February Newsletter 2015

Scleroderma News Spring Issue 2015 In this edition...

Self-Management Trails & Research Top-tips

The Scleroderma Society is a registered charity: 286736

In this Issue A round up of the treats we have in store for you, in this issue of Scleroderma News...

Pg. 2. Welcome Pg. 3 & 4 Society News & Annual Conference Pg. 5 & 6. Orphan drugs & clinical trials Pg. 7 & 12 Tips for living with scleroderma Pg. 13– 16. Fundraising & Volunteer Pg. 17 & 18 Research & NHS Changes Pg. 19 & 20 Doc Spot & Hello from Ireland Pg. 21 Local Support Group News & Competition Pg. 22 & 23 Contacts

Like our new Facebook page https://www.facebook.com/sclerodermasocietyuk Disclaimer: Views expressed in Scleroderma News are not necessarily those of the Scleroderma Society, nor the editorial Board of Scleroderma News. No responsibility or liability will be accepted, either for their contents or accuracy in Scleroderma News. Products, treatments and services on the website and in Scleroderma News are not necessarily recommended by the Scleroderma Society. The Scleroderma Society is not equipped to test and approve products, treatments and services available to the general public. Please exercise your own judgement about whether or not the item or service advertised is likely to help you personally and, where appropriate, take professional advice from your doctor, nurse, physiotherapist or occupational therapist before buying or trying something.

Scleroderma News: Welcome

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Hello again and welcome to the February edition of Scleroderma News I was reflecting and thinking about what I could write to bring you up-to-date for this piece.

Whilst on an early morning stroll today, the 9th of February, there were snowdrops in the woods and the sheep were grazing in the sunshine! This led me to think about how lovely the season changes are and how everything looks different.

That's it; everything is changed. To quote Heraclitus, the Greek philosopher, “Change is the only constant in life".

So I thought I should talk about change and how this has and will affect the Society:  Our website is changing in March 2015, to offer a more up-to-date style with integration to direct donations.  Volunteering and fundraising have changed as we have become more focussed and better equipped to help supporters.  The helpline is changing. It is growing and we are pleased to welcome Cini Bhanu, a doctor who will be volunteering to answer helpline calls with us from March.  Our small team of staff are changing - we welcome Jennifer Malone as Amy Barrick’s temporary replacement.  Conference plans have changed - working together with the RSA on a joint conference at a new location, Manchester.  Trustee meetings have changed, too - we schedule in time to work jointly with the RSA trustees on collaborative projects to enhance services, avoid duplication and give us a bigger voice.

The two organisations have worked together well over the past eighteen months and are thinking about how it might be possible to develop collaborative working into something that is even more productive and permanent.

Looking at our aims of providing better services for those affected by scleroderma, increasing the scope for research, support, and offering a greater voice for people with scleroderma, we are investigating the feasibility of a merging of the two organisations. You will find an accompanying letter from Professor Dame Carol Black, Professor Chris Denton and Professor Ariane Herrick, discussing their aspirations for the future to this effect.

The first stage of our activity during this process of merger considerations is to look at “due diligence”; according to the Charity Commission, due diligence is a set of steps organisations take to assure themselves that a merger is in their best interests; it involves the examination of both of our charities in commercial, financial and legal respects. It provides the trustees of each charity with full knowledge of the organisation they seek to merge with and enables the trustees to make informed decisions about the best interests of their organisations for the future. Whilst the due diligence activity is being undertaken, it is business as usual for the RSA and ourselves, as we focus on delivering the Society’s strategic priorities for 2015 and supporting our beneficiaries.

We do like to hear your thoughts and encourage your input. As I remember saying at last year’s conference, we welcome receiving your views.

Best wishes Helena

New Staff Member –Jennifer Malone I am delighted to be joining the team at the Scleroderma Society, having been offered a temporary contract.

Most of my experience has been in the charitable sector, latterly in a finance and administrative role with Home-Start Harrow (a charity working with young families) and with Age UK Brent.

On a personal level I have nearly 25 years of experience in supporting a family member with a life-long disabling condition and so have a great deal of empathy with the goals of the Scleroderma Society.

I am keenly looking forward to bringing my energy and commitment to this role and to making a positive contribution to the Society's work.

Scleroderma News: Welcome

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Society News Giving with Confidence We know that when you make a donation you will want to know that the organisation is committed to best practice.

That we are responsible in the way we fundraise, dedicated to honesty and accountability.

That is why the Scleroderma Society has signed up to fundraising regulation through the Our Fundraising Promise to Fundraising Standards Board (FRSB). Membership is a commitment to the highest standards You of fundraising. Should there be any concerns about the way we fundraise, we have a robust We are committed to high complaints process in place, notifying the FRSB of any issues raised. It is about being standards We are honest and open accountable for our actions and ensuring that we put you - our supporters – first.

We hope that our membership will give you added confidence in our fundraising and, We are clear We are respectful ultimately, encourage more people to provide vital support to individuals diagnosed with We are fair and reasonable scleroderma.

We are accountable Website The Scleroderma Society are delighted to announce the launch of our new website. You can view our new website be visiting: www.sclerodermauk.org.

useful. Thank you to Dr John Pauling (RNHRD) and Consultant Rheumatologist Will Gregory for assisting in this update of this booklet, along with any members who allowed us to photograph them. The booklet is currently being designed, however we can send you a draft printed copy—just The new website has been designed with you in mind and we call or email the office to let us know you would like one. would like to thank our user testing group, which was made up of 23 members and supporters, for providing feedback.

Newly Diagnosed Kit We hope that you find the website easy to use and enjoy We understand that receiving a diagnosis of scleroderma can discovering more about scleroderma and the charity. be hard. You probably haven’t heard of scleroderma before or know anyone else with the same diagnosis Digital Ulcers and may have lots of questions.

We are extremely pleased to announce that NHS England have completed a policy on the commissioning Sildenafil and To support medical professionals and newly Bosentan for the treatment of digital ulceration in systemic diagnosed individuals we have recently sclerosis: published our Newly Diagnosed Kit. This kit https://www.engage.england.nhs.uk/consultation/specialised- explains scleroderma, has FAQ’s answered services-policies/user_uploads/bosntn-sildnfl-syst-sclerosis- and details the support the Scleroderma pol.pdf Society can offer.

Thank you to our members and clinicians who have been involved in this development!

We would like to thank everyone who participated in our The document is now open to public consultation and The survey monkey “When You Were Newly Diagnosed” and Society will be responding. We would like to invite you to Nicola Whitehill for telling her scleroderma story. Your submit any comments via the charity, thereby allowing us to support has allowed us to create a valuable resource for submit one response from the scleroderma community. newly diagnosed individuals. Please visit the website to The following questions are asked as part of the consultation: download your kit.

1. In your view, what would be the effect of the proposed Rare disease day changes on the service?

2. What further changes, if any, do you think need to be This year we were also a part of the rare disease day campaign, kicking off with a feature in the Independent on the made to this document?

28th February. If you missed the article you can view it on our 3. Are there any other considerations not reflected in the website and social media. document that you wish to draw our attention to?

Eurodis would like you to RAISE AND JOIN HANDS to show If you have any comments, please email them to Chloe your solidarity for rare disease patients around the world! th

by Monday 20 April. If you would like a hard copy of the Help us show we are all mobilised for policy please call or email the o f f i c e people living with rare diseases and chloe@sclerodermasociety.co.uk driven by the same objectives by taking a Oral & dental aspects of systemic sclerosis Jointly with the RSA we have recently updated our publication on oral and dental aspects of systemic sclerosis, which includes a selection of facial exercises that you may also find

Scleroderma News: Society News

photo of you and your friends raising your hands.

Upload your photo here: http://www.rarediseaseday.org/join-yourhands/

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We are delighted to announce that this year we will be holding it jointly with the RSA on Saturday 20th June in Manchester We are excited to reveal that the theme is treatments and topics include a look in to skin and ulcers, the gastrointestinal tract and nutrition, pulmonary hypertension and breathlessness, and highlights of laboratory research and emerging therapies.

Speakers include leading UK scleroderma and Raynaud specialists.

We will also be joined by a variety of exhibitors, which will provide plenty of opportunities for you to get involved. We hope the day will provide an even greater opportunity for you to meet more people who share similar symptoms with you, and for you to share and exchange tips. There will also be the opportunity to put your questions to the speakers.

This event is free to members of the Scleroderma Society and RSA. Barnes Wallis Building, the University of Manchester, Altrincham St, Manchester, M1 7JR We would also like to invite you to join us for a gala dinner following the conference. The dinner will include awards in the following categories: volunteer of the year; local support group of the year; fundraiser of the year; supporter of the year; supplier of the year; and WSD art competition winner. The dinner will be held at the MacDonald hotel (0.1miles from the conference venue). Tickets are priced at £40 per person.

To view the draft programme and book a place to attend please visit our website or call the office—020 7000 1925 Our AGM will also be held on the 20th June 09:00-09:45. To attend please also visit the website or call the office. A formal letter will be sent out with your next newsletter.

We look forward to seeing you there!

Painting by Aspa Palamidas, 2014 art competition winner

World Scleroderma Day 2015 ‘Unveiling Scleroderma’ 29th June is World Scleroderma Day (WSD), and this Art Competition year the theme is ‘unveiling scleroderma’.

We will also be holding an art competition this year following last The Scleroderma Society is partnering with FESCA to launch a year’s success. This year we would like worldwide campaign for WSD. your art work to reflect the WSD 2015 theme. You can be as creative as you like!

On the 29th June we will be launching our 25 video campaign and we want you to help unveil scleroderma to the world by Send your photograph, painting, drawing or even sculptures recording a 25 second video and sharing on social media. To to: Art Competition, The Scleroderma Society, First Floor help spread the word we will be holding recording days at our Bride House, 18-20 Bride Lane, London EC4Y 8EE. office and at the Royal Free Hospital.

Deadline: Wednesday 20th May. You can also find out a bit On the 30th June we will be heading to European Parliament more about the competition on our website. to unveil scleroderma to MEP’s. MEP’s will hear from patients, specialists and patient organisations and we hope to challenge We are pleased to inform you that Alan Brain, contemporary equity for rare diseases. artist and teacher, and Society member, will be judging the competition again. The winner will be announced at the We will also have a range of UK based events for all of our conference gala dinner. Alan will also be holding an exhibition supporters to unveil scleroderma. at his gallery in July, and donating some of the funds to the Society. We are extremely pleased to have Alan on board.

More information will be released soon, just keep checking Search ‘Alan Brain Art’ to see his work. our website for updates.

Scleroderma News: Society News

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Orphan Dru What are orphan diseases?      

In Europe an orphan, or rare disease, is classified as a condition that affects less than 1 in 2,000 Scleroderma is an orphan disease as it affects around 1 in 10,000 people in the UK There are 6,000-7,000 recognised orphan diseases - 80% of these are genetic and 90% are chronic (persistent and long-term) 1 in 17 people will be affected by a rare disease at some point in their life. This amounts to approximately 3.5 million people in the UK. The amount of people touched by the disease will be greater, for instance family and friends Due to DNA sequencing we now know of ¼ more rare diseases and are also able to identify these at early stages Annually pharmaceuticals spend between 3-4% on developing orphan drugs and there is effective treatment available for 1%

More treatment options are required for rare diseases as medicines play a crucial role in maintaining health, preventing illness, managing chronic conditions and ultimately curing disease. Collectively rare diseases are not rare!

Patient involvement with the European Medicines Agency

By Nicola Whitehill In the autumn of 1997, I was diagnosed with diffuse systemic sclerosis (ssc) and Raynaud’s, aged 24, and given a 15month prognosis.

Now, at the age of 41, having stabilised my initially very aggressive disease to a manageable level, I feel well enough, to use some of my experience to get involved in any way that I can, to help in the understanding of this rare, life threatening diagnosis.

In the summer of 2014 I attended my first meeting as a patient expert with the European Medicines Agency (EMA). This was a very exciting day for me, as not only was it the hottest day of the year (thank goodness that I didn’t have to wear my Eskimo outfit, and I was back in my suit), the offices are located at Canary Wharf overlooking the River Thames. And so, it was quite an adventure to just get there, with me living in the seaside town of Southport, on the North-West coast. However, the location more than compensated for my physical efforts that day. Entry into the building as well as to the meeting room, I can only liken to something similar to that which I have seen in a James Bond movie. My fellow colleagues made me feel very welcome and at all times, they Scleroderma News: Orphan drugs & trials

ensured that I was included in the the establishment and development of a discussion, often asking me for my view. policy on orphan medicinal products in the EU, and assists the Commission in The EMA is a decentralised agency of the drawing up detailed guidelines and liaising European Union, responsible for the internationally on orphan medicinal scientific evaluation of medicines products. developed by pharmaceutical companies for use in the European Union (EU). Its So far I have been involved with several aim is to build the best possible COMP meetings- the majority of which, I regulatory system for medicines for have been able to participate in, without Europe and protect the health of its having the physical exertion of going to citizens. These activities aim to foster the London. All of the relevant paperwork timely exchange of regulatory and and documents are forwarded via a scientific expertise, and development of secure electronic system network (James best practices in the regulatory field Bond style, again), and the meetings can across the world. be accessed by teleconference. This participation arrangement has worked The EMA’s main responsibility is the perfectly for me, as my energy, pain and protection and promotion of public and stamina levels are easily exhausted. This animal health, though the evaluation and arrangement allows me to be respectful supervision of medicines for human and of my symptoms whilst being able to veterinary use. provide an active contribution.

Most of the EMA’s scientific evaluation work is carried out by its scientific committees. These committees have various tasks related to the development, assessment and supervision of medicines in the EU. There are 7 committees, and I am a member of The Committee for Orphan Medicinal Products (COMP). This is the committee responsible for reviewing applications from people or companies seeking 'orphan-medicinal-product designation'. It is for medicines to be developed for the diagnosis, prevention or treatment of rare diseases that are life -threatening or very serious.

I have found the content of the meetings to be most stimulating, and I am ex t re me ly hopeful for the future for newly diagnosed ssc patients. I take huge comfort and encouragement in seeing for myself that the pharmaceutical companies, as well as the leading world ssc experts, are relentless with their research for improving the medicines and treatments for this disease, with the ultimate accolade being to discover the, The COMP is also responsible for so far, unknown, cure. advising the European Commission on www.ema.europa.eu/ema/ Page 5

ugs and Trials Chloe attended the WODC to meet key leaders in orphan condition possess the opportunity to advance research in their condition area. Patient charities can enable access to drug development. patients and encourage recruitment.

“Orphan diseases are often referred to as an ‘invisible disease’ and Power of the patient voice management of them is often referred to as ‘mission impossible” (speaker at the Congress).

At a national level the voice of the patient is increasingly being heard. There is an evolving understanding that care and Take home message—collaboration is key support should be tailored to the patient. Achievement of this There is a huge medical unmet need to orphan drug involves collaborative work with the health care provider to development – good treatment and choice is limited, and allow them to understand what is important to the patient. expertise lie sparsely across the globe. Through collaboration Take advantage of your evolving power. we can develop orphan drugs despite these constraints.

Knowledge is power What can we do?

Patients who understand their condition are better able to Identify the unmet need manage it. In turn this minimises potential impacts of the Patient organisations bring individuals with a rare disease condition, i.e. an overnight stay in hospital. Patient together. They provide a unique insight in to the impact of the organisations are able to offer a wealth of information and condition and can demonstrate priority areas of treatment assist in identifying ways to self-manage a condition An need. This can be identified and used to encourage specific informed patient is a powerful patient. areas of research.

To find out more about the congress and to identify ways to get involved visit the ‘Helping You’ section of the website.

Involvement in clinical trials As orphan diseases have low prevalence rates recruitment for Our role is to make scleroderma visible and the mission clinical trials can pose a challenge. Individuals with the possible.

Being part of a research project may mean you get better treatment at the There are lots of reasons to be involved: same time as helping other people with  you might receive a better your condition. treatment;  your condition might be monitored Who does research into more closely; scleroderma?  you and your doctor might learn more about your condition and Hospitals, universities, drug companies thereby tailor treatment more and charities are involved in research appropriately; and scleroderma clinical trials.  you might be helping to develop The research aims to: better ways to look after other  improve our understanding of people who have the same type of scleroderma; scleroderma as you; and  you might be helping to improve the  come up with new preventions, health of society more widely; treatments an d cures for scleroderma; and What are the trials?  improve people’s quality of life with There are 3 main types that you might scleroderma. take part in: Why should I take part in trials?

Observational—no new treatments, Through research studies academics can solely the observation of your current identify if a new prevention, treatment state. or cure is safe and beneficial. This Interventional—involve receiving a new evidence is gathered by carrying out treatment, for example physiotherapy or research studies that involve patients. new drug, to assess the impacts.

Scleroderma News: Orphan drugs & trials

Qualitative—understand more about people’s experience of living with a condition. This type of research involves being asked questions or having an open discussion.

Every drug that is currently prescribed for scleroderma will have been through research studies to make sure that it works and is safe to use.

How do I know if the study is good?

Research carried out in the NHS must follow guidelines set out by the Research Governance Framework for Health and Social Care.

How can I take part in trials?

If you are interested in taking part in a study it is best to discuss this with your healthcare professional or visit our website to find out more.

Please note the Scleroderma Society cannot help you to join a specific trial or study.

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Useful tips for livin Useful equipment and tips by tongue-and-groove plier, but friends are impressed - and I feel A n n e l i s e R o e n n o w , D a n i s h very independent.

Raynaud’s and Scleroderma Also, tools with a good grip are a must. Forks, spoons, Association Who am I? I am 41 years old, and I work as a librarian at the public libraries in Denmark.

I was diagnosed with systemic sclerosis in 2007, but had symptoms many years before.

I have been forced to rethink everything to have the best quality in life with the limits scleroderma gives me. Living alone I have to deal with everything myself. The challenges are cleaning the house, cooking, transport, grocery shopping, getting dressed, taking showers, managing my work….everything.

At this point I need to express a small disclaimer! Most of these tips are a mixture of my own experiences and those of other people with scleroderma. In order to get as many ideas as possible, I asked other fellow scleroderma people I know on Facebook the following questions: Do you use special equipment to help you in daily life?

What helps you in the kitchen, bathroom, grocery shopping, and so on?

knives, pizza slicer, cheese slicer, measuring cups that can stand on the table and you still see how much you have put in them that you don't need to lift it up . Not to mention the electric help you can get – electric devices such as can openers, lid openers, choppers, stirring-in pots and so on.

Bedroom This is where you spend many hours, sometimes also days when things are bad. So it is important that you have a good mattress and pillow. Maybe more pillows to change during the night - this is a trick my nephew taught me. He was right!

You need to take action in order not to wake up during the night with reflux. Raise the head of the bed by 4 to 6 inches, so you can sleep with your head and chest elevated.

You can lift the top end of the bed by sticking blocks or phone books underneath -- although your other half may object once he or she has slid out of the bed a few times! You could also lie on special wedge pillows designed to help you sleep with your head and upper body elevated. Some people invest in an electric bed where you can raise the head of the bed.

The answers were many and I have tried to gather most of them here, so that article will be a mixture of my own experiences and of those from other people with scleroderma. What is useful for one person might not be useful for another as we have different symptoms, and are Use an electric heated blanket so that you don't have to use energy to warm your affected in different ways. bed. Switch it on 30 minutes before If you are unsure of anything you read here, please check with bedtime and enjoy the warm bed. Also your doctor to make sure it is suitable for you to try, and make sure that your feet are warm - it can take hours to fall asleep if your feet are above all, please use your common sense! cold. You might want to use socks to help you. If you are away you can use hot water Let’s look at what help we can get for the kitchen. in a bottle to warm up the bed.

One of the best things I have in my kitchen is the dishwasher.

Putting your hands in hot water while doing the dishes can be Bathroom very painful so I put everything in the dishwasher and I have made sure that almost everything I have in my kitchen can be In the bathroom you make minor changes to fit your needs. If you have a bathtub it might be difficult to get in and out, if you put in the dishwasher. have loss of strength. Consider changing it to a shower If you need to wash by hand, a sponge gives you a better grip instead, and install grab bars near the bathtub/shower - also than a brush. Or find a brush with good grip, as at least with next to the toilet, a grab-bar can be useful or you might consider an elevated toilet seat. Is it hard to stand up in the brushes you can keep your hands out of the water. shower? Then have a shower chair to sit on. And also make sure that you have a non-slip surface, so that you don't fall.

Most of us lose strength in our fingers / hands. Living alone means that there is no strong man (in my case!) to open cans, bottles and lids. I have a multi-grip tool and a nut cracker which can open any bottles, I guarantee! It looks funny when I pull out the Scleroderma News: Top Tips

Just as in the kitchen and bedroom, electric devices in the bathroom can also be considered - use an electric toothbrush, the head on the toothbrush is also a bit smaller so it is easier to brush your teeth. Don't forget to floss your teeth. As it is painful when wrapping the floss around your fingers, Page 7

ng with scleroderma it can be difficult to get your fingers in, use dental floss that you can buy on small sticks.

For laundry, use a tall laundry basket on wheels, which is easy to roll to the washing machine when full.

trolley? And in the shops, have a pair of thin gloves with you. It is cold for fingers in grocery stores - you might want to wear those when getting that chicken out of the freezer.

Getting dressed

And more‌

There are different types of dressing aids available - you can use dressing sticks to help you with getting socks and shoes on, to handle buttons and even zippers in jeans or jackets. One piece of good advice I learnt at the last world congress which I adopted was to turn you socks inside out to put them on.

Gardening - raised garden beds, different sizes of gripping devices.

Putting make-up on is a part of getting dressed and going out into the world. The red dots on our skin that we can have can be covered with a good foundation.

Use a pen to open the pill packs, or a 'poppet pill remover'.

Going out So you finished the morning shower, you got dressed and you are ready to go out into the world. How is the weather out there? What does the weather forecast say? This is what I do: I dress like I am walking at the North Pole.

Dressed in many layers - the more layers, the warmer you are. Cover your face and most importantly, wear a hat and a hood to stop the wind. I also have a neck tube called a “Buff� - it stops the wind, literally. You can put it around you face, neck or under a hat. I use wind-stopper mittens as the fingers help each other to keep warm. Sometimes I use thin gloves inside the mittens. If you are still cold - you can get electric heated gloves, insoles and, jackets. Or use hand warmers - they come in different types. Some have this metal coin in it and when you break it the liquid goes warm.

Then you take them home, boil them in water, and can use them again.

Cleaning house - robot vacuum cleaner.

Rest during the day and use an egg timer to remind you!

Have a good chair (in the kitchen, and the living-room). Also use a fitness ball as a chair!

Use a stylus pen on tablets and smartphones.

Having scleroderma with all the limitations it gives you does not mean that you cannot continue with your life.

Look at your routine - is there something you can change?

Be an inventor and find whatever helps you, so that you can be as independent as possible.

Shopping I live alone. I don't have a car, so my challenges when it comes to grocery shopping are that I need to carry everything home. The best thing is to carry your items on your back and your hands and arms are then free. But if you need to carry your groceries, carry the shopping bags on your arms, not your fingers. You might not have the strength to carry it in your fingers, and you cut off the blood supply to the fingers and this can cause a Raynaud's attack. Also the thin handles can be painful so use plastic carrier bag holders if available. Why not use a shopping

If you have any top-tips that you would like to share please email them to info@sclerodermasociety.co.uk or post them to our Facebook wall.

Scleroderma News: Top Tips

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Useful tips for managing your symptoms Following on from our report published in our April edition of Scleroderma News on the Rome World Congress on Systemic Sclerosis, we now continue with the report and what we learned at the Congress.

Caring for your ulcers

Dealing with fatigue

Barbara Gemmell is the Scleroderma Clinical Nurse Specialist at St. Vincent's Hospital, Melbourne, Australia and gave a long and detailed presentation on taking care of ulcers.

Dr Janet Poole of the University of New Mexico spoke to us about the management of fatigue in scleroderma.

She started by explaining that fatigue is where you have physical tiredness and lack of energy, which is separate from sadness or weakness.

Barbara explained how initially the skin becomes waterlogged as the body attacks itself. The skin tightens and stretches. The normal drainage system (lymph) is slowed, scar tissue is laid down and swelling occurs from the accumulated fluids, damage to small blood vessels results in leakage of blood and poorer circulation. This process can cause the skin to split resulting in painful lesions (damage in the skin).

Barbara described how the waterlogged skin gradually drains leaving behind thickened skin, which is usually itchy throughout the changes. There is further skin tightness, and the skin becomes stuck to underlying fatty tissues. Sweat glands and hair follicles are choked by the extra collagen in the skin, so it becomes dry. After several years, the thick skin softens and either returns to normal, or becomes thinner, but may still be stuck to underlying fat; this is called tethering and may cause deformity. In addition, bumps and scrapes often happen from bent and less mobile joints.

In her clinic, they look to a whole body healing approach.

Give your body the building blockers to repair damaged skin:    

a well-balanced diet plenty of protein a multivitamin to supplement zinc & magnesium used for healing have enough rest and sleep

Avoid irritating the wound area and use protective dressings, gloves, possibly rethinking how you do something that causes repeated trauma to the wound.

Changes in the skin vary throughout the course of the disease with scleroderma. You should keep wounds moist but not wet, and provide protection from irritation.

Permanent changes to the small blood vessels in fingers make them more susceptible to injury, and slower to heal.

Use simple creams to provide flexibility in the skin which may help prevent some cracking. Find dressings that suit your wound and where it is on your body. Ulcers can occur anywhere on the body where irritation occurs and wound healing in scleroderma may take longer than “normal”. If your wound is red or swollen, has an unpleasant odour, or increased pain, then it may be infected and you must seek help for this from your medical team.

Scleroderma News: Top Tips

She gave a quote from a scleroderma patient who described their fatigue as follows: “…Just extreme fatigue where by the end of the day at work, I came home and I laid on the couch and then I went to bed. I didn’t eat, I felt like I was in a coma, I just...I couldn’t move. Just extreme fatigue. And not the kind of fatigue that a weekend of rest and, you know, just laying around would cure”.

Dr Poole explained energy like a bank account. She explained we should imagine that “energy” is like money in the bank. You spend energy doing various things: you lower your balance by withdrawing from your account. You can bring in energy from different sources and deposit it, raising your balance or energy. You can borrow or transfer it from one energy bank to another. If balance (energy) is low, you may have to budget to have enough to do what you enjoy. If you continue to spend energy and do not replace it, you could create a deficit. Fatigue could be thought of as energy “poverty”.

We can “deposit” energy into our energy account by:  Good nutrition  Establishing good sleep habits  Staying as active as possible  Resting  Spending less energy: use good body mechanics; organise work stations; use technology; simplify activities.

Dr. Poole suggested some rules for rest to be very useful:  Rest before becoming fatigued  Take short, frequent rests  Alternate work and rest – plan rest times first  Try different types of rest: lie down, sit, sleep, do a passive or quiet activity i.e. listen to music.

When it comes to organising your work station, some things that you can think about include storing items where they are easy to reach (i.e. between your knees and chest).

Put things that you use a lot into places that are easy to reach. Get rid of clutter! Keep things in the place where they are used (one set of bathroom cleaning products in each bathroom, for example, so you don’t have to move them around.)

Try to simplify activities and eliminate steps from it if you can. For example, serve meals in the kitchen to eliminate Page 9

having to carry food into another room. Change the activity if you can, for example by buying pre-chopped vegetables.

Change where your crockery is stored so that it is easily accessible. Consider sometimes using plastic or paper dishes that can be discarded.

Prioritise and think about what tasks have to be done, what others expect you to do, what tasks you can eliminate, and what you can delegate to others!

In conclusion, the following slide from Dr Poole’s presentation sums up the “energy bank account” ideas!

Budget your energy when levels are low and plan how to use your energy. Make schedules, be organised and prioritise. One example that Dr Poole gave from a patient was, “when my daughter wants to do something for fun, we do something where I can sit down and we make a deal that the next day, we stay at home”.

Give yourself a break and consider what is important: does it really matter that the towels are folded, sheets ironed, or dishwasher packed perfectly?

Tips for Carers and Family Members We also heard from Robyn Sims, President of Scleroderma Australia, on the role of the carer and family members. Robyn explained that to be a good carer you need to make sure you look after yourself. She suggested you may like to seek help from a professional to deal with the emotional stresses a diagnosis may cause: anger, hurt, guilt and frustration, powerless. Draw on your family and friends and together learn about the condition.

through government sources and depending on the amount of care needed you may seek help with wound dressings and things like showering.

Communicate—The person you care for needs to be able to express what they need and require space. Listen! Let them do as much as they can and step back when the time is right.

Hovering over your partner can cause anxiety and stress for you both. Humour can be a wonderful tonic! Not easy, but Be aware of your own mental health—Mental health is just over time this will be a great source of relief for you both. as important as physical health. Don’t give up all the things you like to do and become a martyr (and maybe resentful). Getting support for yourself—At times caring can seem to Educate yourself as much as possible through seminars and be overwhelming. Find a carer’s support group. This can be attending medical appointments. Often the carer may be the catching up for a coffee or even chatting over the phone.

Learn to prioritise things that you have to do so you don’t be last person to realise they are in danger of collapsing! overwhelmed with “to dos” and you take a step back.

Take care of your own physical health—When you are a Think: a. Is it necessary? carer, it is vital that you keep yourself strong and healthy as b. Can it wait a bit? best you can and look after your own health so you can be at c. Can it wait a good while? your best to help your loved one/family member you are caring for. Make sure to eat regular and healthy meals. Do some exercise such as walking, swimming, or gardening. Become involved—Being involved with an organisation for Maybe try some meditation or yoga or meditation. Keep up those with scleroderma can help with your feeling of your own health care and do not neglect your own medical powerlessness. Being at the cutting edge of information on needs. Very importantly, get plenty of rest as exhaustion can new treatments etc. can be very beneficial and you will meet lots of people with lots of experience and willingness to share affect your health. and discuss.

Tap into services—Many countries have excellent support for carers such as home help: meal services, shopping, home You can find out a bit more about carer support on the maintenance and cleaning. You may be able to arrange respite Scleroderma Society website.

Scleroderma News: Top Tips

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...even more tips!

Gastrointestinal problems that people with scleroderma may experience One excellent lecture we heard was from Dr Janet Pope of the University of Western Ontario about gastrointestinal (GI) problems that people with scleroderma may experience. Dr Pope started by giving an overview of some of the problems that can occur. She explained that scleroderma can affect any area of the GI tract: mouth, oesophagus (swallowing tube), stomach, small bowel, large bowel, rectum, and anus.

Dr Pope explained that problems occurring in the mouth extend from having a dry mouth, Sjogren’s syndrome and acid reflux, which can give bad breath and damage teeth as well as poor quality of saliva which can worsen swallowing.

These problems contribute to poor dental hygiene.

Then Dr Pope discussed treatments for these problems.

Principally, you should try to avoid reflux, make sure to have frequent dental care, and consider taking a pro-motility medicine to speed up digestion and help move food through the stomach quicker and reduce reflux.

We learnt that around 90% of people with systemic scleroderma have GI involvement. In regard to intestinal scleroderma, symptoms include diarrhoea, gas, bloating, weight-loss and poor appetite. In cases of severe gut disease, there can be pseudo-obstruction, severe constipation, risk of twisted bowel, perforation, malabsorption and malnutrition. Treatments for severe gut disease may be: prokinetics, Loperamide, nutrition, probiotics, GI rest: nutrition by other routes.

Esophageal scleroderma occurs in 80-90% of patients though it is not always symptomatic. It involves:  Dysmotility, which is uncoordinated contractions and lack of peristalsis (muscle movements)  Gastro-oesophageal reflux: low pressure of the sphincter at the bottom of the oesophagus, and poor acid clearance. This gives rise to esophagitis and complications. The treatment is proton pump inhibitiors to suppress acid production in the stomach as well as H2 blockers and prokinetics to increase the action of the stomach and help faster digestion. It is also important to raise the head of bed to reduce acid reflux at night, and to avoid triggers in diet for reflux.

For problems such as bloating, feeling full early, intermittent diarrhoea, the doctors may consider if there is too much bacteria growing in the bowel. They may give antibiotics intermittently, or on a rotating schedule, or constantly, to try and treat this. They may also give drugs to increase bowel contraction: some antibiotics also improve bowel contractions (erythromycin). Drugs for irritable bowel may Scleroderma News: Top Tips

help as they improve bowel motility.

Gastric scleroderma includes watermelon stomach i.e. vascular lesions (GAVE) which may need endoscopic repeated laser. The person often has anaemia, delayed stomach emptying and this contributes to reflux. Some people are partially responsive to pro-motility agents.

Dr Pope also discussed faecal Incontinence in systemic scleroderma and explained how pelvic floor exercises can be helpful. She also explained that it is a good idea to try to regulate the bowels to avoid both diarrhoea and constipation.

Sacral nerve stimulation may be considered as a treatment for some people. In patients with a weak but structurally intact sphincter, it may be possible to alter sphincter and the bowel behaviour using the surrounding nerves and muscles.

Sacral nerve stimulation is a treatment option for these patients. It involves low-level electrical stimulation applied via electrodes through the sacral foramina to the sacral nerve supply of the lower bowel and sphincters.

In conclusion, GI involvement occurs in nearly all patients with scleroderma. The most common symptoms are acid reflux, problems swallowing and slow emptying of the stomach. Many symptoms such as heartburn, food sticking, feeling full early, bloating and diarrhoea are treatable.

Below are some ways to help yourself with your GI problems, provided by lots of different people with different types of GI problems, that explains what adaptations and changes to their lives they have made to help themselves.

For mouth dryness: Avoid spicy food and mint, avoid dry food, try sugar-free chewing and lozenges and see your dentist frequently.

For dry mouth, small mouth and swallowing problems: Soft food broken down, add a sauce, take small bites, eat slowly and relax, drink water, and eat with spoon and small cutlery with rubber handles.

For slow digestion, some people find it helpful to avoid: Raw vegetables, leafy vegetables, salads, acidic fruits, cheeses, tomatoes, meat, rice, bread and pasta.

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Things that people with slow digestion find helpful include: Low-fat yoghurt, soya milk, pureed fruit, soups, soft fruits e.g. raisins, vegetables cooked until soft, fish, tofu, eggs, white meat instead of red meat.

Tips on bowel incontinence: Wear protective pads and carry spare pads, underwear, wipes and disposable underwear. Find out in advance where the toilets are. Carry a card explaining you have a medical condition requiring urgent access to a toilet.

For controlling reflux: Don’t let your stomach get too full, eat frequent small meals.

Avoid fatty, spicy and rich foods. Avoid caffeine, do not eat or drink late at night, nor lie on your right side at night.

Elevate the bed.

Not all of the above tips will be helpful or useful to everyone—what works well for one person may not work at all for someone else. But hopefully they provide some ideas and things you might like to think about trying to see if they bring you any benefit. Please consult your healthcare team with any concerns about any of these suggestions.

For constipation, top tips: Drink lots of water! Try more, or less, fruit and vegetables.

Find a time to sit on the toilet at same time every day. Avoid processed foods, and avoid using laxative and antidiarrhoeals.

Tips for Having Blood Tests Having scleroderma you may be asked to have frequent blood tests. This procedure can be stressful and difficult for you and the collector (also called a Phlebotomist) due to various symptoms. It could be that veins “disappear” or are hard to find, or that blood flows slowly, that your skin is hard and thick, or that you become anxious or panic.

As a patient there are things you can do to help: Learning to relax can reduce the difficulty of the collection. Stress and anxiety can increase your blood pressure and constrict blood vessels. Relaxing will reduce the chance of adrenaline-like hormones shrinking the peripheral veins in the hands and feet.

If blood draws are painful, ask for the use of some local anaesthetic (EMLA cream) 1 – 2 hours before the draw.

You can also consider self-administering an over the counter local anaesthetic preparation such as 1% lignocaine.

This can be useful; however, be sure to tell the collector when and where the preparation was used.

If you are dehydrated the blood will be thicker and your blood pressure may be reduced. Drinking additional fluids in the hour or two before blood is to be taken often helps. In cold weather hot drinks can warm the inner body and aid blood flow.

Plan your appointment and arrive early if possible. Finding car parks and locating the clinic can be stressful and this will give you the chance to calm yourself and have that extra drink!

If you are cold, there will be less blood flows to the extremities (arms and legs) making it difficult to find a suitable vein.

Dressing warmly and keeping your skin temperature a bit higher than usual will help. Take in your own heat pack and apply to the area before and during the process. Offer it to the person taking blood – it will dilate veins and make them easier to feel and see.

Good communication—this is the key to effective interactions. Some simple and effective strategies when taking blood will improve the experience.

Express your concerns Explain that you have scleroderma as well as any past collection problems and successful solutions that have been undertaken. This includes expressing a preference for collection areas on the body. In some cases one side of the body may be easier to access than the other.

Suggesting which arm/vein works best is good; however, the Phlebotomist taking the blood needs to feel or see the vessel.

They also need to be confident that they can gain access to the vein to draw blood. All collectors have their preferred methods.

Be your own advocate and if someone has had 3 attempts at getting your blood – ask if someone else could possibly try instead. Also consider coming back later in the day or another day – when you are more hydrated, less stressed etc.

Clinicians as a rule are advised not to have more than 3 attempts at taking blood – unless it is a life or death situation. This is a reasonable request.

Booking for a specific Phlebotomist that you are comfortable with is also a good strategy.

Thank you to Scleroderma Australia for providing these tips.

Scleroderma News: Top Tips

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On Sunday 2nd August thousands of cyclists will once again be taking to the closed streets of London and Surrey to complete a 100-mile cycling challenge.

The Prudential Ride London – Surrey 100 starts in Queen Elizabeth Olympic Park with a route winding through the city and into the stunning Surrey countryside. Cyclists will finish at The Mall in central London, shortly before 150 professional cyclists race in the Prudential Ride London – Surrey Classic.

Victoria Bale said “Riding around the sights of London with no cars and riding up the Mall with loads of cheering spectators was amazing. Perhaps the most memorable part of yesterday was the display of British spirit, not just the riders but the marshals and spectators turning out in those vile conditions”.

Unfortunately the heavens opened during the 2014 challenge, which led to the route being shortened from 100 miles to 86 miles. However, Victoria enjoyed the challenge so much she has already joined our 2015 Team.

You could be riding with Victoria as part of Team Scleroderma 2015 as we have 9 places available for our supporters to join the challenge.

Find out more and join Team Scleroderma 2015 by visiting the “Support Us” section on our website: www.sclerodermauk.org Speak to a member of our team to receive an application form by email or post. fundraising@sclerodermasociety.co.uk

Always wanted to take on a once in a lifetime challenge for the Scleroderma Society but weren’t sure if we offered the challenge you want?

If you would like to take on a challenge that is not currently offered then please get in touch. We work with two events partners and we are sure that we will be able to find a challenge to suit you.

020 7000 1925

Scleroderma Society and our event partner, ensuring you have the best experience possible

We know that our old website made it difficult to navigate and find the right Joining an event with the Scleroderma challenge for you. So…we changed it and Society is a fantastic way to raise funds now you can easily find our challenges. for the charity whilst achieving another tick on your ‘once in a lifetime’ list.

Sign up to take on an event today and receive:  Dedicated fundraiser to support you with your challenge and fundraising  Welcome pack including fundraising support and fundraising top-tips Just head to www.sclerodermauk.org  Regular e-updates click on Support Us and Join an event  The support of both the Scleroderma News: Fundraisers

Our challenges are a great way for corporates to team build whilst encouraging staff to lead a healthy lifestyle and fundraise for charity. We can support your company and team with one of our standard or bespoke challenges.

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Sunday 26th April, more than 40,000 runners will be taking to the closed streets of London to complete one of the most prestigious running events in the world, the Virgin Money London Marathon.

The London Marathon is a fantastic event to be a part of or to watch from the sidelines due to the absorbing atmosphere. Spectators and runners come from all over the world to be part of this one day spectacular.

Scleroderma, he never let it stop him in life and he was and is my hero. I am hoping to raise funds and awareness for The Scleroderma Society, whilst having the huge privilege to run London 2015. www.Justgiving.com/RunCoxy

John and Charlotte are a father and daughter team running the marathon to raise funds to help by an ultrasound machine for scleroderma patients at This year the Scleroderma Society has the Royal Free. six runners taking part in the event. Let’s uk.virginmoneygiving.com/johncharlotte meet Team Scleroderma 2015: Greg is running in memory of his Mum, I am running the London Marathon in Gail Bennett. Greg said: “There really memory of my Dad who suffered from are no shortcuts and there is nothing

easy about running long distances. 26.2 miles seems a very long way to go! www.justgiving.com/GregTytherleigh Joining Ian, John, Charlotte and Greg are: Eli Myers Running because I want to do as much as I can to raise money for this condition, which my Grandma was diagnosed with.

Josh Levy Who wants to support the charity as one of his close friends relatives was diagnosed with scleroderma.

Lee Maxwell Who wants to continue fundraising for his chosen charity after completing the Brighton Marathon in 2012.

If you are out supporting runners on the day then come and join us at the Halfway point, Mile 13 and 22. We will be stationed on the Highway, by Swedenborg Gardens. Look out for the Scleroderma Flag on the day.

If you are supporting our team on the day grab yourself a supporters pack for only £12.00.

Includes a t-shirt, wristband, 5 x balloons and stickers.

Order yours today by heading to www.sclerodermauk.org ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——- ——-

If you would like to order your pack by cheque then please complete the below form and make cheques payable to ‘The Scleroderma Society’.

Full Name ___________________________________________________________________________ Address_____________________________________________________________________________ Postcode _______________________________________ Telephone Number _______________________________________________ Your telephone number will only be used if there is a problem with your order.

Quantity of Support Packs Required (£12 per pack) ____________________________ Interested in running the Virgin Money London Marathon for the Scleroderma Society in 2016 then register your interest today. Visit the “Support Us” section of our website: www.sclerodermauk.org or email: fundraising@sclerodermsociety.co.uk Scleroderma News: Fundraisers

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So, I think I may have gone slightly mad - I am voluntarily going to throw myself out of a plane at a height of 10,000 feet. I have wanted to do a skydive for years but I am finally making the 'plunge' on 14th March, a week before my 28th birthday (after several wind god sabotaged try's).

My mum was diagnosed at the age of 34 with Scleroderma and Raynaud’s.

Obviously as you probably know, Scleroderma is a connective tissue disease that affects the skin and other major internal organs and it's had a massive impact on our family.

My mum is the most articulate and smart woman that I have even known. Scleroderma has took so much from her, her job, her health, her marriage - I could list so many more too!

She lives in pain at all times but still manages to support her children and grandchildren as much as her disability allows her physically, but gives us every part of herself with love and support as only a mother could.

I wanted to do something to show her how much her love and support makes a difference in MY life and this is my way of trying to do something to help (I am crazy right?). The support from my colleagues, friends and family has been incredible and I'll have an amazing experience while doing something worthwhile!

I know that the day will come when this disease will take her from us. Until then, Mum, I appreciate everything you do for us and help you live the life you deserve in any way I can.

I read this quote and I guess it sums up how I feel about it all!

It's okay to be scared, but you have to get out there, open up, love, make mistakes, learn, be stronger, and start all over again.

Would I recommend it? I guess j can't answer yet. Ask me again in a month. And pray for no wind!...third time lucky?

Fingers crossed.

If you would like to skydive for the scleroderma society then visit: www.sclerodermauk.org Kirsteen Light at her first skydive attempt which was postponed because of the wind!

Raising funds for a charity in memory of your loved on is a wonderful way to honor and remember them. You can support the charity by collections in lieu, this is where you ask family and friends for donations in lieu of flowers.

Antonino Barber’s family decided to just that and raise funds in his memory in aid of the Scleroderma Society. The charity was extremely grateful to receive £461.00 in memory of Antonino.

If you would like to continue support the Scleroderma Society after the loss of a loved one, you may wish to donate on a special date or create a permanent in memory page where you can share stories and remember together.

Greg Condliffe and his daughter Emma, decided to create an online donation page to allow friends and family to donate a remember, Francis. We would like to thank the Condliffe family for their continued support and for raising £461.90 If you would like to raise funds in memory please visit the “Support Us” section of our website” www.sclerodermauk.org Scleroderma News: Fundraisers

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Now the Scleroderma Society have invested in a new database This year we are kicking off our Medical Equipment Appeal system we are now able to take credit and debit cards (MEA). payments via the phone.

MEA aims to fund medical equipment in each of the six This means that if you would like to donate to the charity you scleroderma specialist centres across the UK, ensuring that can now pop us a call on our normal number: 020 7000 1925 scleroderma patients receive the best quality of care and and we can process your donation over the phone. hospital staff have the resource in order to provide this.

By choosing this option not only is it environmentally friendly Our first medical equipment appeal will be for the Royal Free but is cost-effective for the charity, reducing administration London NHS Foundation Trust. time and cost.

We will be funding an ultrasound machine which will be used If you usually send a cheque in to the charity to donate or within the Planned Investigations Unit (PITU). This machine renew your membership then why not try giving us a call and will aid the nurse practitioners of the Vascular Access Service we can process your payment over the phone. in inserting midlines for Iloprost infusions. The midlines are inserted at the beginning of the treatment and last for the duration of the infusion so repeated cannulation and its sideeffects are eliminated.

You can donate to MEA by logging on to our website, clicking the “Support Us” section and clicking “MEA” or by posting a donation alongside your membership questionnaire.

For more information on midlines please contact Jane Pain Lead Nurse of the Vascular Access Service: jane.pain@nhs.net

At the Scleroderma Society we do not receive any government funding and so rely on the generosity of individuals to ensure that our vital work is continued and we move ever closer to discovering further treatments with the hope of finding a cure.

During 2014 we were only able to achieve the following due to donations and fundraising: 

Mail out to 10,000 GP surgeries to increase scleroderma awareness and engage health professionals with our online medical community  Continue to offer support 365 days a year to diagnosed individuals and their families  Commit over £100,000.00 to scientific and medical Our team is always on hand to offer you support and advice. research projects When you let us know about your fundraising you will also We are grateful for every single donation that is received and receive a fundraising pack filled with a range of items to boost your fundraising and thank donators. the work this enables us to do.

This is why we are asking you to consider fundraising for the charity during 2015. Not only does this raise much needed Visit the “Support Us” funds but also increases awareness of scleroderma and rare www.sclerodermauk.org autoimmune conditions.

Scleroderma News: Donate

section

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website:

Research The Scleroderma Society is committed to improving the lives of people affected by scleroderma. Your membership, donations and fundraising are vital to ensure we are able to make this happen. Over the years the Scleroderma Society has invested nearly one million pounds into scleroderma research.

In 2014 we invested over £100,000 in to 4 exciting research projects, 2 of which are detailed below. We will keep you updated on their developments over the year.

Autoantibodies The use of immunoprecipitation to identify rare and novel autoantibodies in a cohort of patients with anti-nuclear antibody negative systemic sclerosis.

Dr John Pauling & colleagues, Royal National Hospital for Rheumatic Diseases (RNHRD), Bath Background: Antibodies are small proteins produced by white blood cells which help the immune system to fight off infections such as viruses and bacteria. In scleroderma, antibodies are formed which are directed towards normal cell constituents (called autoantibodies).

The identification of these autoantibodies have become an essential diagnostic tool, helping clinicians to diagnose early scleroderma and also help to predict the future occurrence of complications of scleroderma such as lung fibrosis and pulmonary hypertension.

Autoantibodies may also provide clues as to why scleroderma has developed in the first place.

Professor Mayes and her team in Texas have led a study evaluating the progress of a very large group of patients with scleroderma. As part of this study, they have used standard laboratory techniques to look for the autoantibodies often found in the blood stream of patients with scleroderma. They have identified a group of ~200 patients who do not appear to carry any autoantibodies despite having typical features of scleroderma (“ANA negative” scleroderma patients).

Scleroderma News: Research

The project: Dr Pauling leads the clinical and research programmes for Scleroderma at the RNHRD. Professor McHugh’s laboratory in Bath have used specialist techniques to identify previously unidentified autoantibodies in rheumatic diseases such as myositis, dermatomyositis and scleroderma.

The proposed study will incorporate these techniques to look for rare and previously unrecognised autoantibodies in samples from Professor Mayes large cohort of “AN A n eg ati ve” s cler oderma patients. This study will help to identify new autoantibodies in scleroderma which could eventually prevent delays in the diagnosis of patients presenting with early scleroderma. By describing the clinical features found in patients carrying rare and previously unidentified antibodies, this work will guide clinicians to be aware of possible future organ damage which might occur later in the course of scleroderma (and screen for such problems more closely).

The identification of new antibodies may offer clues as to why patients have developed scleroderma in the first place. The proposed study will also help to bring together clinicians passionate about helping patients with scleroderma an d u n dertakin g research in this disease, from both the UK and the USA. Scleroderma genes Identification of the genes involved in the development of scleroderma by studying patient samples.

Sandra Guerra & colleagues, UCL Medical School Background: Scleroderma is an autoimmune connective tissue disease

which is characterised by fibrosis of the skin, organs and remodelling of blood vessels. Scleroderma patients have shown a deregulated immune system which leads to autoimmunity characterised by inflammation of blood vessels which are most noticeable in the skin. It has been reported that naturally occurring variants within the genes of scleroderma patients have shown to contribute to disease development.

The identification of these variants has proven to be key in understanding the role of genes within the disease and have proven fruitful for new therapies. Disease manifestation has also shown to have an environmental component where changes in the level of expression of genes have also shown to occur not due to the DNA sequence, but due to epigenetic processes which can modify the gene sequence or change to efficiency of gene expression.

The project: As scleroderma is a complex disease, it is critical to understand the disease mechanism to ensure better diagnosis and disease management. We propose to investigate the gene profiles of skin and samples from healthy and scleroderma patients to ascertain common genes, varian ts and regulatory pathways.

A study of this kind has never been done before and the results will give us a powerful tool which can then be further investigated. We will examine the top associated genes to ascertain their role in disease development and determine the role of variants within these genes. Overall we aim to develop a comprehensive list of genetic markers which can be used to identify scleroderma and disease subphenotypes. These can then be further investigated and potentially Page 17

used to stratify patients and to identify specific candidates for novel therapies.

By understanding the pathogenesis of scleroderma more clearly it will allow for better disease diagnosis which would lead to improved patient care and treatment.

Patients have been telling NHS England that they want to be offered more convenience, choice and control in how they access GP services. Increasingly, they also want to be informed and involved in decisions about their own care and treatment. As a result there are now a variety of ways you can be involved in the NHS health care that you receive:

Specialised lab. equipment

By David Abrahams and colleagues, Royal Free Hospital, London Support from the Scleroderma Society has been used to underpin 2 important activities within the Department of Rheumatology at the Royal Free Hospital. Firstly to assist in the development and implementation of a database and data-input to store and retrieve large amounts of data relating to scleroderma, and secondly the development of web-sites to provide an external face to the Department and raise the profile of our biomedical research and clinical trials.

The database is important to our research, translational and clinical programmes. It contains clinical, biochemical and historical information on scleroderma and disease sub-sets, and links to the tissue bank allows us to select samples from patients with specific disease characteristics.

5 key areas where the database provides essential value to our work are:  Identifying patients group for trials  Further understanding the natural history of scleroderma and predictive modelling  Building up a picture of disease complications and their risk  Linking our bio bank of tissue samples, serum/plasma and cells to defined patient groups  Shaping aspects of our biomedical research in target and biomarker discovery The support also provided a platform for the creation of the Developmental websites and links to associated web sites.

These sites are essential in projecting the external face of the department which can be readily accessed to acquire relevant useful information. These web sites provide access to our overall goals and vision - to increase understanding of the condition to ultimately lead to patient benefit, via promote emerging talent and innovation through investigation, invention, collaboration, education and clinical trial.

Scleroderma News: NHS Changes

Access to a care plan Everyone with a long-term condition, such as scleroderma, can have a care plan if they want one, and by 2015 everyone with a long-term condition will be offered one. You can decide how you want to manage your health and choose what's best for you. It is an agreement between you and your health professional to help you manage your health on a day to day basis. You can assist in the process of determining what care you need and how you receive it.

If you would like a care plan, talk to your GP, nurse or social worker about the support you need to manage your condition better. Mention things that are important to you and any goals you want to work towards. These can range from losing weight or stopping smoking, to going out more. Also, try to talk about anything that might be worrying you. By talking about your care plan with your health care provider, you can say how you want to manage your health and choose what is best for you.

Access to you medical records online As of April 2015 GP practices are required to offer and promote: online appointment booking, repeat prescriptions and access to summary information (as a minimum). Access to patient record can be extremely beneficial to scleroderma patients – you will see test results as soon as they are added to your record, choose to share your records with other healthcare staff that are treating you and keep track of your medical history, medications and immunisations. This service is completely voluntary. Just let your GP surgery know that this something you would like. Some surgeries are already offering this.

Evidence shows that patients who are informed and involved in their own care have better outcomes. This is why the Scleroderma Society is keen to work with you to help you understand your condition, support you through your care and further your ability to effectively manage your condition.

If there is something you need to know then phone the helpline. Our helpline staff are scleroderma experts - they either have scleroderma or work within the field: 0800 311 2756 At the Scleroderma Society HQ we are developing a range of self-management programmes that will focus on a variety of scleroderma aspects, from nutrition to dealing with fatigue. Keep an eye on our website, Facebook and Twitter to find out about events taking place near you or phone the office: 020 7000 1925.

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DOC SPOT

Chris Denton is a Professor in the Centre for Rheumatology at the Royal Free Hospital in London and runs the Scleroderma Clinic. His major research programme spans both clinical and laboratory scleroderma trials.

A r e m e n i er e s - l ik e s ym p t o m s attributable to Scleroderma and/or other connective tissue diseases…and if they are, why?

Meniere’s syndrome describes the feeling of dizziness or room spinning that results from abnormal activity in the balance sensors of the inner ear.

It may be associated with ringing or buzzing sensations (tinnitus) or poor hearing.

It is not a specific complication of scleroderma although it is sometimes associated with some medication that is used in scleroderma patients such as hydroxychloroquine and others.

Treatments for Meniere’s symptoms include drugs to reduce dizziness and these can usually be taken in scleroderma.

Occasionally scleroderma and other connective tissue diseases can cause damage to the small blood vessels in the inner ear and so this may explain Meniere’s symptoms in a small number of cases.

Treatment is the same as in the much commoner variety of the disease.

Can you get Raynaud’s of the nipples?

I am 37 and not breastfeeding. I did however feel this pain throughout both of my pregnancies. It is excruciating and affects the left more than the right. The only thing that stops the throb is heat. My GP prescribed nefedipine. Do you have any suggestions how to manage Raynaud’s in the nipples, other than walking around with a hot water bottle in my bra?

Raynaud’s can affect any part of the body that has a temperature sensitive circulation and whilst the most frequently affected sites are the hands, ears, knees and feet it can also affect the nipples, tongue and occasionally the penis. When the nipples are affected this can give all the symptoms that you describe.

Some patients have found that GTN ointment can help when applied to the nipples but this can also cause Scleroderma News: Doc Spot

headaches as it can be absorbed into the blood. The same measures that are used to tackle other sites of Raynaud’s attacks may help – concentrating on marinating a warm central temperature and avoiding precipitant where possible. Vitamins or other supplements are worth considering as they often cause fewer side effects than prescription medications and approaches such as evening primrose oil, vitamin supplements or fish oil capsules might be worth trying.

I use a proton pump inhibitor and have incredibly low iron levels, despite eating a good diet. Could the two be related in any way?

Absorption of iron occurs in the small intestine but it can only be absorbed when it is a soluble form. Stomach acid normally increases iron salt solubility and so when you are taking a proton pump inhibitor drug (such as omeprazole - PPI) the amount of soluble iron is reduced. In general low iron levels require supplements to be taken as tablets or liquid formulation. This does make oral iron supplements less effective but almost always the need for PPIs to treat heartburn and swallowing problems outweighs this disadvantage.

It is worth experimenting with different iron preparations to find the best tolerated so that does can be increased. Sometimes infusions of iron are given if levels remain low despite supplementation.

Has anyone else with scleroderma noticed any problems with their eyes and is there anything that can help prevent this from getting worse?

Scleroderma causes thickening and tightening of the skin in affected areas and also later leads to thinning of the fat and muscle layers under the skin.

This can affect the face and sometimes the skin around the eyes becomes tightened. In general facial

exercises can be used to try and maintain movement and the skin should be kept hydrated with regular application of moisturiser, as the skin often becomes abnormally dry.

Is there any evidence of a warmer climate really making a difference with Raynaud’s attacks?

Raynaud’s attacks are triggered when the body sense low temperature and in general attacks are triggered by change in temperature or by emotional or physical stress.

Environmental temperature is only one factor and so moving to a warm climate is unlikely to completely eradicate Raynaud’s symptoms. This is especially true in scleroderma where damage to the blood vessels may be less reversible than in primary Raynaud’s that is not associated with any underlying disease. In addition, even in warm climates there may be marked temperature changes in the evening or in air conditioned rooms.

However medical research has confirmed that in scleroderma Raynaud’s is less troublesome in southern climates compared with temperate regions. Wherever you live, maintaining a warm core temperature with lots of layers is helpful.

There is no evidence that moving to a warmer climate or redu cin g Raynaud’s attacks will reduce the risk or progression from Raynaud’s to a defined connective tissue disease such as scleroderma.

If you have a question for the Doc please email: info@sclerodermasociety.co.uk Page 19

Mary McDaid, Rayn aud’s Scleroderma Ireland

and

Raynaud’s & Scleroderma Ireland, previously known as the Irish Raynaud’s & Scleroderma Society, has had many ups and downs since it was founded in 1988. Originally supported by a very active and hardworking voluntary committee who fundraised every year to support the annual expenses. As with all things, however, the committee members grew older, their supp orters ret ired and fundraising became ever more difficult. It was decided a change of format was needed for the group to continue to exist. With a new chairman on the Board it was decided to push for a more public face for the organisation with the hope of improving awareness and funding.

To this end we moved from my garage (converted I might add) to a small office. A lot of good things happened including increased use of our Facebook page thanks to Aoife Keogh who worked with us for 6 months and was brilliant at managing this. We also set up a closed chat group which has grown hugely and includes global members and has proved to be a wonderful way for people to support one another.

We joined forces with Lupus Group Ireland to hold a joint conference in April 2014, which was a huge success and thoroughly enjoyable. Our speakers included a psychologist, who is also a member, and was truly

inspiring.

Some of our recent fundraising included a cycling event, a rugby evening at which the Irish rugby team coach Matt O’Conner and a TV commentator George Hook. We also had a quirky Swap Style event people brought their designer or boutique clothes and donated them to the charity in exchange for swapping for another outfit donated by someone else.

We celebrated World Scleroderma Day with (among other things) an Afternoon Tea Party in a Dublin city centre hotel. The hotel provided wonderful sandwiches (of the dainty variety) with scones, cakes and treats of all kinds. They looked amazing and tasted just as good! We had a very moving speech from a member about her experience of scleroderma and George Hook gave an entertaining speech. The whole afternoon was a huge success and everyone gave generously.

In 2014 we also employed a Support and Outreach Nurse, Ann Fahey.

This was made possible by a grant from the HSE, the Irish Health Service. This has been a tremendous improvement in the services we are offering to members. Further grant aid has been applied for in the hope of continuing this service. This all sounds very positive and indeed it is.

Unfortunately, however, the move to an office coincided with the recession.

In spite of a valiant battle to r a i s e awareness, i n c r e a s e fundraising and safeguard the organisation we eventually had to face the fact that we had to reduce our expenditure. A further problem was caused for all charities with a large scandal caused by the discovery that several large, government funded charities were paying their CEOs very large wages plus bonuses.

This led to a decrease in donations and even the big charities found themselves with greatly reduced incomes. It had a catastrophic effect on small charities like ourselves who depend on donations for most of our income. And so, in order to protect the nurse position, it was decided that the Executive Director and the administrator posts would be made redundant.

At the end of September 2014 Michelle McGill and I finished work at RSI. Michelle needed to find a new job, which she quickly did. As I was about to retire soon I continued to work for RSI as a volunteer to facilitate the nurse in supporting members.

So, please don’t expect an immediate answer to emails or phone calls! I’m in the office on Tuesdays and I deal with all of those things on that day. I have assistance from another volunteer, Therese Brosnan. Together we parcel up orders, process invoices, answer phone messages and generally keep things ticking over. We have a lot of fun while we work and I really look forward to Tuesdays.

This is a resilient organisation and I hope that it will continue to be around to support people who are dealing with Raynaud’s and scleroderma. We are still members of FESCA and it is wonderful to have this vibrant umbrella group working so hard for all of us. It is also great to know that there are so many similar global groups working for the same important cause.

Scleroderma News: Greetings from Ireland

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Local Support News & R.A.M Update SclSoc and RSA award—nominate your local support contact as support group of the year!

LOVELY GLOVELIES! During the month of February we celebrated Raynaud’s Awareness Month (RAM) by highlighting the problems associated with Raynaud’s, which affects up to 10 million people in the UK (that’s 1 in 6 of us!) with the condition. Our message was clear ‘PLEASE DON’T IGNORE IT!

Does your support contact or group deserve the award for support group of the year?

A HUGE THANKS to all of you who helped us. Many of you put up posters in your local doctors surgery, hospitals and community centers, followed us on Twitter, Facebook and Health Unlocked and achieved fantastic fundraising efforts.

If you have received excellent support, information and assistance. Then we want to hear from you.

We portrayed your stories and shared your experiences from Hastings to the Highlands! Mathew Barry, age 15, from Hastings raised over £1,000 by cutting off his glorious locks. Laurie Hughes, mum of Gracie, aged 2 ½, shared her daily challenges of looking after a toddler with Raynaud’s and Caroline Goldstein’s story of being misdiagnosed with thrush whilst breastfeeding her baby, when it was actually Raynaud’s of the nipples.

This is your chance to showcase the wonderful work of your support contact.

Do you attend a local support group and think they should win?

Please send an email, a letter or phone the office to let us know why. No more than 1/2 A4 page of text is required. Deadline for nominations is 30th April. The winning group will be announced at the gala dinner on the 20th June (following our joint annual conference).

Local Support Group Updates: Bedfordshire group is meeting again on 22nd March 2015 at 10.00am in the coffee shop/restaurant in Frost's Garden Centre at Willington. If you would like to find out more please contact Rita Boulton.

Burton on Trent first meeting of the year and also the 10th year celebration! The celebration will be taking place at 7.30pm on April15th at The Brewhouse. If you would like to come along please contact Helen Nutland.

Our stories and products were featured in the ‘Sun’ and we’ve received editorial in various publications including ‘Chat’ magazine along with some great on-line coverage, all across the globe.

As we all look forward to warmer Spring climes, we are still appreciative of your support, sign up at @raynaudsuk, ask a question at info@raynauds.org.uk or ensure you stay covered and warm whatever the weather, go online www.rsa-shop.co.uk

Break Time Can you solve the riddles?

1. What has a foot but no legs?

2.What travels around the world but stays in one spot?

3. I’m tall when I’m young and I’m short when I’m old. What am I? 4. Mary’s father has 5 daughters – Nana, Nene, Nini, Nono. What is the fifth daughters name?

5. A dad and his son were riding their bikes and crashed. two ambulances came and took them to different hospitals. The man’s son was in the operating room and the doctor said, “I can’t operate on you. You’re my son.”

How is that possible?

Answers at bottom of page

We would love to expand our local support groups. If you are interested in becoming a local support contact and developing a group in your area then please contact Amy Baker: amybaker@sclerodermasociety.co.uk or 020 7000 1925.

Break Time Answers: 1. A snail. 2. A stamp. 3. A Candle 4. Mary. 5. The doctor is his mum.

Scleroderma News: Local Group News

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Local Contacts & Support Services To find out about support groups in your area please email: localsupport@sclerodermasociety.co.uk Bedfordshire Rita Boulton rita@ sclerodermasociety.co.uk 01767 312 544

East Sussex Rosanna Clifton rosanna@ sclerodermasociety.co.uk 01424 426 738

Buckinghamshire & Hertfordshire Marilyn York marilyn@ sclerodermasociety.co.uk 07702 592 387

East Yorkshire Lynn Hind lynnh@ sclerodermasociety.co.uk 01482 354 312

Burton on Trent Helen Nutland helen.nutland@ sclerodermasociety.co.uk 01283 566 333 x5247 Cornwall Alex Pooley alex@ sclerodermasociety.co.uk 01736 755 845 County Durham & Teeside Jessie Pickering 01388 527 840 Derbyshire, S.Yorkshire, Staffordshire & Cheshire Lynette Peters lynette@ sclerodermasociety.co.uk 07947 350 815

Essex, Kent & East London Amanda Thorpe dollydumpling23@gmail.com 01702 344 925 Exeter Mike Corbett mikec @sclerodermasociety.co.uk 07918178613 Hampshire Tracey James tracey@ sclerodermasociety.co.uk 02392 677 476 Ireland Michelle McGill michelle@irishraynauds.com 08183 63 999

Diana Twigg diana@ sclerodermasociety.co.uk 01298 24 539

Lancashire & North West Yorkshire Sheila Procter sheila@ sclerodermasociety.co.uk 01282 42 9004

East Anglia Jacky March jacky@ sclerodermasociety.co.uk 01394 286 637

Merseyside Helen Lingwood helen@ sclerodermasociety.co.uk 01512 801 194

East Midlands Cheryl Darch cheryl@ sclerodermasociety.co.uk 01162 717 180

Newcastle and Northumberland Lindsay Wilkinson lindsay@ sclerodermasociety.co.uk 07880 238 213

North East Scotland Susan Wilson susan@ sclerodermasociety.co.uk 07740 185 627 North Wales Kate Owen kate@ sclerodermasociety.co.uk 01492 515 834 Oxfordshire & Berkshire Melanie Bowen melanie@ sclerodermasociety.co.uk 01865 517 033 Plymouth Anne Sheere anne@ sclerodermasociety.co.uk 01752 338 156 Powys & Shropshire Jennifer Ames jennifer@ sclerodermasociety.co.uk 01544 267 988 Scotland Frances Bain frances@ sclerodermasociety.co.uk 0131 477 1122

02920 625 056 Surrey Nicky O'Shea nicky@ sclerodermasociety.co.uk 01483 764 524 West Midlands Jane Beach jane@ sclerodermasociety.co.uk 01527 459 552 West Sussex Lesley Dodd lesley@ sclerodermasociety.co.uk 01903 753 971 West Sussex Jo Frowde jo@ sclerodermasociety.co.uk 01403 741 445 Wiltshire & North Hampshire Lynn Morton lynn@ sclerodermasociety.co.uk 01980 863 444

Somerset & Gloucestershire Judith Foster judith@ sclerodermasociety.co.uk 07985 335 336 South London Celia Bhinda celia@ sclerodermasociety.co.uk 020 8698 6294 South Wales Belinda Thompson belinda@ sclerodermasociety.co.uk

Join us on Health Unlocked HealthUnlocked is a social network for health. By finding others with similar health backgrounds you can take on day to day health concerns together. www.healthunlocked.com/ sclerodermasociety

Join us on Facebook https://www.facebook.com/sclerodermasocietyuk Join us on Twitter #sclerodermauk

Scleroderma News: Local Support

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Contacts Connecting you to Scleroderma Professionals, groups and nation-wide support networks Susie Hoare susie@ sclerodermasociety.co.uk Helena Rozga Helena@ sclerodermasociety.co.uk Office & Administration Manager: Jennifer Malone 020 7000 1925, jennifermalone@ sclerodermasociety.co.uk Helpline Manager: Helena Rozga, Helena@ sclerodermasociety.co.uk Fundraising/volunteer enquiries: Amy Baker, fundraising@ sclerodermasociety.co.uk Helpline: Although The Scleroderma Society can not offer medical advice, they operate a free helpline, offering a kind ear. We will help in any way we can between 09:00—19:00 every day. Call 0800 311 2756.

Carer Support Contact: Michael Thorpe, 01702 344 925, MTDAAC@live.co.uk

Specialist Nurses

Nurse led lines for general inquiries only, in non emergencies. If the nurse is unavailable please leave a message: Bath Royal National Hospital for Rheumatic Diseases: Sue Brown 01225 428 823 Belfast Ulster Hospital: Audrey Hamilton 02890 561 310 Dundee Ninewells Hospital: Steve McSwiggan 01382 383 233 Leeds Chapel Allerton Hospital: Elizabeth Tyas 01133 923 035 Liverpool Aintree University Hospital: Jan Lamb 01515 255 980 bleep 2231 London Royal Free Hospital Scleroderma queries: 020 783 02326 and Pulmonary hypertension: 020 7472 6354 London Royal Brompton Hospital Respiratory queries: Lucy Pigram 07758 894 3175

Newcastle Freeman Hospital Scleroderma queries: Karen Walker 01912 231 503 and Pulmonary hypertension: Rachael Crackett / Julia De Soyza 01912 137 418 Portsmouth Queen Alexandra Hospital: Paula White / Julie Ingold 02392 286 935

To find more specialists in your area visit the website or contact the office.

Scleroderma News Write to:

The Scleroderma Society Bride House, 18-20 Bride Lane, London EC4Y 8EE

Phone us: 020 7000 1925 Email the editor: info@scleroderma society.co.uk Proof Reading: Rosemary Goodwin Roger Sinclair– Clarke Printing & Publishing: Jarvis & Company Issuu.com

Manchester Hope Hospital: Liz Wragg and Catherine Lambe 01612 060 192

Help us become more environmentally friendly by receiving your next newsletter as an email. Email info@sclerodermasociety.co.uk to chose this option. Don’t forget to recycle this newsletter once you’re finished: share with a friend or pop in the recycle bin.

Scleroderma News: Contacts

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