Scleroderma magazine of the Scleroderma Society
news June 2013
Get Your Gloves On! 2013
welcome When we first thought about the idea of Get Your Gloves On! one of the aspects that appealed to us was the fact that it would be very unusual to see someone wearing gloves at the end of June and it would help to get us noticed and raise awareness. However, given its now the end of May and it’s 7 degrees outside suddenly the idea of gloves in Junes is sounding more like it might be a necessity for most of us.... Still if you have managed to get rid of your winter wardrobe by Friday the 28th June, we really do hope you will get your gloves out again and take part in Get Your Gloves On! Last year lots of people took part and we hope even more will do so this year – and so far this year we have had interest from as far away as Canada and Australia. And you can do as little or as much glove wearing as you like on the day – it’s really up to you but if you want some ideas do take a look at the website getyourgloveson.org. This year we are focusing on lunch – we want as many people as possible to have lunch with their gloves on whether you’re at home, school, out with friends, in the office or down the pub. And don’t forget to send us some pictures – we want more to add to the ones from last year. It’s about raising awareness, having a bit of fun and but more than anything it’s a fundraising opportunity and this year it couldn’t be easier to donate – you just need to text GLOV35 with an amount e.g. GLOV35 £5 to 70070. Finally I’m hoping to see many of you at the AGM on 29th June, full of stories about your glove wearing on the 28th! With best wishes
Richard Sunflowers The sunflower has been adopted as the international symbol of scleroderma. We will be using the image in our publications and also have a pin badge available. If you would like a pin badge to show your support please contact: amy@sclerodermasociety.co.uk
Richard Dodds Co-Chair richard@sclerodermasociety.co.uk
Scleroderma News
After careful consideration and plenty of consultation, we have decided to go with Scleroderma News as the name of the newsletter - hopefully it will do what it says on the tin. While Tightrope was felt by some people to really sum up their experience of scleroderma, there were others who didn’t. So Scleroderma News it is - certainly not controversial, but we hope you like it.
Contact us... Telephone: Mail: Email:
020 7000 1925 The Scleroderma Society Bride House, 18-20 Bride Lane London EC4Y 8EE info@sclerodermasociety.co.uk
society news “our aim is to become the support group for people with scleroderma” Annual General Meeting and FESCA AGM Email newsletter Porto was the venue of this year’s FESCA We would like to keep all of our Conference - Final Reminder Included with the newsletter you will find your official notification for our AGM and conference happening this year on 29th June - World Scleroderma Day. It will again be held at the Royal Free in Hampstead, London. We have a number of quality speakers lined up as usual. Professor Chris Denton will be taking your questions; Dr Linda Kwakenbos is travelling from Canada to talk to us about the SPIN project - a new and exciting research programme hoping to develop psychological interventions for people with scleroderma.
Get Your Gloves On! 2013
With this newsletter you should have received new materials for GYGO! 2013. Included should have been an Activity Pack as well as some stickers and posters. If you haven’t received your materials please contact us at the office and we will get some straight out to you.
Survey
In this copy of the newsletter you will see the first results of our members survey. We are really grateful to the many of you who returned the survey. It has enabled us to produce some really meaningful results and get some real information about how scleroderma is affecting people’s lives. In this edition of the newsletter, we have looked at the results of how people have been impacted in their personal lives as well as the clinical manifestations. In the next edition of the newsletter, we will be looking at the feedback from you on the services that the Society. The good news is, if you haven’t had chance to fill one in already is that the survey is ongoing and we intend to keep collecting information to add to our knowledge. So if you haven’t taken part yet, there is still time.
AGM. Scleroderma organisations from across Europe met to discuss current developments in the individual organisations as well as what was new across Europe.
members in touch with what the Society is doing and what is going on in the world of scleroderma. Often we have more to say than can be fitted into our newsletter so we like to send updates by email. If you would like to subscribe to these updates and have not already provided us with details of your email address please do so. We will never pass on your email address to any third party so you can be assured that it is safe with us.
Presentations were made on a number of new projects including the exciting DeSScipher project which is featured in this newsletter. More information on what FESCA is doing is available via their website at http://www.fesca-scleroderma.eu/
Joint work with the Raynauds & Scleroderma Association
The Brian Norris Appeal
August will see a great concert on behalf of the Brian Norris Appeal for the Scleroderma Society. There are some great acts lined up with music to suit absolutely all tastes. And on top of all that it is being held at the absolutely beautiful Ingatestone Hall in Essex. You would be mad not to buy a ticket.
Belper Games
The 20th July will see a special Belper Games. This year the games will be held in memory of Penny Wheeler who sadly died last year. Penny was a lovely woman and it is great to see the games coninue in her honour. If you are in or around Belper on the 20th you are advised to go along. Why not get involved in the Its a Knockout competition? The Scleroderma Society will be there so come along and see us.
The trustees of both organisations recently met to discuss ways in which the Society and the RSA could work more closely together to improve the support and services that we can offer to all of our members and non-members over the next years. We are currently looking at plans to collaborate on a number of publications with more things to follow after that. Keep an eye out for further joint initiatives.
Want to join or start a local group?
We have a number of active groups that meet on an informal basis to provide both information and support - and also an enjoyable time. If you would like to join a local group please see the details in this newsletter. However, we do not have the country completely covered. We need people to help start local groups - or be local champions - in the North West, the North East and the South West. We will provide you with full support. If you would like to help coordinate a local group please contact us.
Despite articles on these pages about running, walking, cycling events, fundraising is not just about the energetic and fit doing a lot! There are lots of other ways to help. Having a stall at a local fair, making things, or even, as Paul is doing, not cutting your hair or shaving for a year! Whatever ideas you have we can help. Just get in touch and we can supply you with leaflets, posters, collecting boxes, t-shirts, anything you need.
why we fundraise
Kevin Morton
My story is a pretty sad one to be fair as I’m sure most usually are! A friend and colleague of mine, Julie Ball, suffered from Scleroderma for a number of years, she sadly passed away in November last year. I’m ran the marathon in her memory.
Vikki Roberts
I’m supporting the charity not only because I think it’s important to support lesser-known organisations and a while back my Aunt was diagnosed with Scleroderma or CREST as she refers to it. I knew nothing about the condition but wanted to do something to help so as well as raising funds for the charity I also want to raise awareness and take part in some really cool things that my Aunt can experience vicariously!
James Pepe
I decided to run the 2013 Marathon for the Scleroderma Society, a fantastic charity that provides support for both people with scleroderma and their families. Having lost my father to this rare and incurable disease in 2002, I felt the time was right to not only honour his memory but also raise money for a charity that does great work in making people’s lives better. Most of you will know that I am not a long distance runner, so the marathon will be one of the hardest tasks I’ve ever undertaken. With your support I know I can get over the line and I thank you in advance for whatever amount you can spare.
Jennifer Zielinska
My dad’s mother passed away in 2003 after suffering for a long time with scleroderma and strangely for this rare disease, we also lost a close family friend to the disease shortly after. So, as you can imagine, raising money for awareness and a cure to this awful disease means a lot to us.
In Memoriam
Every year many people donate to the Scleroderma Society in memory of a friend, family member or other loved one who has died due to scleroderma. These donations are incredibly important to the Society and are most
Once again, we had some wonderful runners in the London Marathon putting themselves through all sorts of pain in order to raise funds for us. Huge congratulations to them all! They all did tremendously well on what was quite a warm day in London. As a marathon runner myself I know exactly how they were feeling at the time and in the days that followed. A mixture of a real sense of achievement, relief and pain! For the record, their times were as follows:
James Pepe 5:57:48 Vikki Roberts 6:02:44 Jen Zielinska 4:44:40 Kevin Morton 3:57:27 Greg Meacham 5:16:46
So far, the total they raised between them is over £9000 - a fantastic achievement!
gratefully received. We now have a new service which will help support people who wish to give in memoriam donations. we have joined with Much Loved to enable people to produce their own in memory pages.
Through this site you can upload pictures, messages and even videos. If you would like to know more about this please contact us or go straight to the website on www.scleroderma. muchloved.org.
We now have a new way to make a donation to the Scleroderma Society. Not only can you give by cash, cheque and online, you can now TEXT. It is a really easy process and because of Vodafone, all of the money that you donate comes directly to the Society with no administration or other fees. All you have to do is TEXT our code to 70070 and include the amount you wish to give. For instance, at the moment we have a TEXT campaign for Get Your Gloves On! 2013. So for example TEXT GLOV35 £5 to 70070 to donate £5. Easy.
fundraising Heroes As you can see, Vikki Roberts ran the London Marathon. But that isn’t all Vikki is doing to raise funds for the Society. Having already run the Paris Half Marathon, Vikki took part in the gruelling Tough Mudder in May and recently completed a triathalon. As you would expect from an event with a title like that, it is a very difficult thing to do. But Vikki did both! You can read more about Vikki’s incredible exploits at her blog - cantmoveitclimbit.blogspot.co.uk. But we have included a bit about her recent triatholon below.
“
I didn’t expect to enjoy the event nearly as much as I did. I grinned most of the way round, and made some of the marshalls laugh when I shouted gleefully each time a completed a lap on the bike. The bike ended up being my favourite part. I couldn’t feel my legs once I got off so found the run challenging but still achieved a good pace.
“
The weather was so lovely we had a little post-race picnic by the car, watching later wave cyclists zooming past. It was a wonderful event, a great day out and I would love to do another. I think the only thing I was slightly dissapointed with was that you don’t get a medal.
Vikki appeared in her local paper the “Farnham Herald”. Fundraising for charity will often get covered locally and is a great way of raising awareness of the cause. If you would like help to do this contact us on info@sclerodermasociety.org. uk or call the office.
Fundraising or hair-raising? Paul and Kealey Le Grave came up with a novel idea for raising funds. Paul decided to not cut his hair or shave for a whole year! Starting on 1 January 2013, Paul is going right through to 31 December without going near a razor! Keep a lookout on our facebook page for updates on what Paul looks like. As with everyone who raises funds for the Society, Paul has his story to tell. “My mum is an amazingly strong woman. She has 4 children and has always been there for us. She was diagnosed with Scleroderma a few years ago now and is in constant pain which she never shows and has to take many tablets every day for the rest of her life. She is always in and out of hospital but she has never let it get her down. She is always so strong and never complains. She cooks a family dinner twice a week for at least 11 people. She is the heart of our family. She is always the one we all go to for advice and help, now I want to do something to help her. Scleroderma is not well known and even GPs are not fully aware of the disease. We need to help with raising funds for more research and to raise awareness.”
Our survey said... Many of you have completed one of our 10 page surveys over the past few months. We now have well over a hundred surveys completed which gives us figures that are meaningful and tell us some interesting things about how scleroderma has been affecting you. In our next newsletter we will be looking at how you rate what the Scleroderma Society does to support you but here we outline some of the personal and clinical factors that have an impact on your day to day life. How do you compare?
clinical presentation
The Face
FINGERS AND HANDS
No 5% Yes 95%
Fingers and hands
It is no surprise to see that the majority of people surveyed with scleroderma had problems with their fingers and hands with only 5% saying no. This fits in with what we know about scleroderma as a whole. We now need to do more work to find out about the impact of digital ulcers and calcinosis.
We asked a number of questions about the face and this showed perhaps, an area in which we should fund more research or produce more support and information literature. Almost 60% said that their face had been affected by scleroderma with the majority also being affected in the mouth. Leading on from the mouth, almot a third of people surveyed said that their teeth were affected - it is clear that we need to look at this area and look for more dentists who have some experience of treating patients with scleroderma and to make their details available to members.
FACE
No 42%
Yes 58%
MOUTH
No 40%
Yes 60%
TEETH Yes 31%
Almost 45% of people stated that they had had problems with their eyes. We did not ask what kind of problems that people had with their eyes but assume that a significant number had secondary sjogren’s syndrome.
STOMACH (INDIGESTION & REFLUX)
No 69%
EYES
No 25% Yes 44% No 56% Yes 75%
The Gastro-intestinal System
BOWEL /ANUS
No 49%
Yes 51%
Do these results tally with your experience? We would be very interested to hear of your experiences, especially with relation to the gut. We are currently reviewing our leaflet and information with regard to the gut and would appreciate feedback from people who have been affected in this area.
NECK (SWALLOWING)
No 49% Yes 51%
The majority of people had some difficulties with their gastro-intestinal system. Three quarters had experienced either indigestion or reflux while just over 50% had had problems with either their bowel or anus. Just over 50% had also had problems swallowing.
Yes 18%
Prefer not to say 2%
No 80%
One of the areas that we were particulary interested in was the impact of scleroderma on people’s lives. Much is know about the clinical presentation of scleroderma but less is know about how it affects individuals in emotional or psychological ways. We asked a number of simple questions and while the answers did not provide a surprise, they did point to areas in which the Society could possibly do more in terms of support.
HAVE YOU HAD EMOTIONAL PROBLEMS?
No 46% Yes 53%
Prefer not to say 1%
Relationships of people with scleroderma were obviously affected with a lmost a fifth of people saying that they had had relationship problems and a third saying that they had had sexula problems.
HAVE YOU HAD SEXUAL PROBLEMS?
Over half had experienced emotional problems due to their scleroderma and this is clearly an area where more work needs to be done. Ability to work was also significantly impacted with 63% saying that their ability to work had been affected and that of the people surveyed almost 80% were not economically active although that number did contain a significant number of people who had retired from work.
Yes 33%
No 54%
Prefer not to say 13%
2 to 3 years 5%
No 35%
Yes 63%
3 to 5 years 14%
1 to 2 years 7% Under 12 months 3% Still seeking diagnosis 1%
5 to 8 years 14% 8 to 10 years 12%
Age Range 25 20 15 10 5 0
Prefer not to say 2%
PEOPLE ECONOMICALLY ACTIVE Not economically active 79%
Prefer not to say 1%
Economically active 20%
GENDER Male 11%
Female 89%
gender
diagnosed age range
Over 10 years 44%
HAS YOUR ABILITY TO WORK BEEN AFFECTED?
work
If you have not filled in a survey and would like to, please email us or call us at the office and we will send one out to you.
DIAGNOSED FOR..
personal factors
scleroderma strikes at the heart of who you are
HAVE YOU HAD RELATIONSHIP PROBLEMS?
Get Your Gloves On!
Lunch in your gloves!
Organise a fundraising lunch
How about organising a fundraising lunch. Invite a few friends round for lunch on the 8th or 29th June, take a picture of them eating in their gloves and ask them to make a donation to the Scleroderma Society for the cost of the lunch. Its an easy thing to do, raises awareness and money and is also a great reason to get together with people that you like.
Put up a poster
This year we are producing a range of different posters in two different sizes. A great and very easy way of raising awareness is to put one up. You can do this pretty much anywhere. At home, why not put one in your front window so people walking past can see. If you go to work, a community group, the library or even your GP’s surgery, why not ask if you can put one up on a noticeboard.
Activity Pack
This year we are encouraging people to have their lunch wearing gloves. Sushi in boxing gloves anyone? Why not wear your gloves to eat your lunch in. You could be at work, at home with friends or family or out at the pub or a cafe. You can use the materials we will provide to explain what you are doing and, if you can, get people to make a donation to the work of the Scleroderma Society.
Get Your Gloves On! 2013 is around the corner and with this newsletter you will have received copies of our activity pack, posters, bookmarks and stickers. You can get involved in any number of ways from putting a poster up in your window to writing to your MP or local newspaper all the way to organising a gloves based fundraising event. We are here to help you out so if there is anything you need that we have not already sent you please get in touch. We can help with a local press release, a letter to your MP, or a poster designed especially for you. And remember, you are part of a much bigger group both in the UK and overseas. But it is Get Your Gloves On! all down to individuals and what you do - many thanks in for people with Scleroderma on the 28th June 2013 advance. If you haven’t got your activity One of the posters that pack yet or would Your GlosclevesrodeOnrma! Get for people with like extra copies have been specially made or our friends at please contact Scleroderma Australia us at the office or on where different versions are being used info@sclerodermasociety.org.uk. around the country 29th June 2013
information om.au for more
sclerodermaaustralia.c
getyourgloveson.org
Upload your pictures We want to see you wearing your gloves. Whether you are giving us the thumb’s up in your favourite pair, eating your sandwich in cricket gloves or wearing your gloves in a group - let us know by uploading the picture to our Facebook page or on Twitter. and if you don’t know how to do it, why not ask a son, daughter, grandchild or riend to upload your picture.
International Scleroderma Day
International Scleroderma Day is held every year - since 2009 - on the 29th June which is the anniversary of the death of the artist Paul Klee. This year a number of countries including Canada, Australia and Greece will be celebrating the day by Getting their gloves on! At the Society we are celebrating on both the 28th with GYGO! and on the 29th with our annual conference is being held on the 29th at the Royal June 2 Interna 9 Free Hospital. tio
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SCLERODERMA – THE SEARCH FOR THE CAUSE The epidemiology of systemic sclerosis or ‘who gets scleroderma?’ This article is about the systemic form of scleroderma, also known as systemic sclerosis or SSc. It does not include the localized form of the disease (morphea). It has been clear for many years that systemic sclerosis (SSc, scleroderma) affects women more than men (approximately 80 – 85% of SSc cases are women), and that adults are more often affected than children. However, it has only recently become clear that there are major differences in the geographic distribution of the disease. Studies of prevalence of a rare disease are somewhat difficult to conduct, since mild cases may be missed and therefore not counted. A study of scleroderma in Northeast England in 2004 estimated the prevalence to be about 82 per million and a study in Japan in 1991 only found 38 cases per million. The highest prevalence of SSc has been reported to occur in the Choctaw Native American group with a higher prevalence (660 cases per million) than the U.S. population in general (250 cases per million). The Choctaw tribe originated in the Southeastern part of the U.S. and were relocated to the West (Oklahoma) in the early to mid 1800’s. In 1990 Dr. Howard, a rheumatologist trained at the University of Texas, was assigned to the Indian Health Service Clinic on the Choctaw reservation in Oklahoma. It was there that he observed a ‘surprising’ number of scleroderma cases, a disease that was supposed to be rare. This observation led to a formal collaboration with researchers at the University of Texas to calculate the prevalence of scleroderma among the Choctaw and to try to identify the reason that there were almost 3 times the expected number of cases in this group. As the story unfolded, the affected individuals, who were not known to be closely related, were all descended from a single ‘founder‘ family, suggesting that there was a genetic component to their susceptibility. One of the questions that naturally comes to mind is the simple query: ‘Does scleroderma run in families?’ Up until about 2001, the answer would have been a categorical ‘no.’ Most scleroderma patients have never heard of the disease prior to their own diagnosis, and have no affected family members. However, when this question was addressed in a systematic way, the answer was surprising. It is true that 98% of scleroderma patients have no affected family members, but 2% actually do have a first degree relative (parent, sibling or child) who also have scleroderma. This study was done looking at a group of over 700 scleroderma patients in the states of Michigan and Texas, There were 14 multi-case families among the 704 cases. Although this is a relatively small proportion of multi-case families, it is actually far greater than one would expect. This also explains why most physicians, including most rheumatologists as well as many patients, were reluctant to consider that there might be a genetic component.
Maureen D. Mayes, MD, MPH Professor of Medicine Division of Rheumatology Director, Scleroderma Clinic and Research Program University of Texas – Houston Medical School
This led to a search for the genes that could be responsible. The first genome-wide association study (GWAS) in SSc was published in 2010 and included 2,300 cases and 5,200 controls. This and several other studies have now discovered about 30 genes that are clearly associated with scleroderma and most of these genes are related to immune function. I was surprised that many of these gene variations are also seen in lupus, rheumatoid arthritis and other auto-immune diseases. Thus far, we have not found an association between genes that affect collagen metabolism (collagen is the main component of scar tissue and fibrosis) and the development of scleroderma. An international collaboration of U.S., U.K., Canadian and European investigators has come together in order to do these studies. This research is still ongoing, and as usual with research, there are still more questions than answers. We have not yet identified all the genes that contribute to scleroderma susceptibility nor do we know what effect these gene changes have in immune responses. It is clear that genes play a role but do not, and likely will not, explain why scleroderma occurs in some people and not in others. There must be something in the environment that triggers the disease. This “something” could be a viral or bacterial infection or some other environmental exposure. There is much conjecture but little hard evidence regarding the nature of this external or environmental trigger. It is tempting to speculate that this is a virus or a bacterial infection that interacts with particular genes resulting in a chronic auto-immune disease. According to this theory, the infection might be a common one but only causes scleroderma in those with the ‘right’ or perhaps ‘wrong’ combination of genes. Several studies have investigated exposures to environmental agents like silica dust (miners and sandblasters), dry cleaning chemicals, and other substances as possible triggers. Thus far, the only convincing evidence has been for silica dust exposure. However, out of the more than 2,500 scleroderma patients I have seen, only one has had this exposure. That leaves 2,499 cases with no identifiable cause. In collaboration with researchers in the U.K., Canada, Europe and – most recently – China I am optimistic that we can solve this mystery, and, by identifying the responsible genes, figure out what the likely triggers are. We all look forward to the day when scleroderma will be understood, curable and, therefore, uninteresting.
Hannah tackles the Yorkshire 3 peaks
Hannah Williams is busy raising money for the charity as well. Hannah says: “On Saturday 27th July 2013, I will be taking part in the Yorkshire 3 Peaks challenge - 24.5 miles, 3 peaks and only 12 hours to do it in! Anyone who knows me will understand how hard this will be for me! I am raising money on behalf of my mum, Julie who lives with this condition. She still works even though everyday tasks can sometimes be challenging, but she never complains!! Scleroderma is a rare and life changing condition and there is no cure.” You can help Hannah reach her goal at http://uk.virginmoneygiving.com/HannahWilliams7
fundraising
Jake sets his sights on a Tuff Mudder
We have produced some new fundraising resources to help people spread the word on scleroderma. Along with the normal things like sunflower pins, bracelets and pens, we have also produced a new basic leaflet which explains a little about scleroderma and the Scleroderma Society - these are great to use when collecting or talking to friends about why you are supporting the Scleroderma Society. We also have new blue collection boxes and we are awaiting the delivery of a new set of cardboard home collection boxes that can be stuck in the kitchen or at a party or celebration. We also have t-shirts announcing that the wearer is supporting the Scleroderma Society. If you would like one of these please let us know. All of our fundraising resources are available for free and we are also happy to help if you would like a special poster or leaflet for your event. Don’t hesitate to ask.
Jake Stevenson is taking on a very difficult challenge to raise funds. Here is his story. I live in Calne, Wiltshire, I’m 25 years old and I’m a landscape gardener. Me and my group of friends (Tom, Nick, Ben, Dave, Lawrence and Tom) are competing as a team in Tough Mudder 2013 on September 21st. We’re raising money for the Wiltshire Air Ambulance Trust and the Scleroderma Society. I picked the Scleroderma Society because my mum is a sufferer and a frequent visitor to the Royal Free Hospital in London. My mum has a very severe case of Scleroderma which is life threatening. She’s on oxygen 24 hours a day and has a continuous infusion of epoprostenol to keep her alive, that’s delivered through a pump that goes straight into the heart. We also picked the charity because the disease is little known, both publicly and by professionals. We hope any funds raised will help towards further research of the disease, treatments for the disease and help and information for the families affected by it. We also want the good work of the scleroderma society and the disease itself to be recognised nationally.
Presdales School
Presdales School made a magnificent effort this year to raise over £12,000 for the Scleroderma Society. Pictures and reports will follow but suffice is to say that the fashion show was fantastic and our Co-Chair Kim Fligelstone had a brilliant night with all of the students and staff involved. We are very grateful.
Coming Up Whether you walk it at a pace that suits you, jog parts of it, or take it on as an epic riverside run - the Thames Path Challenge will be a unforgettable and rewarding experience. Sign up in teams of friends, family or colleagues and help each other; join as an individual and meet fellow trekkers along the way! For more info on the Thames Path Challenge contact Tony at tony@sclerodermasociety.co.uk
14-15 September 2013
Another way to fundraise for the Scleroderma Society is to take part in the Thames Path Challenge. You can walk it, jog it, run it or a combination of all three! It is without a doubt a tough challenge but there will be plenty of support and help along the route. There are various options from 50km to 100km
Entries have now closed for the 2013 RideLondon - good luck to our riders. We hope to have their stories and pictures in the next newsletter. However, if you feel you would like to take part in the event next year, why not let us know so we can confirm and save you a place. We have a limited number of bond places so the earlier you get in the better.
P.S.
We have linked with Skyline to provide exciting fundraising opportunities such as sky diving, white water rating and treks. Please let us know if you would like to take part and we will help to organise it for you.
The greatest European initiative ever for systemic sclerosis, with financial support from the EU By Beata Garay-Toth, administrator and manager of the FESCA aisbl participation in the DeSScipher project DeSScipher aims at improving clinical practice in the management of the rare autoimmune rheumatic disease systemic sclerosis (SSc), for which no orphan drug but only off-label treatments are available. Over a three-year period, and building upon the expertise of an established, large, multidisciplinary and experienced consortium combining physicians, biostatisticians and biologists with long-standing experience in the field of SSc, the DeSScipher project will provide a systemic, integrated approach to the disease. This approach will be based on observational trials, which will address different crucial dimensions of the systemic disease: (i) early detection/prevention and treatment, (ii) functional impairment and severe complications, (iii) its affect on different organ systems (skin, joints, lungs and heart), (iv) juvenile and adult patients. The specific objectives of the DeSScipher project are: lTo improve clinical practice in the management of SSc for both adult and juvenile patients; lTo facilitate earlier detection and prevention of SSc and its organ manifestations and subsequently initiate disease- or organ-specific treatment lTo improve both quality of life and morbidity/mortality in SSc by addressing functional impairments caused by digital ulcers and hand arthritis, as well as life-threatening organ complications due to interstitial lung disease, pulmonary hypertension and severe heart disease lTo develop and validate a reliable algorithm for detecting organ manifestations of SSc at an early stage in adult and juvenile patients at risk using novel tools such as the VEDOSS tool lTo compare outcomes of prevention and treatment regimens in SSc to define appropriate outcome measures for SSc trials lTo evaluate the efficacy and safety of off-label treatments currently used to target the main disabling and life-threatening organ manifestations of SSc lTo contribute actively to the development and rapid dissemination of national, European, and international guidelines for the diagnosis and management of adult and juvenile SSc using the EUSTAR and PRES international network. Patients have a vital interest in improvement. Due to their severe disease and clinical symptoms such as fatigue and disability, they often have no voice listened by politicians, and SSc lacks adequate public attention. Due to the orphan character of the disease, the severe disability, the number of co-morbidities occurring in SSc such as malnutrition and depression, and the entailed prognosis, the number of patients organized is limited and therefore, awareness is at present not as high as needed. To make the guidelines and project results readily available for the patients, the project will include SSc patients and their representatives throughout the dissemination process, through the agency of FESCA aisbl.
The reason FESCA is one of the main partners of this project is that FESCA centralises patient participation, having regular involvement by patients and their relatives in many forms, including the biannual international World Scleroderma Congress and the EUSTAR training course. The role of FESCA in this project is also to encourage the active involvement of policy-makers and pharmaceutical companies. Working with doctors to secure earlier diagnosis and better treatments, FESCA will contribute to creating greater awareness of SSc so that those who suffer from it can access proper and equitable care. SSc being a prototypic disease for autoimmunity, vasculopathy, and fibrosis, patients with related diseases would also profit. DeSScipher results and guidelines will be translated into all national languages and will be disseminated to physicians and patients through organizing annual “National SSc Days� at key centres of the DeSScipher partners, with interactive presentations on selected topics of SSc, the DeSScipher guidelines, and the results of a continued observational analysis of the impact of these guidelines. Those results will also be presented at national patient conferences and other events held every year on the International Scleroderma Day (June 29th) by all FESCA member organisations. Furthermore, patient information brochures will be prepared and disseminated to hospitals, healthcare centres and general practitioners through the mailing lists of national medical societies. Working on the project submission to the European Union started at the end of 2011, and DeSScipher won this submission, receiving entire support from EU. The Grant Agreement was signed by all parties and thus DeSScipher became official and commenced on the December 1, 2012. The first official kick-off meeting as the start of the project, with all the 14 partners including FESCA aisbl and representatives of the EU, was heldon the 22nd January in Frankfurt, Germany. News about the DeSScipher Project and regular status updates will be released on the FESCA website and in its newsletter, and will be sent to all of its member associations during the upcoming three years. This means that all the member associations can translate the news, and make official press releases in their own countries as an essential part of establishing awareness of the disease and of the progress of the project DeSScipher.
morphoea Andrea Murray outlines recent Scleroderma Society funded research being carried out in Manchester
Tonia Moore and Graham Dinsdale at the back, Andrea Murray left and Jo Manning right at the front
research
Morphoea, or ‘localised scleroderma’, is a type of skin thickening that affects a localised area, sometimes with involvement of the underlying fat and muscle. Unlike systemic sclerosis (sometimes termed ‘scleroderma’), the internal organs are not involved in morphoea. Despite this, morphoea can be very debilitating because the areas of skin thickening (known as morphea plaques) can be extensive and are often disfiguring. Very little is understood about why morphoea plaques occur, and there is currently no effective treatment available. In order to learn more about morphoea, we carried out a study to image both the structure of the skin and blood flow within affected areas, comparing our findings with unaffected areas of skin. We used two imaging techniques to look at skin structure, and two to look at the blood flow of the skin. For structure, we used high frequency ultrasound (HFUS) optical coherence tomography (OCT). HFUS uses sound waves and OCT uses light waves to image and, together, this allowed us to measure boundaries within the skin. For blood flow we used thermal imaging (an example of a thermal image is shown in the figure below) which looks at the heat emitted by the skin, known to represent skin blood flow. We also used laser Doppler imaging (LDI) which uses laser light to give us information on the speed of red blood cells and, therefore, on blood flow within the skin blood vessels. Thirty-two patients were recruited into the study. One plaque from each patient was imaged. Blood flow and skin thickness within the plaque were measured using the techniques described above, alongside an area of unaffected skin. Using both HFUS and OCT, we found that the upper layers of the skin were thinner in the centre of the plaque as compared to the unaffected skin. We also found, using LDI and thermal imaging, that blood flow in the deeper, larger blood vessels but not in smaller vessels closer to the surface of the skin was increased in the centre of the plaque as compared to unaffected skin. We found a relationship between both deeper blood flow (as measured by thermography) and superficial blood flow (as measured by LDI) and the thickness of the upper layers of the skin. The study found that blood flow is increased and skin thickness is decreased within morphoea plaques, in keeping with previous findings. Our study, however, increased our understanding of this. We now know, for example, that it is deeper blood flow that is affected by morphoea and that the upper layers of the skin, rather than the deeper layers, are thinned. More research is required to fully understand how skin thinning and increased blood flow begins, how it progresses and how morphoea plaques may respond to treatment.
Picture: An example of a thermal image of a part of a morphoea plaque. The skin affected is to the left of the line and unaffected is to the right of the line. Red and white colours represent higher skin temperatures and yellow, green and blue show lower skin temperatures. Arrows show areas that were used for measurements of skin temperature.
New leaflets
The Scleroderma Society has been producing education and information leaflets since it was founded 30 years ago. We now have over 30 leaflets dealing with a wide range of scleroderma related topics. This year we are working towards the Information Standard which is a Department of Health supported initiative to ensure that materials giving health information meet a set of required standards both with regard to the clinical information that they give as well in terms of readability and accessibility. The next two years will see us redesigning and rewriting all of our publications to make sure that they conform with the standard and are as good as they can be. We have been fortunate to have had over 100 members volunteer to help evaluate and assess our new leaflets as they come on stream - many thanks for that. Our first new leaflet is, as you might expect, on systemic sclerosis, and it has been well recieved by both people with scleroderma and by medical professionals. We are currently planning our schedule with the following leaflets coming next: Scleroderma and the skin; localised scleroderma; morphea and the fingers. If you would like a copy of any of our leaflets either for yourself , someone else, or your doctor or medical professional please contact us at the office.
We recently attended Primary Care 2013 to launch a brand new leaflet aimed at Allied Health Professionals such as physiotherapists, occupational therapists and nurses. Many of these professionals see patients with scleroderma - although often very few, and we felt that it was important that they had a good idea of what scleroderma was and how they could support patients in their role. The leaflet “Scleroderma and your healthcare profession” proved to be very popular. If you feel that your physiotherapist, OT or nurse would benefit from one of these leafets, please let us know and we will send you some.
faSScinate
Professor Chris Denton to speak at Hampshire Group Meeting
A Systemic Sclerosis Research Study is starting recruitment. The faSScinate clinical study is evaluating the safety and efficacy of an investigational medication for patients with systemic sclerosis (sometimes called scleroderma).
On Friday 2nd of August at 4pm we are very fortunate to have Professor Denton attend our next Hampshire group meeting at QA Hospital. The plan is to have a question and answer session with Professor Denton. Clinical nurse Paula White will send written invites to all our members (family and friends of members are welcome too).
Clinical trial participants must: • Be aged 18 or older • Have a clinical diagnosis of systemic sclerosis • Have had systemic sclerosis for less than 5 years
Paula White has also in the process of organising a connective disease conference in September and will send information out as soon as she can.
Study participants will receive study related examinations, lab tests, and investigational medication. If you are interested in learning more about the study please visit the following link: http://apps.who.int/trialsearch/WA27788 to the World Health Organisation (WHO) international clinical trials registry platform or visit the link to clinicaltrials.gov on http://www.ClinicalTrials.gov To receive further information on the study please email: global.rochegenentechtrials@roche.com
It will be wonderful to see you all at each of the events above. Should you require any further information then please do not hesitate to contact me. I would like to take this opportunity of thanking Colin Beevor, Paula White, Julie Ingall and everyone at QA who works so hard to support us. Tracey James Tracey.james93@ntlworld.com
local contacts noticeboard for more information on you r local group activity go to our websi te cal wh lo r ere ou eac is h y loc al wa gro llo up Ho has Liz its If you own page. Please contact us group co-ordinator. if you on on would like to start a local gro need more informati up. the We will support you to do thi local groups contact s and @ liz it or can be very rewarding. We are local co-ordinator o.uk particularly in need of a loc sclerodermasociety.c al group in the North East of England.
Remember to sign up for this years Wear Your Gloves
We are looking to set up new groups in the North East, the North West and the South West. If you would be interested in forming a group please contact Liz Holloway. ROYAL FREE Group Next meeting 26th July 2pm - 4pm Contact Tani at the RF for more details
Bedfordshire Rita Boulton Tel: 01767 312544 ritaboulton@gmail.com
Hampshire Tracey James Tel: 02392 677476 tracey.james93@ntlworld.com
Scotland Frances Bain Tel: 0131 477 1122 iainandfrancesbain@msn.com
Buckinghamshire & Hertfordshire Marilyn York Tel: 077 0259 2387 andrewandmarilyn@btinternet.com
Lancashire and West Yorkshire Sheila Procter Tel: 01282 429004 sheila@sclerodermasociety.co.uk
South London Celia Bhinda Tel: 020 8698 6294 mcbhinda@btinternet.com
Derbyshire, South Yorkshire Staffordshire & Cheshire Diana Twigg Tel: 01298 24539 diana@sclerodermasociety.co.uk
Merseyside Helen Lingwood Tel: 0151 2801194 jonandhel@sky.com
South Wales Belinda Thomson Tel: 02920 625056 belinda@sclerodermasociety.co.uk
East Midlands Cheryl Darch Tel: 0116 2717180 cheryl@sclerodermasociety.co.uk
Surrey Nicky O’Shea Tel: 01483 764524 nicky.oshea@live.co.uk
East Anglia Jacky March Tel: 01394 286637 jacky@sclerodermasociety.co.uk
North Wales Kate A Owen East Sussex Tel: 01492 515834 Rosanna Cliton kate.owen09@btinternet.com Tel: 01424 426738 rosanna@sclerodermasociety.co.uk Oxfordshire & Berkshire Melanie Bowen East Yorkshire Tel: 01865 517033 Lynn Hind melanie@sclerodermasociety.co.uk Tel: 01482 354312 lynn.hind@hotmail.co.uk Mid Wales & Shropshire Jennifer Ames Essex, Kent and London Tel: 01544 267988 Amanda Thorpe ames60@talktalk.net Tel: 01702 344925 dollydumpling23@gmail.com
Become a Scleroderma Society Champion We are looking for scleroderma champions. Would you like to be one? It can involve as much or as little time as you can give. Sometimes it will be about writing a letter to your local news paper, at other times it might mean being available to take a call from someone with scleroderma in your county. We are hoping to build a small group of scleroderma champions who can help us spread the word throughout the country. We will provide you with direct support and it will be fun as well as fulfilling. Email us on info@sclerodermasociety.co.uk or call the office.
West Sussex Joint contacts: Jo Frowde & Lesley Dodd Jo: 01403 741445 or 07922274167 jo@sclerodermasociety.co.uk Lesley: 01903 753971 lesleydodd@hotmail.co.uk Wiltshire and North Hampshire Lynn Morton Tel: 01980 863444 lynnm@sclerodermasociety.co.uk
Contacts
Office The Scleroderma Society Bride House, 18-20 Bride Lane London, EC4Y 8EE 020 7000 1925 info@sclerodermasociety.co.uk Chief Executive Mike Rich 020 7000 1925 07977 487761 mike@sclerodermasociety.co.uk Executive Administrator Amy Barrick 020 7000 1925 amy@sclerodermasociety.co.uk
Trustees Kim Fligelstone (Co-Chair) 020 7229 4750 kim@sclerodermasociety.co.uk Richard Dodds (Co-Chair) richard@sclerodermasociety.co.uk Steve Holloway (Secretary) steve@sclerodermasociety.co.uk Susie Hoare susie@sclerodermasociety.co.uk Helena Rozga helena@sclerodermasociety.co.uk Events Calendar calendar@sclerodermasociety.co.uk
Helpline The Scleroderma Society operates a free helpline. We cannot offer medical advice, but if you feel you need someone to talk to we can be a good listening ear and will help in any way we can, 09:00-21:00 every day. Tel: 0800 311 2756. Calls are free from UK landlines. Nurse Specialist Advice Lines These nurse-led advice lines are for general inquiries only, not emergencies. If the nurse is unavailable, you may need to leave a message so he/ she can call you back. Liverpool Aintree University Hospital Jan Lamb 0151 525 5980 bleep 2231 Bath Royal National Hospital for Rheumatic Diseases Sue Brown 01225 428823 Belfast Ulster Hospital Audrey Hamilton 02890 561310 Dundee Ninewells Hospital Steve McSwiggan 01382 633957 Leeds Chapel Allerton Hospital Elizabeth Tyas 0113 3923035
London Royal Free Hospital Scleroderma queries: 020 783 02326 Pulmonary hypertension queries: 020 7472 6354 London Royal Brompton Hospital Respiratory (lung) queries: Lucy Pigram 07758 8943 175 Manchester Hope Hospital Liz Wragg and Catherine Lambe 0161 206 0192 Newcastle Freeman Hospital Scleroderma queries: Karen Walker 0191 223 1503 Pulmonary hypertension queries: Rachael Crackett / Julia De-Spyza 0191 213 7418 Portsmouth Queen Alexandra Hospital Paula White / Julie Ingold 02392 286935 Carer Support Group The Carers’ support group is run by Michael Thorpe. For more information, please contact Michael. 01702 344 925 michael@scleroderma.co.uk
Alongside the ISN Forums the Scleroderma Society have also set up a new and complementary community powered by HealthUnlocked. If you are a Facebook user you may already have seen advertisements for the forum pop up on your page. Why not give both the forums a go. Have a look round and meet and get support from many other people with scleroderma.
www.sclerodermasociety.org.uk info@sclerodermasociety.co.uk 020 7000 1925