Raynauds - Hot News

30th Anniversary Edition

Issue No. 120

Minister Praises RSA Following our House of Commons Reception to celebrate the RSA’s 30th Anniversary, held on 1st February, Alsager’s MP Fiona Bruce, made a plea to Parliament on behalf of Raynaud’s and scleroderma sufferers. She asked Health Minister, Paul Burstow, what steps were being taken to provide services for people diagnosed with Raynaud’s and scleroderma.

The commissioning of those services directly through one national commissioner to a national standard should ensure better planning and co-ordination of services, which will be of benefit to patients. The scope of this commissioning, and the extent to which it will cover complex rheumatology services, is still being considered”.

In response, Paul Burstow MP paid tribute to the work that the Raynaud’s & Scleroderma Association has done over many years in raising funds, raising awareness and making sure there is a greater focus on the complexity of these conditions. He said: “The work we have done in establishing the NHS Commissioning Board will mean that in future, for the first time, there will be one organisation that will be able to look at issues involving specialised and complex needs that require a national focus.

Comment from Prof. Chris Denton:

Routine commissioning is a local responsibility, which in future will be led by clinicians, who best understand patients’ needs. From April 2013, the NHS Commissioning Board will have a clear focus on commissioning services for people with rare, specialised conditions.

"Scleroderma is a rare disease but one that requires co-ordinated care involving a large team and integration of specialist and non-specialist services. It is critical to keep scleroderma within the group of diseases that receive special attention within the NHS to ensure that patients receive good care and to elevate standards. There are major initiatives to define the best approach to treat scleroderma and its complications through the efforts of the UK scleroderma centres and also the British Society for Rheumatology."

Volume 2 2012

In this issue...

Prof. Chris Denton Bumper Doc Spot Pages 3 - 6

Dr. Bridget Griffiths Lung article Page 8

Cool Million Reaches £600,000 Thanks to everyone who has supported the Cool Million Appeal. We have now reached a total of £600,00 but we still need to raise a further £400,000 by the end of the year, so please do keep up the momentum with your fundraising events, donations, sponsorship etc.

Tel: 01270 872776 www.raynauds.org.uk

There has been a tremendous response to the request to raise funds for specific research into Raynaud’s and scleroderma and some projects are already underway, with others being considered.

Scleroderma Awareness Week 11th - 16th June Our theme for the week is ‘Scleroderma - It’s More Than Skin Deep’ Book now for our 30th Anniversary Conference - 22nd September

Celebrating 30 years 1982 - 2012

Prof. Alan Tyndall Self Management Page 9`

Donation Burton upon Trent Support Group Page 15

Welcome Dear Readers, Anne H Mawdsley MBE

We have been busier than ever at the office over the past few months and can’t thank our members and their supporters enough for their efforts and contributions to our Cool Million Appeal. The support for my pedalo challenge has been overwhelming, which has spurred on my training!

Raynaud’s Why do we include both Raynaud’s and scleroderma in our title and in our newsletters?

Let me explain - when I founded the charity in1982, it was called the Raynaud’s Association.

However, over the years it has become apparent that many of our members also have scleroderma. Our original Trust Deed stated that we could only fund research into Raynaud’s.

We therefore changed the name of the charity in 1990 to what it is today - the Raynaud’s & Scleroderma Association.

Research which is being carried out into scleroderma also includes research which will ultimately help people with Raynaud’s as Raynaud’s is very much a part of scleroderma.

The high frequency of primary Raynaud’s makes it an important issue for all doctors.

We know that Raynaud’s is very common affecting approximately 10 million people in the UK. Primary Raynaud’s is a benign condition that varies from being very mild to more severe. On the whole Raynaud’s is debilitating, but association with connective tissue disease and especially with scleroderma (systemic sclerosis), occurs only in a small minority of people with Raynaud’s. The chance of anyone with Raynaud’s developing scleroderma is very small as there are only 8,000 people with this condition in the UK. At the Royal Free specialist centre, about 1 in 100 cases of Raynaud’s is linked to a major connective tissue disease.

Nevertheless the consequences of missing an associated diagnosis can be serious and it is important to highlight the connection.

We have heard from many of our members who have told us that they thought they had primary

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Raynaud’s. It was only through reading articles in Hot News that they sought advice from their doctors as to whether they had an underlying condition. This very much helped either to put their minds at rest or meant they could get an early diagnosis of scleroderma and therefore begin treatment.

I feel it is important to get across the message that the name of our organisation should not increase concern and anxiety amongst sufferers of primary Raynaud’s. Our aim has always been to give up to date, relevant information on both conditions.

However, if you have any queries which are not covered and which you would like us to address please do give me a call on 01270 872776 rather than worrying!

Scleroderma Week 11th - 16th June This year we have changed the date of Scleroderma Week to coincide with my Pedalo Challenge. We would really appreciate any help regarding creating awareness of scleroderma. Raynaud’s will also be included for the reasons given above but as you will be aware we hold a special month for Raynaud’s in February each year.

We need to get the message across that it is not just our skin which is affected in scleroderma.

If you think you could help to spread awareness, we can supply press releases, posters etc. The press are always keen to have case studies so this is an area which we would like to explore more fully with your input.

I think every day of the quote from one of our members, Maureen Collins, when someone said to her how well she looked, her reply was “It’s not my face that’s ill”. How very true is that?

Chief Executive Anne H Mawdsley MBE President Prof. Dame Carol Black DBE Trustees Barry Hicks Joanna Kaddish Beverley Myers Jeremy Pearson

New Staff Members We were delighted to welcome Lucy and Helen as part-time members of staff, who were a great help particularly over the Christmas period. Many of you will have spoken to them on the telephone. Lucy will be leaving us at the beginning of August as she is expecting her first baby in September. We wish her well as a new mum.

Online Shop Karen has been working extremely hard to dispatch the many online orders we receive every day. All of the recommended products are available on our website www.raynauds.org.uk and we are always happy to help should you need any assistance in ordering.

Postage You will be aware there has been a significant rise in the cost of postage. This is having a substantial effect on our charity.

Could I please request that when members write to us and require a reply, that you send sufficient postage or supply an email address.

Patrons Sharron Davies MBE Roger Jefcoate CBE Veronica, Lady Piercy Nick Ross David Wilkie MBE Medical Advisors Dr. M Anderson Prof. J Belch Sister S Brown Prof. C Denton Prof. A L Herrick Dr. C Lovell Dr. R Macdonald Prof. P Maddison Prof. R Moots Prof. D Scott Prof. A Silman Dr. D Veale

Head Office: 112 Crewe Road, Alsager Cheshire ST7 2JA Telephone: 01270 872776 Fax: 01270 883556

Conference We are looking forward to welcoming members to our 30th Anniversary Conference the weekend of 21st/22nd September. There are still a few places available but we are limited in space at the hotel in Chester.

This year will be a very special conference to be opened by our president, Prof. Dame Carol Black.

Enjoy the summer!

Anne

Email: info@raynauds.org.uk www.raynauds.org.uk Charity Reg. No. 326306

Disclaimer: The Association does not accept responsibility for the information contained in the newsletter, either medical or the advertised products.

Remember what suits one person does not necessarily suit another. If in doubt consult your doctor before trying any suggested remedies.

Doc Spot Your Questions Answered Professor Chris Denton

FRCP

Consultant Rheumatologist, Royal Free Hospital

I sometimes get cool or cold hands while at home. Though my skin does not turn white, they still feel cool. Should I wear thin gloves during these times or let them stay cold? If I warm them up, they return back to coldness a few minutes later.

It is usual for patients with Raynaud's to have cold hands but unless there is pain, loss of sensation or difficulty using the fingers then probably it is more important to keep warm centrally with lots of layers - in primary Raynaud’s phenomenon (RP) attacks are triggered by the body feeling cold rather than the temperature of the fingers.

I developed Raynaud's after taking a regular doses of paracetamol over 2-3 weeks due to an injury. I read afterwards that paracetamol can be a cause of primary Raynaud's.

The possible link to Raynaud's was not on the warning label or instructions for paracetamol. Would this be grounds for legal action?

I am not aware of any association between paracetamol and Raynaud's but stress, injury and other traumatic events have been reported as triggers - although this is probably a case of becoming aware of the symptoms rather than the RP actually starting for the first time.

If my hands feel cool or slight cold, should I always warm them up through external agents (gloves, heater etc.)? Would letting my body try to warm them by itself without the use of aids build its defences?

Patients with Raynaud’s phenomenon (RP) become very expert at working out what works best for them - a combination of using local approaches (gloves, hand warmers etc.) and keeping warm centrally as well as taking supplements/medications is usually helpful. The RSA provide excellent advice and support via their website and literature. RP may not be life threatening but it can be very debilitating and patients benefit from expert advice wherever it comes from.

I have read that many people ‘lose’ Raynaud's over time. Would moving to a warmer climate increase this chance? Do coldness ‘attacks’ reduce this chance? Is there any evidence about why some people’s Raynaud's gets better while others do not.

For primary RP many patients do find that the symptoms become less troublesome over time. This may reflect lifestyle changes and simple measures, medication that has been prescribed or it may reflect a reduction in underlying severity. This might occur for example with the hormonal changes that occur with age (RP is mainly a female condition and can be affected by oestrogen levels for example).

Unfortunately improvement does not occur in all cases.

My problem concerns a persistent and painful blister near the cuticle of my right index finger which I've had for about two years. It becomes angry-looking and throbs, then will subside and form granulations. It then appears to dry up but a week to ten days later it forms again. I have tried many products - zinc, vaseline, sudocrem, manuka honey etc. - to no real effect. When antibiotics did nothing either, my GP referred me to a dermatologist who believes the cause is primary Raynaud's which I have had for many years. After trying betnovate, without effect, he prescribed Elocon ointment, a corticosteroid, which I applied for three weeks.

After this time there was an improvement, leaving just a small dot with no swelling.

However, two months ago the blister reappeared and the cycle has continued, although not as painful as it was originally. I am due to see the dermatologist again but would like to know, in view of your specialised knowledge, what treatment you would recommend. Neither my GP nor the dermatologist have referred to it as an ulcer.

Raynaud’s phenomenon is associated with poor nutrition of the fingertips and the edge

of the finger nail is often a site that is affected. This leads to a susceptibility to local infection and this may explain your problem as it can lead to blistering and pain. Chronic infection may lead to overgrowth of the healing ‘granulation’ tissue and the treatment that has been given seems very reasonable. It may require a more prolonged course of tablet antibiotics.

Being new to Raynaud's is it possible my aching calves are an underlying symptom?

Primary Raynaud’s without any associated disease would not be expected to lead to aching calves but some of the disorders associated with secondary Raynaud’s could lead to additional symptoms. You should discuss this with your doctor as some simple tests may help to clarify any association.

My hands are really rough, with flaky skin and my fingernails are also brittle and flaky and just will not grow - they split all the time.

Is this connected to the Raynaud's?

Changes in the skin may suggest an associated condition in cases of secondary Raynaud’s, such as scleroderma or muscle inflammation (myositis). However, the finger nails may be affected in primary Raynaud’s due to reduced blood supply to the finger tips. Treatments that benefit Raynaud’s may help and simple approaches such as keeping the skin well hydrated with moisturiser and nails trimmed short can also help. Sometimes damaged nails are associated with infection and this may require specific investigation and treatment.

Is hyperbaric oxygen used for the treatment of Raynaud’s?

Many patients choose to access this treatment privately and can access some of the muscular sclerosis (MS) facilities at less cost than fully private. Some of my European colleagues have reported positive effects for very severe ulcers but it is not something we have used in the NHS, or privately, although I know some patients say it has helped their Raynaud's. However, there are some concerns about the impact on lung disease in scleroderma and there is a lack of trials in this area. Good information can be obtained from The British Hyperbaric Association www.hyperbaric.org.uk

If you have a question you would like Professor Chris Denton to answer, please send to Head Office 03

Doc Spot I've seen a rheumatologist and he can't understand why I have numbness and tingling in my fingers and tip of my nose ALL the time. I am led to believe that normally the tingling/numbness usually only lasts as long as the white fingers are present. Can you shed any light on this problem?

Raynaud’s attacks can reduce blood supply to all extremities including the tip of the nose and fingers. This can result in nerve damage with numbness but your doctor is correct that this is usually intermittent and related to attacks and so it may be worthwhile having further assessment and investigation to look at nerve conduction or other evidence of a nerve abnormality (neuropathy).

Raynaud’s affects me all over my body and also internally. If I eat or drink something cold my tongue, cheeks, lips, and oesophagus swell up. Is this common?

This symptom can occur in several connective tissue diseases and is termed angio-oedema. It is important to have assessment to try and define this better – sometimes specific antibodies are present in the blood and this may help to define the best treatment.

Is it common to bruise easily if you have Raynaud’s?

Some of the medical conditions that are associated with Raynaud’s, or treatments for them, may be associated with easy bruising. An example of this is if Raynaud’s is related to connective tissue disease that requires steroid therapy. This is something that should be discussed with your doctors as it may be important to do some additional blood tests if there is not already an explanation.

I was diagnosed with Raynaud's about 4 years ago and with hypermobility around 3 years ago. Having visited the hypermobility web forum, I’ve learnt that quite a lot of people also suffer from Raynaud's, either primary or secondary and some even have scleroderma. I was just wondering if there was a known correlation between the two?

Hypermobility can occur when there is an inherited increase in the flexibility of connective tissue components such as collagen. Collagen is important in blood vessel structure and so it is plausible that hypermobility of the blood vessels may lead to or aggravate Raynaud’s. This is an area worthy of further research.

I have been diagnosed with Raynaud's for a couple of years, and despite nifedipine, have

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freezing feet every day. I even had an ulcer for the first time. My nose gets very cold sometimes too. However, I've only ever had a couple of times when my hands were involved? Am I odd?

Raynaud’s attacks can affect any extremity including the nose and ears although most patients also experience symptoms in the hands. Nifedipine may help reduce symptoms but does not work in all cases and so it would be worthwhile exploring other treatments. Your doctor can advise you on this.

I have Raynaud's. Will I eventually develop scleroderma?

Although almost all patients with the systemic form of scleroderma develop Raynaud’s phenomenon it is still a very uncommon condition. Less than 1% of Raynaud’s sufferers develop any associated disease.

I have had vasculitis in my lower extremities which is now in remission. I have also developed neuropathy from a chemotherapy drug. Now I have Raynaud's in my feet and hands. The only time I have any relief from the pain in my feet is when I'm able to get some sleep. I feel very alone. Although I have friends that are supportive, I feel like the pain is winning the battle.

Vasculitis is often associated with symptoms of Raynaud’s and also of neuropathy. There are treatments that can improve these symptoms and also some potential for the nerves to recover and so you should seek specialist advice.

Sometimes nerve blocks or medication to reduce nerve sensitivity such as gabapentin can be used.

Are ulcers a result of Raynaud's, scleroderma or both? Am I destined to get them from Raynaud's?

Primary Raynaud’s phenomenon that is not associated with any underlying disease is very unlikely to cause ulcers, but in scleroderma up to half of patients report having ulcers at some point in their disease and so it can be an important problem.

Fortunately treatments are becoming better and more available.

I first went to the doctors with pins and needles and numbness in my feet. I had blood tests which were all clear. My fingers had been going white and numb but I put that down to working in a cold garage as there was no heating. I was diagnosed with Raynaud’s a month ago as my fingers had been going white and numb all summer.

Blood tests were repeated - still clear - but my feet are still numb with pins and needles.

Sometimes my right foot feels like it is burning and sometimes I get bad cramp at night. Could this be Raynaud’s or something else? My doctor told me to stop thinking about aches and pains and learn to live with it, which is not the answer I wanted.

Some of these symptoms are certainly typical of Raynaud’s but if there is a persistent problem in one extremity as you describe then further investigation may be needed to check for any nerve or additional blood vessel abnormality. I suggest you discuss this further with your doctor.

Sometimes routine blood tests are negative even when there is an associated connective tissue disease with Raynaud’s.

I wondered if there is a possible connection with hypothyroidism. Certainly cold hands and feet are symptoms of this disease. My daughter suffered at one time from very cold hands which I think might have been diagnosed as Raynaud’s. I myself have hypothyroidism and as you know it is possible, if not quite common, to have more than one autoimmune disease. From experience I know that diagnosing hypothyroidism in this country is sadly not good owing to the wide range used in blood testing. I therefore wonder whether Raynaud’s sufferers are being misdiagnosed due to these blood tests.

I myself only got diagnosed by insisting on the antibodies test. Even though I feel better now in that I have more energy, I still suffer from cold extremities and my nose in particular is always cold. I am however considered to be on the correct amount of medication according to these wretched blood tests, so put up with this problem. Is it therefore just possible that some Raynaud’s sufferers are undiagnosed as having underactive thyroid and if they were asked about other signs or symptoms of hypothryoidism, just how many may have this?

Thyroid disease is common and so can occur in association with Raynaud’s. The normal function of the thyroid gland is to helps regulate metabolic rate and that impacts on body temperature, an underactive thyroid can trigger or worsen Raynaud’s symptoms. Most thyroid disease is autoimmune and as autoimmune conditions can cluster together, about one in three patients with scleroderma will eventually also have some thyroid blood test abnormality. This is the reason we recommend regular thyroid blood testing in all patients with scleroderma, who will of course mostly also suffer from Raynaud’s.

These tests should be routine in assessing a new case of troublesome Raynaud’s.

Doc Spot I wonder if you can throw any light on my problem. I have always sung in many choirs. About five years ago my voice deteriorated and I had to leave the choirs.

Now I cannot sing at all, just croak. This has been very distressing. I saw a specialist and he said the vocal cords were curved shaped () instead of ||. Do you think this could be caused by scleroderma?

Changes to the voice are unfortunately quite common in scleroderma and usually result from a reduction in mucus to lubricate the vocal cords or inflammation of the vocal cords due to reflux of acid from the oesophagus (see below). This may lead to scarring that could alter the shape of the cords as you describe.

Raynaud’s phenomenon does not usually cause itching but it is a common feature of scleroderma, especially in the early stages of the condition when there is a release of proteins from activated inflammatory cells in the skin. Usually this settles as the scleroderma becomes more established.

Treatment can be difficult but antihistamine tablets and other medications and ointments may be helpful.

My right thumb joint is very painful and I am right handed. Are there any exercises that would help? It affects the strength of my grip and I drop things all the time. Anything that requires pushing/pressing causes pain to shoot through the thumb joint.

The most consistently effective drugs are those in a class called ‘proton pump inhibitors’. These reduce stomach acid and help reflux but higher doses than normal may be required.

These symptoms are likely to be a result of arthritis in the joint at the base of the thumb. This is a common site for osteoarthritis. It would be sensible to have this assessed as there may well be exercises and local treatment that may help. Patients with Raynaud’s phenomenon often have an increase of symptoms of other joint problems.

I have Raynaud’s and scleroderma and the main thing that impinges on my day-to-day living is finger ulcers. I have regular iloprost infusions and have just started to try viagra to help with circulation. Can you make any suggestions how to help the ulcers to heal?

I have scleroderma and have been really weepy and low lately and finding it hard to sleep - all I seem to do is cry. Is this to do with the disease or is it just me? I have never been like this before but it seems to be getting worse.

Ulcers on the fingers occur in up to a fifth of scleroderma patients at any time and around half of scleroderma patients report ulcers at some time in their disease.

Treatment includes medication to improve blood supply that helps ulcers to heal and also drugs to reduce ulcer formation.

Drugs such as iloprost, Viagra® (sildenafil) and bosentan (Tracleer®) are used although these are not suitable for all cases. Treatment of any local infection and careful dressing of ulcers is also important to help them heal and sometimes operations might be considered such as digital sympathectomy. Pain control is also very important.

Scleroderma can have a major impact on quality of life as well as causing many difficult physical symptoms and so it is not surprising that it can impact on mood. It is increasingly recognised that depression is an important aspect of many chronic diseases and should be addressed.

What are the most effective medications for Acid Reflux caused by scleroderma?

Is it common to get the most terrible itch in my legs? I have on so many occasions made my legs bleed from scratching them.

The reason I have been told I get the itch is because it is the blood flowing back to my feet which can feel like an itch. It goes away when I have had treatment for my Raynaud's (iloprost) but when the treatment is wearing off the itch comes back so it is definitely connected. It is also worse when my feet have been numb or freezing for a prolonged amount of time. How can I stop it?

I have probable peripheral neuropathy which has been treated for a while with amitriptyline, but is becoming painful again now. I'm wondering if I've actually developed scleroderma. I also suffer from gastro-oesophageal reflux disease.

MCTD is rather a confusing term that, as the name suggests, applies to patients that have developed features of more than one rheumatic disorder such as systemic lupus erythematosus (SLE), scleroderma, arthritis or myositis (muscle inflammations). Most patients with this condition show some features of scleroderma but this can be quite mild.

I have only recently been diagnosed with Mixed Connective Tissue Disease and scleroderma but my hands and feet are always really hot, so hot I have to soak them in cold water from time to time. I have never had Raynaud's apart from in one finger a couple of times so that hardly counts. Is this usual?

This may reflect a condition called erythromelalgia that can be associated with Raynaud’s and connective tissue disease.

In warm environments blood vessels dilate excessively and the extremities can feel very hot. Sometimes treatment for associated Raynaud’s symptoms can be helpful for both symptoms.

Does anyone know if scleroderma can cause one to see bright flashing swirls if one blinks in darkness?

This is not a feature of scleroderma but may be due to migraine which is associated with both primary and secondary Raynaud’s phenomenon.

I get repeated eye infections and I am fed up with them. Could this be connected to scleroderma?

Scleroderma can often reduce the production of tears due to inflammation or scarring of the tear glands. This can cause dry gritty eyes and also because tears are important in preventing infection this may explain an increase in eye infection.

Artificial tears or eye drops can be helpful.

What does ANA mean?

It would be sensible to see a specialist to assess whether blood tests or other investigations such as nailfold capillaroscopy confirm or exclude the presence of a connective tissue disease.

These symptoms could have several alternative explanations.

I have Mixed Connective Tissue Disease (MCTD), with scleroderma. I was recently diagnosed after being treated for Rheumatoid Arthritis for two years and would like to know more about it.?

ANA stands for antinuclear antibody, which is a specific test that reflects an overactive immune system which can occur in a number of diseases. Antinuclear antibody tests can be helpful in determining whether Raynaud’s is primary or secondary to an autoimmune rheumatic disease. ANA may be followed by additional tests that are considered subsets of the general ANA test and that are used in conjunction with the person's clinical history to help rule out a diagnosis of other autoimmune disorders.

A negative ANA is usually reassuring.

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Doc Spot Please can you tell me about bosentan? I have read about it on the internet but my consultant says it is still on trial.

Bosentan is one of several drugs that block a very specific protein called endothelin.

Endothelin may cause some of the blood vessel damage that complicates scleroderma to cause pulmonary hypertension in the lungs or ulcers on the fingers. Bosentan is used to treat some cases of scleroderma but is a very specialised treatment that should only be prescribed in appropriate cases. Another similar drug for pulmonary hypertension is called ambrisentan and there are other related drugs under trial at the moment.

I am taking mycophenolate and have enlarged lymph nodes in my stomach that need a biopsy. Is this common when on this medication?

Enlarged lymph nodes can occur for many reasons that include infections and inflammatory diseases. Often the only way to know why there is enlargement is to take a biopsy so that the tissue can be examined under a microscope or sent for culture of any infection.

Within a couple of hours of my first iloprost treatment my joints started to ache really badly and my hands and feet became painfully stiff and swollen. It was suggested I stop the treatment for a couple of hours and after that I felt a little better but within an hour of being plugged in again my discomfort returned. It was jointly decided that iloprost was not for me but two days later I am still suffering the ill effects of it.

Why is this?

Iloprost is the medical form of a natural substance called prostacyclin and although it often causes side effects during administration (usually via a ‘drip’) these are very rarely persistent as the iloprost only stays in the body for a few minutes once it is no longer being administered. If there is a possibility that iloprost makes symptoms worse then it is certainly sensible not to have repeated treatments.

Is skin peeling on the hands a consequence of Raynaud’s? I developed a severe flare up of autoimmune disorder(s) almost overnight. Part of my diagnosis is rheumatoid arthritis with Raynaud's

phenomenon. My consultant thinks I have another disorder too. Is this picture a clue to what it might be or could this symptom be due to Raynaud's? When I was really ill I had such severe muscle weakness that I could not lift my arms or get up from a seated position. I also had severe joint pains, muscle pains, trigger fingers and a flu like illness. I am desperate to know what is wrong.

NURSE ADVICE LINES Sally Reddecliffe & Adele Gallimore (For Pulmonary Hypertension Enquiries)

Specialist Nurses

020 7830 2326

Royal Free These symptoms are suggestive of a connective tissue disease called dermatomyositis that leads to rashes and muscle weakness and can be associated with arthritis. The diagnosis can be confirmed by standard tests and you should seek advice from your doctor and probably see a specialist.

Sue Brown

01225 428 823

Bath Liz Wragg

0161 206 0192

Manchester

How will I know if I have scleroderma? I have looked at the list of symptoms of CREST and have four of the five listed. I was diagnosed with Raynaud's last year after my hands started to ulcerate. My hospital visit to see the rheumatologist lasted two minutes and I never received a follow-up appointment.

Specialist Nurse

Scleroderma can usually be diagnosed by clinical assessment but it is usually necessary to perform specific blood tests and other assessments. Sometimes Raynaud’s changes or progresses and if you have developed ulcers on your fingers this certainly warrants a re-assessment.

Newcastle upon Tyne

I first developed Raynaud's about a year ago, and it affected all my fingers. In recent weeks it has changed and is affecting only the tip of the index finger on my left hand.

Does this mean the Raynaud’s is getting better?

Raynaud’s symptoms often vary in severity and this is especially the case in primary Raynaud’s or if you take treatments that improve the condition. Improvement like you describe is generally reassuring.

Jan Lamb

Pins and needles can be associated with Raynaud’s due to reduced blood supply to nerves in the affected part. Treatment for Raynaud’s may be helpful but if not you need to have further assessment to provide an explanation.

0151 529 3034

Liverpool Karen Walker

0191 223 1503

Audrey Hamilton Belfast

02890 561 310

Paula White & Julie Ingall Portsmouth

02392 286 935

Jayne McDermott Sheffield

0114 2711 627

Lucy Pigram Brompton

077588 943 175

(For Lung Enquiries)

Steve McSwiggan Dundee

I seem to have constant pins and needles.

What can I do to stop it?

0113 3923 035

Leeds

01382 633 957

(Only available Mon, Tues & Wed mornings)

The nurse advice lines are not a replacement for care by your GP but very often it helps to talk to a nurse who can listen and offer advice. The nurses who run the advice lines also have very busy schedules and therefore on occasions you will get an answerphone to leave your details for the nurse to get back to you.

Publication costs for this issue of Hot News is supported by GSK. GSK have no editorial input or review of the content of this newsletter and the opinions expressed are the opinions of the RSA and/or the individual authors and may not necessarily reflect the opinions of GSK.

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020 7472 6354

Royal Free

Anne’s Challenge Map of the Course

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To date my sponsorship for this challenge has reached £13,000 My justgiving page is www.justgiving.com/rsapedalo

My Pedalo Challenge I am just overwhelmed and so grateful to everyone who has sponsored me for this event which takes place on 11th June on Lake Windermere.

As so many people have been helping to boost the Cool Million I wanted to do my bit. It seemed like a good idea at the time and then my husband and I went to the Lakes to finalise details for the event. The Lake looked huge and our meeting with Dennis, the Lake Warden proved to be most interesting and he was most helpful. He did however warn me of the dangers e.g. a passing ferry could send a big wave which could at worst capsize the pedalo, the wind could change direction, we could be hit by a passing speed boat ... and so it went on.

I said I could swim but he said the water could be very cold even in summer - so I said we would stay near to the shore. No, it didn’t put me off because a challenge is a challenge. I have spoken to Don Gilet and explained that he needs to be fit!

The training however is something else! Unfortunately an operation in March held my training back for a few weeks but I am now back in action - in all weathers. My husband asked why I had chosen a challenge in my 70th year which required the most training of all and also involved him, as I can’t do it on my own! The pedal exerciser donated by enjoy-able has been great for practising indoors and my heated gilet from EXO2 has been great for outside practise. Companies have been most supportive by providing equipment needed Windermere Lake Cruises have donated the use of a motor boat for the day to go alongside us just in case help is needed, Halfords have donated a cushioned seat and we have had great support from the rangers at Fell Foot National Trust Park. We will have a tow rope, buoyancy aids, a horn etc. and other essentials which I am still working on, like waterproof clothing in case it rains as there is no cover on the boat.

I have no idea how long the event will take but anticipate 5 - 6 hours depending on the weather and my legs! For members who have joined recently I thought you may be interested in my previous challenges.

These are as follows: In 2000 - I challenged Sharron Davies MBE and David Wilkie MBE to swim a mile; 2002 - I was Co-driver in a Subaru with British Rally driver Charlie Jukes, going around the Sweetlamb Rally Circuit in Wales; 2004 - I swam with sharks (not in a cage!) with David Wilkie; 2006 - I went down the Olympic Bob Run in St Moritz; 2008 - I took part in a simulated skydive with Nick Ross (from Crimewatch) and David Wilkie; 2010 - I took a team of Huskies across Finland in minus 32 degrees (in spite of having severe Raynaud's), and raised over £28,000 on this crazy venture. All these challenges totalled over £120,000 for research.

Any local members would be welcome at the finish!

Anne

FESCA - Second World Scleroderma Congress Anne Mawdsley and RSA Trustee, Beverley Myers, travelled to Madrid in February to attend the second World Scleroderma Congress and to represent the RSA at the FESCA (Federation of European Scleroderma Associations) networking meeting at the end of the Congress. It was a most informative few days. In all, 59 countries were represented by 261 people who attended the patient programme and 1,141 doctors for the medical programme. The speakers were excellent and gave very positive and informative presentations. Not only were consultants and health professionals speaking but there were also some very moving talks given by people who have scleroderma.

This photograph shows representatives from the European groups who were present at the Fesca meeting

Some of the presentations from the Congress can be viewed by logging into www.fesca-scleroderma.eu

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Lung Involvement The Lungs in Scleroderma Background The lung can be affected in two main ways in patients with systemic sclerosis (SSc): pulmonary fibrosis (inflammation in the lungs) or pulmonary hypertension (increased blood pressure in the arteries in the lungs). Patients can also develop other problems such as infections or a pulmonary embolus (clot in the lungs).

Systemic sclerosis, scleroderma, is a very heterogeneous disease which Dr Bridget Griffiths means that everyone is affected in a slightly different way; some people will have one symptom and others will have another and some will have a combination. We recognise some patterns for example diffuse cutaneous systemic sclerosis and limited cutaneous systemic sclerosis. Patients with diffuse cutaneous SSc will have skin thickening above and below the elbows and knees and sometimes affecting the abdomen and chest.

They are also more likely to develop pulmonary fibrosis and to have a particular antibody in their blood called Scl-70. This type of scleroderma affects approximately 30% patients. In patients with limited cutaneous SSc, their skin will only be thickened below the elbows and knees. They are more likely to develop pulmonary hypertension and more likely to have the anticentromere antibody.

However, not everyone with diffuse disease will get pulmonary fibrosis and not everyone with limited disease will get pulmonary hypertension but these are the things that we particularly monitor as we now have treatments for these problems.

Assessment It’s easiest to treat these problems if we detect them early, so approximately each year we will arrange lung function tests (breathing tests) to see if the lung volumes are decreasing and to measure how the oxygen is passing across the lining of the lungs. If the lung function tests are deteriorating, then we may arrange a CT scan of the lungs to look for lung fibrosis.

This test is more detailed and therefore more precise than a chest Xray – it takes several pictures at different levels within the lungs. We will also arrange an ECHO (heart ultrasound scan) to estimate the pressure in the arteries in the lung. If the pulmonary artery pressures appear to be rising, then you may be referred to your nearest Pulmonary Hypertension Service (e.g. in Newcastle, Sheffield, Cambridge or London) for further assessment. This might include a cardiac catheter test to accurately measure the pressures in the right side of the heart.

Pulmonary fibrosis This affects approximately 80% patients with diffuse SSc. The inflammation within the lungs stops the lungs from expanding as well as usual and so reduces the volumes. Sometimes this will not change but sometimes it becomes worse, often within the first few years of diagnosis. We measure the total lung capacity and the ability for oxygen to go across the lining of the lungs (the diffusion capacity). A patient with pulmonary fibrosis may have a cough or be short of breath on exertion compared with their friends the same age but sometimes patients do not have any symptoms and their chest sounds clear and their chest Xray is normal, hence the regular monitoring.

In the last ten years we have found through clinical trials that treatment with a drug called cyclophosphamide can help improve

08

symptoms in patients with moderate to severe pulmonary fibrosis.

Cyclophosphamide is a type of chemotherapy and has been used to treat cancers, but it has also been used by rheumatologists over the last thirty years to treat patients with severe inflammation in the kidneys, lungs, nerves and brain. It is a strong drug with potential side effects so has to be used and given by doctors and nurses who are trained and familiar with it. It can be given as tablets or a drip treatment into the vein. I prefer to use the drip form as it has been shown to cause fewer side effects and to be as effective. Before starting this treatment, a patient will be counselled about potential side effects and how to avoid/treat them.

In Newcastle, we feel that because not all patients have the same severity of disease, we need to tailor their treatment according to response. Everyone therefore gets six treatments (pulses) and then we repeat their lung function and CT scan to see if their lung function has improved, stayed the same or in fact deteriorated. If it has improved, then we will stop giving the infusions and either monitor or switch to a tablet called mycophenolate.

This treatment can also dampen down inflammation. If the results are much the same, then we continue with the pulses but at less frequent intervals and continue to monitor the lung function tests closely. If the tests are worse, then we can increase the dose and continue to give frequent infusions until the results hopefully start to stabilise. We are in the process of analysing our data but on average we have found that the lung function can stabilise or improve using this protocol. We have certainly found that we get the best results if we start treatment early.

Pulmonary arterial hypertension (PAH) PAH is present in nearly a quarter of patients with SSc. Patients may notice that they become more breathless on exertion. Previously there were no beneficial treatments for this condition but in the last ten years several drugs have become available e.g. sildenafil, bosentan, ambrisentan and iloprost. They are now approved for use in the UK as long as patients meet certain criteria, as assessed at the National Pulmonary Hypertension Centres. Clinical trials have shown these drugs to improve the distance a patient can walk in 6 minutes (the 6 minute walk test).

Finally It is important to remember other ways of looking after your lungs e.g. stopping smoking, doing exercise and watching your weight. If you become very breathless, then you may need portable oxygen or a concentrator fitted at home. Your doctors can assess your suitability for this. As a patient, you can also help to monitor your progress. Please report to your specialist/ nurse if you become more breathless. You may need earlier lung function tests and/or ECHO. We recommend that you have your annual flu jab. If you develop a chest infection and start to cough up green sputum, then you probably have a bacterial infection and will need antibiotics. It is best to start these promptly.

Summary The lungs can often be involved in patients with systemic sclerosis.

We now have treatments for pulmonary fibrosis and pulmonary hypertension so careful monitoring and early detection are the key.

The good news is that more drugs continue to be assessed, to determine their effectiveness in treating the lung complications seen in patients with systemic sclerosis.

This report was taken from a presentation at our Annual Conference 2011 by Dr Bridget Griffiths, Consultant Rheumatologist, Freeman Hospital, Newcastle upon Tyne

Self Management Scleroderma - Tips on Self Management The most important first tip is to find a competent specialist in the field and have no hesitation to contact them any time.

Scleroderma may have many subtle manifestations which need to be detected EARLY by relatively simple special techniques e.g. echocardiogram for diagnosing high blood pressure in the lungs and for which early treatment is available and effective. The first signs may Professor Alan Tyndall be just feeling a bit more “puffed” climbing stairs. Many doctors are rather nihilistic about scleroderma treatment, so don’t be fobbed- off with facile explanations.

Staying warm: Core body temperature is as important as extremity temperature, so thermal underwear, hats and socks, plus high quality gloves are critical. Thermal protection must be used BEFORE exposure to cold, otherwise a vicious cycle of blood vessel spasm and tissue damage is established. Gloves with built-in heating battery operated elements and a variety of heating aids for pockets are available.

Be careful that numb hands and feet are not burned by too hot devices! In addition, wet extremities with a wind chill factor will induce blood vessel spasm, even if the ambient temp is not low e.g. swimming areas in summer.

Examination rooms and especially operating theatres should be extra heated for scleroderma patients.

Staying mobile: Scleroderma patients may have an inflammation of the muscles called myositis, and this is often missed, since it is often not painful. There are blood tests Creatine Kinase (CK) and MRI examinations which confirm the diagnosis.

However, even without this infrequent muscle inflammation, fatigue, stiffness and weakness are common complaints in scleroderma. Stretching, weight training and supervised light toning exercises with blood oxygen monitoring are also anti-stress and very useful for both maintaining fitness and self esteem. Also, various hand exercise devices keep fingers mobile.

Staying infection free: Minor injuries to the extremities may result in poorly healing ulcers which do not respond to antibiotics quickly. Both problems are due to the reduced blood supply to the skin. Therefore protective gloves and shoes in the first instance, plus early local and systemic treatment once established are critical to avoid larger tissue loss. Also a persistent cough and malaise may herald a lung infection called pneumonia, which may be difficult to detect on a simple chest x-ray if some lung scarring is already present.

Staying nourished: Reflux of stomach contents into the food pipe and “lazy bowels” may lead to indigestion and poor food absorption.

Small frequent meals, elevation of the head of the bed by one brick’s height, plus antacids, help to reduce reflux.

Low fat diets and when indicated, bowel motility stimulants and antibiotics may reduce ‘bloating’ and improve bowel function.

Staying human and dignified: The humiliation of fecal incontinence, erectile failure or a painful tight dry vagina may be difficult subjects to raise with a doctor or even a partner, but should not be ignored in the face of more “dangerous” but easy to talk about problems such as blood pressure and lung scarring. Unless addressed, they may become more destructive to life quality than other issues. There are approaches to ease these problems (carrying extra pads and underpants, rotating antibiotic schedules, penile erectile implantable devices which are not excluded in scleroderma, vagina widening operations etc. and although beyond the scope of this article, are familiar to specialist clinics.

Staying “yourself”: Changing physical appearances, especially one’s face, may lead to a feeling of dissociation from self and identity loss. Selective plastic surgery e.g. removal of deposits of calcium or unsightly enlarged vessels on the skin (telangiectasias) may do a lot to improve selfesteem, but don’t forget that love and attraction is not based solely on appearances, despite popularistic clichés to the contrary. Think how you would react if somebody you loved was in a similar situation.

Staying moist: Dryness, especially skin and mucus membranes may be troublesome. Constant moisturising creams and artificial tears plus vaginal lubricants and bronchial mucus softening agents (biosolvon) may greatly reduce the suffering.

Final decisions are very personal but should only be made after the appropriate information and counselling is made available to the patient plus/minus partner.

Rapidly progressive dental caries may be the first sign of such a problem, and in some countries a letter from the specialist may enable medical insurance to also cover some dental bills.

Summary: Many of the above comments are personal opinions and most taught to me by my patients.

Smoking, both passive and active are to be strictly avoided and careful dental hygiene is essential.

Our thanks to Professor Tyndall, Head of the Department of Rheumatology, University of Basel, Switzerland, who kindly agreed to write this article, which was based on his talk given at the 2nd World Scleroderma Congress in Madrid.

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Digital Ulcers Digital Ulcer Questionnaire Results In our August 2011 volume of Hot News we asked members who have scleroderma and who presently have, or have ever had, a digital ulcer to fill in a short questionnaire about their experience. The questionnaire was sent out and completed during the summer months of July, August and September.

Whilst several members indicated that they did not usually suffer with ulcers during the summer, 48% of respondents still answered that they currently had a digital ulcer despite the warmer weather.

Other questions asked were as follows: • Where are the ulcers? (i.e. fingers or toes) • Do you dress the ulcers yourself? If no, who does?

• What dressings have you found to be helpful?

• Do you take any medication when you have an infected ulcer?

• Have you found any treatments to be helpful? If so, please state.

The questionnaire asked members to briefly describe how ulcers affect their quality of life on a daily basis. A sample of the most representative, clear and insightful comments have been chosen for a new handout on digital ulcers. A copy of the full handout is available on receipt of an SAE. Finally, the RSA used this opportunity to ask members which type of scleroderma they had diffuse (dcSSc) or limited (lcSSc). The response to this question gave some concern, since 22% were unsure about which type they had.

The complete figures obtained were as follows: 143 responses in total Currently have an ulcer

Total no. % of total 68

48%

111

77%

4

3%

Both fingers and toes

28

20%

Dressed by Health Professional

24

17%

119

83%

9

6%

Taken medication

87

61%

Found a helpful treatment?

77

54%

110

77%

31

22%

Fingers only Toes only

Self-dressed Self-dressed (occasional medical help)

Calcinosis Don’t know which type of SSc

As the figures show, the majority of respondents suffered ulcers in their fingers rather than toes and very few (3%) had ulcers on their toes only. In terms of patient care, only 17% of respondents had their ulcers dressed by a health professional (mostly district or practice nurses or podiatrists). Only one reported having their ulcers dressed regularly at a specialist clinic. Of the 83% who dressed their ulcers themselves, 9 respondents (6%) had their ulcers dressed occasionally by a nurse – usually if they were difficult to reach or very troublesome.

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The relationship between calcinosis and digital ulcers is evident as 77% of respondents reported that they had experienced both calcinosis and digital ulcers. In people with calcinosis, ulcers can be caused by calcium deposits breaking through the surface of the skin. These ulcers can be extremely painful and difficult to heal. Calcinosis tends to occur at pressure points, and so calcinosis-related ulcers can occur in other places besides the fingers and toes, for example the elbows and knees.

Only 54% of all respondents had found a treatment that they believed helped their ulcers. Of the 119 who self-dressed their ulcers, 65 (55%) had found a treatment they found to be useful. Interestingly, the percentage was lower in those who had their ulcers dressed by a health professional, with 12 (50%) of the 24 reporting they had found a helpful treatment.

Both figures indicate that around half of all respondents had found nothing to help them manage their ulcers.

Management The following treatments were frequently cited as being useful: Iloprost infusion, Fucidin Cream, Wax Bath, Nifedipine, Tildiem and Sildenafil.

Members’ tips for managing ulcers • I went to a hand clinic and they taught me how to massage E45 or cetraben emollient cream into my hands which helps the ligaments that tighten to become more supple. Making a fist is now easier.

• Lots of foam padding and bigger flat trainers. Using silver plasters, silver packing, keeping warm and dry and exercising to increase circulation has helped me to manage.

• Surgical debridement followed by application of Echinacea cream has helped my ulcers.

• I applied neat lavender oil to ulcers which hadn’t healed after 3 months. They then began healing. After a week I added tea tree oil and my skin soon healed.

• I avoid getting too cold and try to dress my fingers as soon as I feel any pain.

• Iloprost possibly helps to speed up healing but is no use for me long term. However, potassium permanganate, manuka honey dressing and semi-compression bandages seem to help.

• Savlon cream helps to numb my ulcers and reduces pain at night.

The following complementary treatments were also suggested: Manuka honey, ginkgo biloba, soaking in sterile, lukewarm saltwater and Youki spray. Others reported that wearing a finger splint had helped (an occupational therapist can advise on these).

The dressings most frequently used by those who dressed their own ulcers were: Mepore, Mepilex, Inadine, Mepitel, Duoderm and Elastoplast ‘silver healing’.

Our thanks to everyone who sent in their responses which will help to give the medical profession an idea of how digital ulcers really affect people on a daily basis.

If anyone would like a copy of the questionnaire results please send an SAE with your request.

Supporting Medics Students’ Examinations whether they turned white and blue and how often this happened. They were asked by the examiner what this could lead on to and to my delight, the majority of the students diagnosed scleroderma and some, when asked, what other organs can be involved said kidney, lungs and heart and oesophagus.

Melanie Bowen

I was called and asked by Dr Catherine Swales, Rheumatologist, whom I had met at one of my visits to the Nuffield Orthopaedic Centre for Iloprost, if I could take part in the exam finals as a patient and be examined by the medical students at the John Radcliffe Hospital - I very willingly agreed.

I was picked up at home by taxi at 7.15am and taken up to the John Radcliffe where I was led to a waiting area where I joined the rest of the patients, helpers and examiners. We were given a very nice breakfast and given badges. At about 8.30am we were asked to go to our rooms which we would be in for the duration of the day.

I was joined by Dr Anthony Bradlow, Consultant Rheumatologist at the Royal Berkshire who was one of the examiners for the morning session. We had a very brief chat and then at 8.45am the bell rang and the first student entered the room.

During the whole day I had over 55 students, mostly young women aged 23 and 24 who looked very nervous.

Each student entered the room, shook hands with the examiner and were then asked to examine my hands and face. They had four and a half minutes to give a diagnosis. I have small digital ulcers on 3 of my fingers tips and one on my little finger nailfold and other remnants of ulcers, plus areas of telangiectasia on my hands and fingers, so this all helped them to ask me if I ever got cold hands and

We had a break for lunch which lasted for 45 minutes and we reconvened for the afternoon session at 2pm. I was then joined by one of the examiners, Consultant Rheumatologist, Dr Ian Cheesebecker. We had a quick chat and then the bell rang and a student entered my room. A couple were very unsure of the diagnosis and one even diagnosed scabies. The examiner was not impressed and asked if he honestly thought they would allow a patient with scabies into the hospital!

To summarise, the students were polite, introduced themselves, washed their hands and asked if they could examine me. They said if I experienced any pain during the examination to let them know. A few told the examiner that they thought it was either lupus or scleroderma and when asked why it could be lupus, they said that I had flushed cheeks and neck. The examiner told them to have a closer look at my hands and face. This they did and then decided that it was scleroderma and that the flushing was actually telangiectasia.

At 5.30pm the bell rang and the exams were over. We were then offered a refreshing cup of coffee or tea with biscuits and headed home feeling weary but overwhelmed with the success of the day and that so many students had an awareness of Raynaud’s and scleroderma.

The above was written by member Melanie Bowen when she attended the clinical students' final examination for the University of Oxford degrees of Bachelor of Medicine and Bachelor of Surgery held at the John Radcliffe Hospital on Thursday 26th January 2012.

Six Minute Diagnosis After last year’s conference, the Rheumatology nurse at the local hospital contacted me to ask if I would be interested in assisting young doctors who were preparing for their consultancy exams.

After another call from a hospital doctor and someone from the Education Unit, I had a letter inviting me to attend the PACES Course.

There were about six patients in the waiting room, some in hospital gowns, awaiting chest examinations. I had no idea what to expect and waited with my cup of coffee to be called. Dr Singh informed me that I would be introduced as ‘Mrs Mellors who has a problem with her hands’.

When I was called into the lecture room and seated, a doctor, who had not yet assessed any of the other patients, introduced himself to me and began his assessment that had to be completed in six minutes. He was very efficient and asked all the appropriate questions - how did the condition affect me? How did I cope with it? What medication was I taking? When asked for a diagnosis he confidently announced CREST syndrome. The doctors were then asked the significance of the medications I was taking and then discussed it. In all, I suppose I was at the hospital for over two hours, but time soon passes when you are chatting to other people.

To my surprise, the following week I received a letter of thanks from the Education Unit and Dr Singh and a voucher for Marks & Spencer.

I thought you would be interested to know that there are some doctors coming up who are able to diagnose systemic sclerosis by recognising telangiectasia and by relevant questioning, all in six minutes!.

Angela Mellors

Annual Report & Accounts The audit for 2011 is in the process of being finailised. Once the accounts are available they can viewed either on our website or the Charity Commission’s website.

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Members’ Stories I Feel Like a New Woman 57 year old Susan Emery who has just seen the arrival of her first granddaughter, said: “I'm glad to be alive”.

Susan has two sons, Chris and Michael and a daughter, Shelley.

During the children’s early years she was a foster parent, a very rewarding job and one that she enjoyed immensely at the time.

Unfortunately due to Raynaud’s and scleroderma which was diagnosed 33 years ago, Susan has had to have six of her fingers amputated and both of her legs below the knee – but she said she feels like a new woman. The four months she spent in hospital before the operation were, she recalls, “some of the darkest in my life”.

Susan Emery

But her life is now on the up. “When it started I had to avoid getting my fingers cold or they would turn blue because of the lack of blood circulation. There would be this stinging soreness, much more than pins and needles, as the feeling gradually returned. I had to be careful going out in the cold and even putting my hand in the fridge” said Susan.

In 2000, her condition took a serious turn for the worse. The stress of her marriage break-up and the loss of a job she loved, caused ulcers to develop on her fingers which would not heal and turned gangrenous.

Susan said: “Between 2001 and 2005, I had about 12 operations as I lost bits of my fingers. I was given drugs, to try to open up the blood vessels, but within a few years my toes had started to go, too. It was a very dark time. I was on morphine for the pain and I felt as if I was in a thick forest.”

By December 2008, Susan had to be admitted to hospital where, for the first four months, she was too poorly to undergo an operation. She ended up with blood poisoning and her family begged doctors to do something. They operated, and both her legs were amputated below the knee. She woke up a different person. It was brilliant, and everyone was flabbergasted. Susan said “It felt like a little miracle. I wasn't upset at all about losing my legs. I just felt happy to be alive and I felt like a new woman”.

Eight weeks later, Susan was up and walking on artificial legs. She said: “My stumps are beautiful and I have never had any trouble with them”. Susan, who lives near her daughter Shelley and one-month-old granddaughter Freya, said: "I have appointments every three months at the Queen's Medical Centre, in Nottingham to open up my blood vessels and lots of students come and see me as they don't get to see many very rare cases like mine.

“I don't work any more but I have an electric wheelchair and am getting on with life. I like sewing and gardening and I'd love to think about driving again.”

The above was extracted from an article written by Cathy Buss which appeared in the Leicester Mercury, February 2012.

Thank you Susan for sharing this with us. You are a very brave lady and also an inspiration to others.

Nurse Hit by Rare Disease Even though she was an experienced nursing sister, Kate Owen had never heard of the rare disease which robbed her of the career she loved.

Kate, 44, of Old Colwyn had a responsible post in the intensive care unit at Ysbyty Glan Clwyd in Bodelwyddan, but can no longer work there after being diagnosed with a debilitating condition called scleroderma, which affects the use of her hands. It also leaves her feeling exhausted and unable to stand for long periods of time.

“I loved nursing and hoped for a long career, but decided I would have to retire from it when I found I was dropping things and no longer had the energy for the long hours on my feet it requires,” she explained. Kate was fit and healthy until attending a bonfire

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night party in 2007 when she noticed that even though she was wearing thick gloves her hands were very cold and she had lost feeling in them.

“It was some time before I regained feeling in them, and my first thought was that I probably had a condition called Raynaud’s which affects the circulation. At first I did nothing about it, but I had other occasions when I lost circulation to my hands, and I went to see my GP”.

Her doctor also thought it might be Raynaud’s, but blood tests revealed it was in fact the much rarer and more serious scleroderma. She was referred to a consultant rheumatologist at Ysbyty Glan Clwyd who started her on a course of steroids. “It was a shock to know I could lose much of the use of my hands, but I was able to continue

working until March 2009 when it became obvious I couldn’t continue,” she said.

Kate has developed recurring ulcers on her fingers and fears her lungs are also affected, but she tries to keep active doing voluntary work.

The above was taken from an article

by Eva Ketley, which appeared in the North Wales Weekly News.

Kate has taken a positive approach to her condition and has been helping to create awareness for the RSA by giving talks to health professionals.

Case Studies Do you have a story you could share with our readers? If so we would be pleased to hear from you.

Members’ Stories The Issues of Raynaud’s in the Hands and Feet are Real Joining the Raynaud’s & Scleroderma Association has been great because someone actually recognises that what you are describing is something very real and it’s not all in the mind!

I have had cold hands and feet in the winter for years and about 15 years ago, was told by my GP that it was Raynaud’s although no test was ever done. The cold has always had an unbearable effect on my hands and feet and Kate Norbury not just due to the weather but simply going into the freezer, the refrigerated aisle in the supermarket or air conditioning.

Two years ago in the summer I was conscious of my left foot feeling numb, which I felt was affecting my balance. I checked with my GP that it was nothing to do with a middle ear infection etc. It wasn’t. For no apparent reason, it continued to get worse. I also developed very hot facial flushes which once started would often last for hours.

Various consultants have been investigating with numerous scans and tests for peripheral neuropathy, without any positive results, although the very comprehensive blood test did reveal a positive for ANA which didn’t engender any comment.

I need to add that I have an underactive thyroid, and have taken thyroxine for the last 10-12 years. I have just started a course of nifedipine 5mg. three times a day without any noticeable effect as yet.

The pins and needles and numb feeling is with me permanently day and night in both feet and the facial flushes continue. I only suffer from cold fingers in cold temperatures and they go very white, taking a long time to recover. My feet are made worse by standing in one position for more than 10 minutes. The flushes, I have realised, occur when I am under stress, although I have always thrived in such situations. For example I have been a public speaker and chaired committees all my life. Because of this I am no longer as much in control or enjoy these things as I used to.

Having what feels to me to be a face positively on fire and two numb feet is very inhibiting when standing on a platform trying to look totally relaxed and in charge! I spend most evenings with my feet resting on a hot water bottle or rubbing them. It doesn’t make much difference but at least gives me a different sensation to the numb ‘pain’.

I have always resisted the reference to age but even at 79 years of age I don’t say it’s time to give up the things I love doing, like raising money for good causes and helping people to feel more positive about themselves and their skills. I am a very health conscious individual, I am not overweight, don’t have high blood pressure and with a background in nutrition I have a very healthy diet, do pilates and walk a lot.

The issues of my hands, feet and face are real, they are not in my mind and it would seem that in the RSA there is someone out there who agrees and is doing something about it. So all power to your elbow and any help I can give I will.

Kate Norbury

Social Media - How Does It Work?

Over the last year, we have had a fantastic response on our social media sites, HealthUnlocked, Twitter and Facebook.

The three sites all work differently and offer varying levels of support for our members, depending on whether they just want to keep up to date with what the RSA is doing or whether they want to become more involved and interact with other people who may have similar health concerns.

HealthUnlocked is designed with patients and carers in mind and can be a useful starting point for any medical queries you might have. Each member has different experiences of Raynaud’s and/or scleroderma and sharing them can be helpful in many ways – for research and information as well as for support. On the site, which is free to use, you can review hospitals, take part in polls (set by the RSA), write a blog about your experience of Raynaud’s/scleroderma and ask other users questions about their experiences with the two conditions. The site is very busy, with almost 900 users asking questions on a daily basis.

Facebook and Twitter are also useful for supporting the work of the RSA and raising awareness. Our followers are kept up to date with awareness campaigns, member stories or advice and other users can use the page to gather support for fundraising events. Although not as interactive as

HealthUnlocked, members have made contact with each other and found answers to some questions about their health on Facebook and Twitter.

These sites are playing an increasingly important role in patient understanding and can be a great source of advice, experience and support. However, the information shared on these sites must not replace the relationship you have with your GP or consultant and we advise you to exercise caution when seeking advice from others. Don’t forget, the RSA is always at the other end of the phone if you need to chat!

To keep up to date with the RSA, follow us on Twitter (@raynaudsuk) or visit our Facebook page, www.facebook.com/raynaudsandsclerodermaassociation

Our HealthUnlocked site can be found at http://raynauds.healthunlocked.com If you have any queries about any of these sites, contact Hannah at the RSA on 01270 872776 or email hannah@raynauds.org.uk

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News Skin Camouflage cover cream

We have been contacted by Changing Faces, the leading not-for-profit organisation which supports people with conditions that affect their appearance, to inform us that they have now taken over from The British Red Cross in running the Skin Camouflage Service. This service helps people with skin conditions to build their confidence and independence and for many it can make the difference between staying behind closed doors or facing the world, getting a job, making friends or going to school.

The Skin Camouflage Service was established in 1975 within the British Red Cross and since then trained volunteers have provided the service in hospitals, GP surgeries and health clinics. It is provided for people who have skin conditions, such as telangiectasia and every year more than 6,000 people across the UK use the service for various reasons.

The current Red Cross service volunteers have now transferred their skills and experience to Changing Faces, ensuring people will continue to receive the same high quality skin camouflage advice they need. Most individuals are referred to the service by their dermatologist and, as public awareness of the service is growing, more people are accessing the service directly themselves. The camouflage practitioner will spend an hour carefully matching skin type with the right products and educating their client on how to apply them. The specialist waterproof products will then be prescribed on a regular basis by a GP. This may be of interest to members who have visual telangiectasia on their faces. It really is worth the time and effort to try this as it can certainly help with self esteem and only takes a few minutes to apply.

20%

discount off all orders placed via internet or telephone using code VEILJUN12

More information may be obtained from the Changing Faces website www.changingfaces.org.uk/Skin-Camouflage or by telephoning 0300 012 0276.

Have you considered including the RSA in your Will?

It is a common myth that only the rich and famous leave money to charity when they die. This couldn't be further from the truth. The reality is that without the gifts left in Wills by supporters of all financial circumstances, many of the charities we know and support today wouldn't even exist. Often when people begin to make a list of their assets (for example, house, car, savings and shares) they are surprised at how much they actually have.

The RSA receives no government funding so legacies are hugely important to us. Approximately 17% of the RSA’s donated income in 2011 was from legacies. Without such contributions, we would not be able to fund and extend our vital research and welfare projects. Without the legacies that have been left to the RSA over the last thirty years, the many research projects that have been funded could not have taken place.

According to surveys, 74% of the UK population support charities during their lives and when asked, 35% of people say they'd happily leave a gift in their Will once family and friends had been provided for. Despite this, unfortunately only 7% actually get around to including their chosen charity in their Will. The good news, however, is that it has been estimated that just a 4% change in the number of people who include a charity in their will would generate an addition £1 billion for charities in the UK every year.

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If you are considering including the RSA in your Will, we can supply information to help you. It is very easy to include a charity in your Will, but we recommend that you consult a solicitor to ensure your intentions are clear. Even if you have already written your Will, a solicitor can easily draft an addendum, called a Codicil, which is not expensive to do.

Your support could really make a difference to people living with Raynaud’s and scleroderma. The legacies which we have received to date have already helped to support vital research into these conditions.

For more information, download our leaflet ‘Where there’s a Will, there’s a way’ from www.raynauds.org.uk/leaflets.

Alternatively, contact Head Office on 01270 872776 or email info@raynauds.org.uk to request one.

Fundraising Coffee Evening Raises £535

10K run in Hertfordshire

Burton upon Trent Support Group

Sylvette Wood who raised £108 by taking part in a local 10K run in Hertfordshire.

Our thanks to Rena Forest and friends who enjoyed a charity coffee evening with entertainment at Calderhead Erskin Parish Church, raising £535.

Fun Evening raises £500

Going for Gold!

Member Barbara Buchan has raised almost £16,000 by selling second hand bits and pieces of jewellery sent to her by members.

Barbara goes to various craft fairs to sell her wares for the RSA. If any members have any jewellery they no longer use or even broken pieces, please send them direct to Barbara Buchan, 6 Meadlowlands, Woolpit, Bury St Edmunds, Suffolk IP30 9SE Please enclose an SAE for Barbara to acknowledge receipt of packages (or a telephone number), and please do check that the correct postage applies.

We received a cheque for £500 from Shirley and John Lynch (pictured above), who held a ‘fun evening’ to help raise money for the ‘Cool Million Appeal’. The event was held at Pershore Football Club, where Jonathan Small gave a most inspiring talk about his sponsored climb up a couple of volcanoes in Equador to raise funds for the RSA. Jonathan has offered to give talks to other groups if interested. His talk was followed by a jacket potato supper and a game of ‘killer’ skittles, which was won by one of the group’s helpers, Brenda Preston.

Their next event will be the Pershore Plum Fair on August Bank Holiday Monday.

Used Stamps

We have recently received several packages of used stamps from members.

Unfortunately, due to insufficient postage, it has cost the RSA a significant amount for these to be delivered as this includes not only the additional postage but also £1.00 Royal Mail administration costs.

Whilst we are most grateful we would appreciate if you could check and use the appropriate postage for your package.

Gift Aid It! If you are a UK taxpayer, please make sure that you or your friends and relatives let us know on all donations, as this can make a huge difference and doesn’t involve you in any work or cost.

Thank you very much Barbara for all your efforts on our behalf.

Donations

We have received an enormous amount of donations from individuals, Charitable Trusts companies, and voluntary organisations over the past few months and there simply isn’t enough space to list them all. However, everyone will have been thanked and we really are most appreciative.

In today’s economic climate we are doing reasonably well but still need to raise a further £400,000 by the end of this year to reach our ‘Cool Million’ goal.

Thank you all for your support!

Pedal Exerciser This exerciser has been essential in my training for the pedalo challenge. It has helped to strengthen my legs! My thanks to enjoy-able who not only supplied me with this piece of equipment but also donated one to my co-pedaller, actor Don Gilet.

£29.99 plus £6.49 P&P

For details visit www.enjoy-able.co.uk or call 01923 282430

Anne Mawdsley was invited to speak at the 6th Annual General Meeting of the Burton on Trent Support Group on 29th February.

Following her presentation she was presented with a cheque for £1,000 from Gordon Shelby and Helen Nutland, on behalf of the group which will go towards the Cool Million Appeal. The meeting was well attended by members and patients who attend The Queen’s Hospital in Burton. Anne said “I am very impressed by the enthusiasm of this group and can’t thank them enough for their efforts both in raising funds and for the support which they give on a regular basis to people with these conditions”.

For information about the Burton Group call Helen Nutland on 01283 566333

Give As You Earn

Why sign up to Payroll Giving?

Payroll Giving allows you to give to charity direct from your salary before tax is deducted. This means if you give £10 a month to the RSA and are a basic rate taxpayer, the gift will only cost you £8 from your net pay. If you pay a higher rate of tax, £10 a month will only cost you £6 from your net pay but in each case, the RSA would still receive £10. Payroll Giving is easy to set up and you can change your donation at any time by informing your HR department at work.

MissionFish We are most appreciative for donations made to the RSA from eBay sellers. Every time an item is sold, the seller can donate a percentage of the final sale price to our charity via MissionFish.

What are the benefits?

Adds credibility and visibility to your listings. All eBay for Charity items receive the charity ribbon.

Make your money go further - Select Gift Aid and the taxman will add 25% to your donation. eBay will also credit back your basic insertion and final value fee (equal to the percentage you donate).

Keep it simple - They do the work for you.

You choose our charity and the % you want to give, then MissionFish do the rest.

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NIGGLES CORNER

TRAVEL INSURANCE

Trying to do up zips

The following travel companies have been recommended to us by members.

If you have any others or have had a good or bad experience in getting travel insurance please let us know.

Coins in supermarket trolleys Changing toilet rolls with spring fittings Gripping a toothbrush Retractable tapemeasures - they

Burton on Trent, Helen Nutland

01283 566333 Ext. 5032 Bristol, Margaret Goff

01454 310225 South Cumbria/ Ruth Randall North Lancs 01524 903493

All Clear

0845 250 5200

Club Direct

0800 083 2466

Freedom Travel

01223 454290

Free Spirit

0845 230 5000

Knaresborough, Tony Overend

Go Travel

0870 152 5840

Manchester, Gill Holden

Insurance Choice

0844 5577 703

Insure and Go

0844 888 2787

It’s So Easy

0844 357 1315

spring back and catch your fingers!

Picking up dropped credit cards

LOCAL CONTACTS

Eastleigh, Kathy Allen

02380 610678 Fife, Rose Bevan

Taking seals off fruit juice and milk containers

01382 552272 01423 862551

Small key pads on mobile phones Cold steering wheel Metal seats at bus stops and stations Difficult lever on Dyson cleaner Pull tops on plastic squash bottles Trying to put stamps onto envelopes Undoing food packaging

Please keep sending us your niggles

01942 877259 Merseyside, Helen Lingwood

0151 280 1194 07751 333 633 Email: jonandhel@sky.com

Orbis Services

0845 338 1638

North East, Jessie Pickering

Post Office

0800 169 9999

N. Ireland, Patience Bradley

Saga Travel

0800 015 8055

Text Giving Did you know that you can donate by text?

All one needs to do in order to donate to the RSA via a mobile phone is to text COOL22 followed by the amount that you want to donate and send it to 70070. For example, if you wanted to donate £5 you would text COOL22 £5 to 70070. The donor will receive a text message receipt and the chance to add Gift Aid if a UK taxpayer. Just imagine the impact if the 10 million people in the UK with Raynaud’s each donated £1! Please encourage your friends to donate in this way.

01388 527840 02890 592370 Mobile: 07966 416553 North Wales, Kate Owen

01492 515834 Oxford, Melanie Bowen

01865 515067 Portsmouth and Hampshire, Alison Wright

02392 367960 & 07986 900262 Rickmansworth, Marilyn York

01923 284935 Shipley, W Yorks, Bev Myers

The RSA Team

07903 448038 Suffolk & North Essex, Jacky March

01394 286637 Surrey, Fay Collings

01737 762005 Worcestershire, Shirley Lynch

01386 553392

Contacts Wanted

Congratulations to Hannah on her marriage to Martin Old on 31st March

From left to right: Karen Littley, Helen Teal, Fay Hayward, Anne Mawdsley, Hannah Old and Lucy Meek

Mrs Kingston would like to be in touch with a local member in Pembrokeshire.

Mrs O’Loughlin from Windermere would like to hear from any other members who have scleroderma and diabetes. She is having great problems using needles on a daily basis.

Several mums of young children have been in touch and would like to share ideas.

Do get in touch via the RSA if you can help.

PUBLISHED BY THE RAYNAUD’S & SCLERODERMA ASSOCIATION

112 Crewe Road, Alsager, Cheshire ST7 2JA Tel: 01270 872776 Fax: 01270 883556 Email: info@raynauds.org.uk Websites: www.raynauds.org.uk www.scleroderma.org.uk EDITOR: Anne H Mawdsley MBE Charity Reg. No. 326306

Raynaud's & Scleroderma Association © Copyright 2012. All Rights Reserved.