very active in their a magnificent five year Charity Our home town of Harpenden has support of the A-T Society via of the Year Partnership with the active Inner Wheel and Rotary our friends at Kings Lynn Cycle Pentagon Shopping Centre in Clubs and we were welcomed Club. They have organised Chatham. The Medway area has a by both last year to speak about fundraising days and high number of A-T diagnoses and our work and the special link with supported the club with their the centre has been a fantastic Harpenden where the charity was dynamic fundraising for us. way to share information and raise founded in 1989. It means a lot to awareness of ataxia-telangiectasia. us to establish a connection with We’ve enjoyed dressing up in a these two wonderful local groups. We would also like to thank a variety of costumes and taking Suzanne was invited to the June number of other national and part in parades and fun days. We meeting of Harpenden Village local companies who also provide thank the Pentagon Management Inner Wheel to receive a cheque match funding and more low-key team and all of the stores for their for £1,000. This was followed by a support for the charity. We very generosity and wish them success donation from the Rotary Club just much appreciate their engagement in the future. a few days later. with us.

Chelsea film Some of you may know that our Chairman, Mike Detsiny is a huge fan of Premiership Champions Chelsea FC. When he heard that Broghan Sewell was being scouted to join Chelsea Juniors, Mike was able to secure funding for a short video to be made about the very different futures Broghan and his twin Brae (who has A-T) will have. The film, ‘A Game of Two Halves’ also features Mum and Dad, Siobhan and Brian Sewell and was Photo-credit Olivia West shot entirely on location in and around the home they had moved into only days earlier! Talented You can find the film on our Film Producer Tom Worsley A-T Society YouTube channel created a very special video which and on our website. was featured in The Sun and on Channel 5 news when Broghan Make a £5 donation by officially signed for Chelsea FC. texting FOOTY to 70500

Photo-credit Olivia West 26 Fundraising A-T Society News

Global Make Some Noise

When we were adopted as one recorded a lovely ‘get well’ video of the Global Radio ‘Make Some clip to speed her recovery. But the Noise’ charities last summer, we best was yet to come… there was never imagined for a moment all of an air of excitement, little whispers the wonderful things which would and giggles as Lian and daughter happen during our amazing year. Alecia, dressed loud in honour of the day, were escorted into the It all started with a group of five Heart FM studio to meet Stephen young adults being invited to visit Mulhern and Emma Willis. As the radio station for interviews and they chatted away about Alecia’s a new significance for the charity VIP tours, meeting a smattering adoration for a particular megastar, and his family when Rupert died of celebrities along the way. For who should walk in but the man suddenly in January this year. Merry, Ebony, Jordan, Charlie, himself, Mr Michael Buble. If you Amy and their mums this was an Other visits were arranged for haven’t seen the video Global opportunity to tell their stories ready people with A-T who asked to be made, check out their website and for broadcast in the run up to Make shown around the radio station. see, for the first time ever, Lian Some Noise Day – October 7th. They were made welcome and utterly speechless as Michael mentioned on air during their Buble charmed his way still deeper studio tours. into Alecia’s heart! As the money poured in to Make Some Noise, the interviews with our five young adults were on air across the country, raising awareness of A-T for everyone On Make Some Noise Day itself, who heard them. We loved being Suzanne was up bright and early there and being part of something ready for a 7.30am live interview quite extraordinary. with Nick Ferrari on LBC. Before But that wasn’t all! Global were Suzanne’s knees had stopped determined to keep giving people knocking, Lian Yarlett was giving with A-T a voice and Rupert a mother’s perspective for LBC’s Prokofiev along with composer breakfast audience. brother Gabriel and William Davis It was quite a party at the Global were interviewed by John Brunning HQ with Mylene Klass playing the on Classic FM, talking about the piano in reception and rock and condition and their grandfather, pop stars wandering through. As Sergei Prokofiev. Rupert loved Kay couldn’t be there, Aled Jones taking part, and his words took on A-T Society News Fundraising 27

In January Suzanne was asked to Kaycee Carrington. As we stood that, so instead we want to give speak at a huge Make Some Noise in reception mouths dropped open you this” She handed over a fundraising Burns Night event and more than one chin hit the huge presentation cheque which along with Globals’ Jenni Falconer. floor when Take That walked past none of us could see… Then Suzanne held an audience of 500 us. The hyperventilating ladies turned it round to reveal £90,000! spellbound whilst she explained amongst the party were swiftly Hands flew to mouths (fortunately the impact on families of life with escorted into a room equipped covering a few excited expletives), A-T. At the end of the evening with a camera crew and a lot of Kay hugged everyone and there the event had raised a whopping Global staff who were clearly very wasn’t a dry eye in the room – £30,000 more than anticipated for excited. Kay and Suzanne were even behind the cameras! Make Some Noise and William miked up ready to be interviewed What a finale to our year with had shown us all how to dance a by our friend Jenni Falconer and our wonderful friends at Global, Scottish jig! the cameras started to roll… especially Hannah and Kara Weeks later we were invited to “Now” said Jenni, “you asked us who with constant good humour bring ‘someone who loves cooking’ for £26,000 so here’s a cheque encouraged and supported us to The CEO Cookoff, a feast of for that amount”. We all cheered throughout the process. a fundraising event for Jamie and Suzanne clutched the cheque Check out the celebration film Oliver’s charity. Step forward Jilly very tightly. made by Make Some Noise here: Shah who has raised over £2,000 “But” Jenni went on, ripping the www.makesomenoise.com/ for the charity with her ‘Jilly’s cheque out of Suzanne’s hand thank-you. Cupcake House’ fundraising page. with barely concealed glee, “we Jilly had a riot, meeting various thought we could do better than chefs and celebrities including Jamie Oliver, Emma Bunton and she even caught a smile from the Duchess of Cornwall! But the best was still to come. Our grant request to Make Some Noise was for £26,000 and we knew that would make a big difference to our family support in 2017. Global had other ideas and asked us to round up a small group of people to attend ‘an event’ at the station in mid-March. Media-savvy Ebony and Amy were joined by Gracie, Brae, Broghan and Kira and their Mums along with Mark and 28 Fundraising A-T Society News

Inspirational fundraisers Challenge Sara 50:50 for A-T! by Sara Metcalfe On nearing 49 in September 2016 I decided to do something challenging and different for a year to mark my upcoming 50th Birthday. As a family we have organised big Bash in the Barn Fundraisers every two years when the proceeds are split between the A-T Society and the MS Society. This was to be different and I would spend my year fund raising by completing challenges as a mark of respect for my gorgeous nephews, Charlie and Arthur, who face challenges every day with some strange looks and it was the North and meeting Ebony who their A-T life. My husband has only at the finish when runners was doing a massive challenge MS and some people did wonder actually began to ask what on herself. When I caught up with why the donations were not to be earth I was doing. them I was already halfway shared with the MS Society but I As more and more people heard through a four-day “Dress as a can help make his life easier on a of my idea the challenges started Teletubby” challenge so the look daily basis whereas we live miles coming in thick and fast – a on Matt Baker’s face when LaLa away from the boys. Roseberry Topping walk that I jumped out of the car in front assumed I was doing with just of him was a picture! I got quite my challenger, Mel, turned into emotional again talking to Ebony virtually a full village outing and and her Mum in the support bus they were all waiting to surprise and it spurred me on to make sure me in the car park as I pulled up. I raised as much money as I could. This made me quite emotional at the thought of so many people supporting not just me but my nephews and of course the charity so close to my heart. Challenges were to be set by On a frosty Sunday morning in friends and family and they had December you would have seen to be involved in the challenge as elves, Santas, reindeer and “real” well while donating to my cause. dogs running around my cross My husband set the very first one country course, jumping over – to get into my wedding dress jumps and into the water jump after 17 years of marriage and he for a fantastic, fun filled hour. would try to do all the buttons up! Warm mince pies and mulled wine Challenge one complete! followed to warm us all up. The Friends got involved and soon after sight of Phil dressed as Santa with came a 5km run dressed as Mrs a very full white beard zooming Elastica from The Incredibles film. along the top of the hill on his The lycra suit was a tad short, very mobility scooter is one none of us hot and with a full face mask and will forget. awful wig! This was a competitive One of the highlights was catching 5km race and I was the only one up with the Rickshaw from dressed up – needless to say I got Children in Need when it was in A-T Society News Fundraising 29

LaLa and Tinky Winky have made various appearances over the past few months as people heard how much my 15 year old son, Jasper, is embarrassed by it! Picking him up from school in a bright yellow suit has amused everyone except him on a number of occasions… I have completed runs, cycles, walks, cooked stews, made fruit vodka, helped at horse events and bathed Shetlands. I have clung to rock faces in the Lake District while hyperventilating and crying with fright and jumped into the sea challenging and I just want to do on New Year’s Eve dressed as something to show how much Mrs Claus, all pushing boundaries respect and love I have for them all, that some friends did not even while raising vital funds to support realise I had. I have “cantered” the Society that supports them. round the dance floor at the A-T If anyone reading this has a Hope Ball while wearing pink Dr challenge they would like to do and Martens (thank you Suzanne would like me to come along and Roynon for that one!) Why? support them while raising money Because whatever Challenges I for A-T then Challenge Sara 50:50 get set until September 2017 will for A-T !!! Contact Suzanne or in no way, shape or form match Sophie at the A-T Society and they those that A-T can deal the people can pass on the message and put and families affected by it. Those us in touch! that they overcome are far more

Walking for A-T - a family affair by Anne Saunders It was May 2014 after I had taken process (on the advice of my part in the Hope 24 event in physiotherapist) after having had Plymouth when the idea came two spinal operations in 2006. to me. I thought why not take Walking gives me time to reflect, something I enjoy and from that time to put things into perspective develop a plan to raise money for and at 64 years old is a very good a charity that’s so important to our way of keeping fit. family. When my beautiful great A couple of weeks after making niece Gracie was diagnosed with this decision my plans were halted A-T it was, as all families hearing – I had an argument with the this know, a really difficult time solid wooden leg of an armchair, dealing with the heart-breaking which I lost, ending up with a news. For the next few years my broken toe! This took more weeks thoughts kept turning to ‘how can I than I expected to heal so I had do something to help?’. to complete as many 5 mile circuits to postpone my plans. When as you can/want to within 24 hours At the start of 2015 I looked at 2016 arrived, I was even more set in beautiful Newnham Park. events I could take part in to set determined and came up with the I completed 10 circuits including myself walking challenges whilst idea of taking part in a series of some through the night; a new raising much needed funds for the walking events over the space of a experience for me walking with A-T Society. I’ve always enjoyed year to cover a total of 300 miles. only a headtorch to light my way. walking and in fact it became It seemed appropriate to start the a very big part of my recovery series with Hope 24, a challenge continued overleaf 30 Fundraising A-T Society News

I took part in six more events in the with a Gin in a Tin at Cabot following 12 months; the longest Tower…..wonderful! and most challenging being the My final challenge, and the 62-mile Race to the Stones which most important to me, was to was a non-stop walk over 21 hours walk the 65 miles from home along The Ridgeway ending at to Gracie’s front door. Over two Avebury Stones. I’ve been very and a half days I walked a route lucky to have amazing support I had planned using Ordnance from family and friends during all Survey and Google maps, with the events and in fact two of them the whole family (including my 8 were organised by my children, month old Grandson) supporting Matthew and Aimi, who have me. An emotional journey, a both been such strong emotional very appropriate finale and with support through all the challenges. a lot of plugging to friends and Matthew and his wife planned and friends of friends it (along with accompanied me on a 30 mile the other challenges) has raised walk along the SW Coast Path money which will be ploughed from Millbrook to Fowey. Just a into research to extend lives and stroll along the coast path I thought eventually find a cure. – I could not quite believe how many steps there were up from Looking back on the last year Polperro to the cliff top, followed and remembering that first ‘long’ by many steps back down, to be walk I took after my surgery – only followed by even more steps back three miles (but a very important up again and so it continued….. three miles) – I’m proud of what really tough on my legs but such I’ve achieved and I’m proud of my stunning scenery and all pain soon family for rallying to the cause and forgotten by the promise of a pint helping me make this last year’s of Cornish Ale waiting for me at 300 mile journey such a success. the end! Aimi’s planned walk was a Every step I took and every penny total contrast – together we walked raised was not just for Gracie but a 16 mile circuit around Bristol, for every young person living with taking in the Clifton Suspension this devastating condition. Bridge, Observatory, Cumberland My plans for the future? To keep Basin and many more sights that walking and if another challenge I’ve driven past, not realising how presents itself… remarkable they were. We finished A-T Society News Fundraising 31

Prudential RideLondon 2016 by Aisling Patterson In summer 2015 I set myself a challenge to cycle 100 miles in a day by taking part in RideLondon 100. I had never cycled 100 miles before, my closest was 90 and RideLondon would be a fitting event to reach this milestone. I offered my support to a small charity with a big heart at its centre, the A-T Society. I went to meet the A-Team, who were lovely, warm and so supportive. They provided lots of ideas for fundraising. We spoke about A-T and their work. I was extremely touched and humbled by the impact and the difference to so many young lives this wonderful charity made, their tireless work and the passion that drives it. It was new fundraising on my own. I set up an online donation site and sent emails to friends and family pleading with all to part with their money. I hosted a cake sale, baked 100 gingerbread bikes and held a coffee morning, and it all came together. I reached my target on 7th July and I thought ‘GREAT I can concentrate on training now!’ 10 days before RideLondon, I took a MASSIVE tumble when I hit a stunning countryside made famous out in front of you, nice and flat. I band of gravel. I somehow landed by the 2012 Olympics. There are have never experienced anything underneath my bike and my elbow 26,000 riders and the first thing like the fast and furious SPEED of took the impact and deep gashes, that strikes you about the event is the event. Riders need to reach but my first concern was my bike... quite how well organised it is. The certain points around the course did I scratch it! No. Phew! One signage into the starting pens, the on time or face diversion or even of my cycling buddies suggested marshalling at the start is excellent, getting swept up as a backmarker. I defer my place as I was on loading bays are colour coded, I marvelled at the average speed antibiotics, but I thought ‘NO WAY!’ there are porta loos in the pens for showing on my Garmin...24mph I wasn’t going to let my injuries those nervous bladders! on those flat miles. I kept thinking I just wanted to make it around the rob me of riding. My fundraising I was in awe. I just remember course without incident or injury. efforts and everything I had trained thinking to myself this is it, this It was about keeping safe and for and anticipated meant I was is ‘RideLondon’ hold onto this staying focused and constant risk adamant to ride. moment. We set out from the heart assessing. The day dawned with a very early of London towards Richmond park start on a wonderful course! It with its stunning views and then We soon faced the first and follows a 100 mile route on closed onwards and downwards to the shortest of our 3 big climbs.... roads through the capital and into Surrey hills. The first 40 miles roll Newlands corner, this was a gentle

continued overleaf 32 Fundraising A-T Society News

climb, next up was Leith Hill at mile 55. It is just under 2 miles long. The gradient varies constantly, and the road winds and it’s impossible to see more than 100 yards ahead. Next it was on to Box Hill at mile 65, on its approach crowds gathered and cheered, music With the hills behind me there A moment to savour when filled the air, guitars strumming, all was a wonderful exhilarating The Mall loomed into view with singing ‘Sweet Caroline’ it gave us decent into Leatherhead. With a Buckingham Palace in sight. a great mental boost. warm reception in Oxshott, and What a moment! It was magical. a gel or two, I was flying along The cheers from the crowds, the I must say I enjoyed the Box Hill the route from Esher past the euphoria, road lined with Union climb, 3 miles long with an accent river into Kingston and then on Flags, the finish line up ahead! of 557ft. It is a lovely smooth to Wimbledon where we faced Choked with emotion, the tears tarmac zig-zag climb, open with our last climb. By now we had welled in my eyes, I couldn’t stunning views and a pretty completed 90 miles, the end was believe I had done 100 miles in constant gradient. It was a pacey in sight. a time of 5 hours 52 minutes. climb and there was a feed stop at the top. I wasn’t going to stop I felt utterly exhausted the pace Mostly, I was just thankful to return but then I saw a rider drinking a was so fast and my legs had safely and to have completed nice chilled can of COKE! At that nothing left to give. It was all down the ride for such an amazing and moment in time I couldn’t have to mental endurance. As I turned worthwhile charity, the A-T Society. imagined anything better, I was the last corner, the cheers from craving a sugar and caffeine hit... the crowd spurred me on, and I so I caved in. I was in HEAVEN discovered a reserve of energy with my ice cold can of full sugar which pushed me on around and then I was off again. Trafalgar Square and that was it. A-T Society News Fundraising 33

Champagne ride countryside, with grazing cows and beautiful villages. However There was excitement, hard France is a big country with lots of big rivers in deep valleys, so there cycling, camaraderie and was plenty of climbing to keep us enormous satisfaction on the serious. And of course there was ride to Champagne, says that storm and the flash flood. William Davis We stopped every 16 miles or so to take on food and water and Thunder rolled around the black ensure that the front-riders didn’t sky lit up by flashes of lightning, get too far ahead. Frances would marble-sized hailstone rattled whip out her sketch book and off our helmets and stung any draw the scene, the well-equipped exposed flesh but the flat fields Jarno, would produce another around us offered no shelter bit of kit and Scottish tour-leader at all as we cycled across the Kevin would tell us the hills of the French countryside. By the time next stage were “almost flat - we reached a village, the road a few wee undulations!” was under a foot of muddy brown therapist, artist, gas-fitter, publican, water. We took shelter under an social-worker, art-installer to name When we finally rode into Epernay, archway, from where a kind lady but a few. Jarno Vannucchi from we pulled up at the Moët Chandon invited us in to drip in her kitchen. Erydel, the company involved in building to take photos with 20 minutes later the flood had the ATTeST trial, had come from the statue of Dom Perignon. vanished we made our way to the Italy to take part. And of course we popped a hotel under butter-wouldn’t-melt couple of corks for a sparkling Most of the group had set off evening sunshine. toast, followed by a few more from St Albans Cathedral early on that evening as we enjoyed an This was a bit more excitement Wednesday morning. Our friends excellent celebration meal. than we had planned on. The ride from Pizza Hut had turned up with from St Albans to Epernay was cheese-on-toast and coffee to supposed to be a new and original wave us off, the vice-dean came take on the traditional London to out to send us on our way with a Paris or Amsterdam, which we blessing and the first swifts of the hoped would appeal to potential year flew above us as we pedalled supporters in the area around off in the sunshine. our offices, close to St Albans. The blessing prevailed and the sun A couple of days out of the office, shone all day, without being too a good ride and a celebration hot. Still 104 miles is a long way, feast on arrival – naturally enough, especially if the route seems to with a glass of bubbly. go across most of the hills in the The final route was 350 miles south-east of England. And Garry over 4 days – with a 3-day option and Dave managed to miss a turn for those who wanted to join at and ended up doing an extra 12 Portsmouth. Two days involved miles. But eventually we all met rides of over 100 miles and both up in a pub in Portsmouth for a the English and the Normandy legs more-than-welcome pint and food, involved a lot of hills. only to be told “You’ve only got 5 minutes to eat, before we have to The group of riders was a real Overall verdict? Hard work but get on the boat!” mixed bunch, with people from extremely rewarding. And the most their 20s to 70s, some family or Cycling in France was a pleasure. important thing of all: everyone friends of people with A-T, others The roads were wide and well- raised well over their fundraising with no connection whatsoever. surfaced, with little traffic for the target. There was an amazing array most part. The weather was mainly of jobs too: tour-guide, beauty- fine and we rode through green 34 Fundraising A-T Society News

BBC One Show Children in Need Rickshaw Challenge About this time last year we Just two families responded and spent an exciting day in the studio received a call from the Producer both Ebony Robinson and Alecia meeting Little Mix and enjoying her of the Rickshaw Challenge asking Yarlett made it through to the final photoshoot with the other team us if we might have anyone assessment weekend where they members. We encouraged families interested in taking part in the met Matt Baker and other young to watch whilst we bubbled with event. He explained that it was a people who hoped to represent excitement. With one flick of her complex selection process with a range of different charities. We long dark hair, a star was born! two interviews, a fitness test and knew with two girls in the mix we On 10th November the special psychology assessment as well stood a very good chance of one promotional film about Ebony gave as an ability to cycle for a minimum of them being selected and we A-T the widest TV coverage ever… of one hour on a heavy trike. waited on tenterhooks for another so far! The donations started to roll week before the call came…Ebony in to Children in Need. had made the team! The next day Ebony and her mum The summer rushed by in a flurry Lorna set off for Jedburgh on the as Children in Need prepared Scottish Borders, the official start their website and filmed promo of the 2016 Rickshaw Challenge. videos of the team members. Kay travelled to Ebony’s home in Ebony was universally adored by Bournemouth to be filmed and CiN her team-mates, the crew and the worked with the A-T Society to tell presenters. If anyone thought she her A-T story online. couldn’t hold her own on the trike, well they were mistaken. She did It was a real struggle to keep the her bit through biting cold, rain, big secret and quite how Ebony wind, storms and snow and was and her family managed, we have This gave us lots of food for regularly doing over 2 hours solid no idea! thought, so we put a post on cycling at a time. As we reached mid-October the Facebook to ask families to tell Families living along the route planning and pre-publicity for us about their teens and trikes. came out to support the team with the Rickshaw Challenge was That’s the funny thing about our Merry James and Sara Metcalfe complete. We knew the team apparently random Facebook (dressed as a Teletubbie) waving would be revealed on The One posts, you just never know what the rickshaw through. might be behind our questions! Show on 19th October and Ebony A-T Society News Fundraising 35

of the rickshaw team who was greeted with hugs all round. On Children in Need Day the team cruised into London and did a circuit of the sights before reaching their final destination at BBC Broadcasting House. No one wanted it to be over, firm friendships had been made and this remarkable young team had raised over £3.5 million for Children in Need. Stop Press: If you are attending the Family Weekend in October, Ebony will be telling her inspiring Rickshaw Challenge Story in her own words. Don’t miss it, no seriously…don’t miss it!

Ebony gave the team a fright Challenge featured on the One when the rickshaw hit a large Show and the nation got to know stone and tipped precariously onto the six remarkable young people; two wheels before being caught Andy, Salar, Phoebe, Ross, by TV presenter Matt Baker and Olivia and of course Ebony. brought back to earth. Matt and At the end of the daily 10 hour the outriders were considerably cycle one of the team ‘rides in’ to more shaken than Ebony who just the destination for the night. With laughed! fanfares, cheers and an honour On Monday night The One Show’s guard from our friends at KLCC, coverage focused on Ebony as Ebony’s arrival town was Kings she cycled with Matt. She spoke Lynn and the Rickshaw was candidly about her A-T and the bedecked in pink and sparkles impact on her life and those of the with Ebony’s cycle helmet now friends she has lost to the condition. sporting a pink tiara. Ebony’s dad She explained how she makes the and brothers made the journey best of the life she has, knowing to surprise her and the Sprawling her A-T deteriorates every day. Matt family were there too. It was quite was unable to hide his emotions a party! and broke down on camera. There By Thursday the end was in were no dry eyes anywhere. sight and the Airforce Museum That night alone, the Rickshaw at Duxford was the night’s finish Challenge raised over £1 million. point. A contingent from the A-T As momentum for the event grew, Society and various young Ebony the team would stop at schools fans were there to greet the team. and to meet people in the towns on In the front row was Alecia, on route. Each evening the Rickshaw great form as an honorary member 36 Fundraising A-T Society News

Making a splash for A-T! Justine Sprawling and her friend Tracy Dugdale from Dereham swam 22 miles, the equivalent of the English Channel, last September in a swimming pool. They allowed themselves six weeks to complete the challenge, raising money for Brooke and the A-T Society with every stroke! The pool at Nuffield Health Farnborough was buzzing with A-T fundraising activity in December when Lesley Bromige and the Junior swimmers of took part in a week-long sponsored swim. Friends Jacky Ellis and Lucy Hodge both took part in a Swimathon, each swimming 5k for Winston and raising money for the A-T Society.

Dry January

Classical concert We had four Dry January fundraisers this year, all enduring 31 booze-free We had a great start to days. Alison Baldwin, Olivia and the fundraising year with Adam Langton and Jack Bartlett a magnificent classical spent the whole month of January concert in our home town of foregoing their regular tipples – Harpenden. The Hertfordshire they were able to save money, feel Chamber Orchestra, with healthy and energised AND raise our own William Davis on money while they were at it! double bass, was joined by conductor Graham Ross It doesn’t just have to be alcohol and the wonderful and you give up; super siblings Sophie celebrated violinist Jennifer and Matthew did the impossible in Pike. The concert was 2016 (in our eyes!!). For the whole a great success and the year, Sophie gave up sweets audience were spellbound by and Matthew gave up chocolate, Jenny’s lightning fingers and quite how they managed this is a emotional performance. mystery to us but we are all in awe of their amazing achievement! A-T Society News Fundraising 37

Party, party, party... Local supporter Jacqui Doyle held a Drinks and Raffle night, everyone enjoyed the wine & cheese on offer and due to lots of fantastic raffle prizes donated, which included beauty treatments, art and champagne, lots of raffle tickets were sold! A fantastic evening was had by all who attended the ‘Fizz’ fashion Show in Haslington near Crewe, organised by Sinead Ward. Jo Betts’s Aunt and Uncle had Every penny an anniversary party in Nottingham and kindly took donations on counts the night. One of our young supporters, And nannas like to party too! Flo, donated her pocket This is our amazing Knitting-Nana money to The A-T Society Nora proving that it doesn’t and was really happy when matter where you are, who you she received a thank you are or how old you are... you letter back! can make life brighter for everyone living with A-T by She is planning another finding your own way to fundraise. fundraiser soon, a cake Nora and her friends created sale after school. Thanks hundreds of beautiful knitted Flo, every penny makes a chicks to raise money. difference to us.

We had a ball! Our focus on building our support in the area around the office paid dividends this year. Thanks to the hard work of our former Corporate Fundraiser, Eve Audis, the first Hope Ball was held at the glamorous Walled Gardens at Luton Hoo. Local company, Plowman Craven, agreed to sponsor the event and the auction raised thousands, as well as the blood pressure of those who were bidding in the spectacular auction. Data management company LDex very generously matched the final auction total. 38 Fundraising A-T Society News

In the running! Brighton Marathon Jonny Luke, Patrick Turpin and Adrian Johnson ran the Brighton Marathon on 9th April in celebration of the life of Rupert Prokofiev. They were joined by last minute entry, Ed Reilly. The sun came out on the day and Rupert’s family were there in force, cheering on our team. This event has been a fixture in our fundraising calendar for a number of years – everyone loves the seaside.

Great Manchester North Devon Run Marathon A-T Society Marv Hine ran fundraiser and the North Devon cousin of Cleo Marathon for Brady, Suzie Lola and the A-T Hadfield ran her Society, this is half marathon one of the most in style. After challenging months of and visually training and a stunning events London huge slice of in the UK. This courage she achieved her goal is Marv’s first event as part of Marathon in Manchester this May. the Internet Watch Foundation’s Adrian Johnson – one Charity of the Year fundraising. man, TWO MARATHONS! Running a marathon is Worthing 10k always impressive, but running two is really rather Running isn’t wonderful! Two weeks Tracy Coyne’s after he ran the Brighton favourite Marathon Adrian got out pastime, in fact there again and ran the she actively London Marathon, sharing his dislikes it! For fundraising between us and this reason the Child Death Helpline at running the Alder Hey Hospital. Worthing 10k was a truly Lake Vyrnwy Also wearing our colours on amazing achievement for her. Simon Roscoe took on the the iconic course was Matt She set herself the challenge and Tarr, who completed his beautiful Lake Vyrnwy Half she smashed it, raising funds and Marathon for stepdaughter first London Marathon and awareness in support of Orla and dedicating his amazing run in Kayleigh to raise funds and A-T research projects with every awareness for A-T. honour of Rupert Prokofiev. kilometre! A-T Society News Fundraising 39

Colour Run Our very own wonderful Communications Officer, Wolf Run Kate McEleney, joined by her two young daughters, Wild running is THE new challenge David and Sean Cole, along and husband Pete, rose in the running world. A Wolf Run is with last minute stand-in Cameron to the Colour Run challenge a unique combination of off-road tackled the Wolf Run in searing in Brighton whilst having a lot running: mud runs, trail runs and heat this April inspired by James of colourful fun along the way! obstacle runs. A hardcore 10k and Rachel Ferguson and run across raw natural terrain, everyone with A-T. including open ground, woodland, cold lakes and thick mud.

Malvern Ultra 50 miles - all cross country - over the Malvern Hills. Total ascent: two kilometres. Total descent: two kilometres! That’s what the brains behind our “A Game of Two Halves” Chelsea Video, film-maker Tom Worsley did. Inspired by Brae Sewell he took part in the Malvern Ultra in May. The route goes all along the Worcestershire Way to the town of Malvern - a marathon distance up and down some serious hills, and all off road - then when he got there, he turned around and ran back again. That’s two marathons in one go and he Hastings Half was still smiling at the end! Marathon This year we had two runners in the Hastings Half Marathon, Simon Custerwell and Joe Powell did the A-T Society proud by running 13 miles, inspired by Joe’s son Winston. 40 Fundraising A-T Society News

Climb every mountain… Last year two brave men to the summit of Mount Kilimanjaro Gary passed the ‘Kilimanjaro conquered the world’s tallest in Africa. This is a difficult and baton’ to Daniel Fox who also freestanding mountain. dangerous trek which took Gary bravely took on the challenge and seven days but he took it all in his conquered this 5,882m beast of a A-T Dad, Gary Bromwich took on a stride late in October. mountain for his brother Conor and mammoth challenge and climbed sister Sarah.

Giving A-T a dressing down… Singing their hearts out!

Dress-down days have become In April Nav Cooper and the Joy Ferguson and the an increasingly popular way for Paediatric Department at Oxford Concordia Choir have been schools and organisations to raise Children’s Hospital had a dress- money for charity. They are simple down day and hamper raffle and working their musical magic again! In December, they to organise, everyone can get Anayah Lee and Gaelscoil Eadain warmed the hearts of Marks involved and of course, they can Mhoir had fun NOT wearing be lots of fun! uniform to school all looking cool and Spencer shoppers as while raising money for a great they sang in-store at M&S Brooke Sprawling’s school, cause. Ask your school to get into Bridgnorth and got everyone Swanton Moreley Primary had a mufti for A-T. It’s even more fun in the festive mood! Their dress-down day in February where with a theme. support continues in 2017 pupils traded boring uniforms for with a series of concerts in a their own, much more imaginative range of local venues. clothing! A-T Society News Fundraising 41

Hair-raising fundraising

Before After

Proving that you don’t need to run What inspired you to shave What will you do with your a marathon to raise money for your heads for charity? hair after it’s been shaved? charity, these fantastic folks from Lilly: Well, firstly I think women Lilly: We’re thinking about Hastings all got shaving! have a really hard time donating our hair! Three amazing young girls, sisters living up to today’s beauty Are you planning any different Alice and Bibi Dindar along with standards set by the media hairstyles/looks for when your their friend Lilly Cooper, decided to etc. So to defy them, and hair grows back? do something exceedingly brave to find beauty in not what the raise money. They were inspired mainstream media believes, Bibi: I want to experiment during by their friend and fellow resident I think will be massively summer with different of Hastings, Winston, to shave liberating for all of us! And colours so when it grows off their hair. then the idea of shaving our out it can be natural but heads for Winston and the whilst it’s short I want to We interviewed the girls to find out A-T Society came about, look like Eminem. more about why they decided to which was an amazing take the plunge and lose the hair! offer I couldn’t refuse. Whose idea was it to shave How have your friends and your heads? family reacted to this amazingly Bibi: We all had it in mind then brave decision? found out about Winston Alice: Some of my friends thought and it was definite. it was crazy and all of family Who will be up first when it have been very supportive. comes to the big day? Why did you decide to hold this Lilly: Maybe Alice because event at the pub? Do you have Emmett Ives shaved off his she’s super brave, or me lots of local supporters? remarkable beard for the because we’ll then build up Alice: We know a lot of people A-T Society at The Royal to Alice and Bibi’s massive will turn up to the event Standard pub in the old manes getting sheared off!! and the pub just has a town in Hastings. Ha-ha. very welcoming atmosphere rather than a hall, venue etc. 42 News A-T Society News

Skydiving superstars Walking on sunshine

The ‘Troopers4AT’ Team is made up of Lloyd & Sue Evans, Tim & Annette Hughes, Chris Madden and new boy on the block Paul Hughes. Inspired by Rachel and In support of Brae Sewell James Ferguson this Francesca Piccolo took part in a wonderful team took on Tandem Skydive with her brother The Bridgnorth Walk, a jumping from 13,000 feet over challenging 22-mile walk the Palm, Dubai. The resulting starting at Bridgnorth High photographs had us gasp in awe! Street and covering some hilly and often challenging Jayne Rainbow also threw terrain. herself from a plane and dedicated her amazing feat In spite of a testing route the of bravery to Lola Bloomer. terrific troopers smashed their target and triumphed for the A-T Society!

Fighting our corner Having never boxed before, Barry Thomson is currently Roddy Grant took an 8-week residing at Her Majesty’s pleasure intensive training course and in HMP Shotts in Lanarkshire. then got straight into the ring He has been a good friend to for a white-collar charity the charity and organises team boxing match. fundraising events for us when he can. A prisoner can ‘earn’ just Armed with an impressive level £11 each week, and many have of determination and his new- sponsored more than their weekly found skills he went on to win the wage, making Barry ’s 2017 team event for little Daisy Goble and fundraising total of £553. everyone with A-T. A-T Society News News 43

Cycling steps up a gear In anticipation of Sara Metcalfe’s 50th birthday she decided to do something rather wonderful: she signed up to take on 50 different challenges to be put to her by friends and family to raise money. Inspired by her two nephews who both have A-T she came up with this really unique way to raise money for the A-T Society. For her 27th challenge, Sara took part in a Mini Tour de Yorkshire which was a 45km cycle ride on a circular route. Along with some friends and supporters Sara got on her bike and cycled across Yorkshire with aplomb!

The Kings Lynn Cycle Club got The following day the club were busy fundraising this summer. joined by cyclists from all over They also did a GoRide challenge East Anglia as they took part in a to cover the distance from John Sportive event on a route through O’Groats to Paris, some 3838 laps the West Norfolk countryside to around the athletics track to cover raise money for the club and the the exact distance. A-T Society. There were stalls, activities and lots going on to ensure a fantastic time was had by everyone involved!

New A-T Christmas Christmas cheer cards on sale now! Festive fundraising was in full force with a Christmas raffle Families were involved with the held by Lisa Baker at the design of the 2017 Christmas Elvetham Primary School in Cards and we think they’ve done Fleet, while Sarah Jackson a stunning job. Our three happy organised a Christmas Talent angels Hope, Joy and Love will Show at Kings School in spread their light around the world. Worcester and the St Albans Packs of ten cards cost £4 plus Round Table organised a postage. We sold out last year, so Santa Sleigh Collection. order your cards soon. Purchase online from www. atsociety.org.uk/products !"#$%&$'()*%+*(,)*-(

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