News

ONLY nine places remain if you want to take part in this Year’s BUPA London 10,000 on Sunday May 27^th.

You will have a one-off opportunity to experience the Olympic course before the official athletes do and take in the sights of Buckingham Palace, Big Ben, and Westminster Abbey, to name but a few.

But if running is not your thing, then why not support our runners in a different way? 

We have put together a corporate letter (see below) for you to send out to businesses in your area which asks them to pledge support to The Trust. 

The letter tells the story of Nik Kontoyannis, the son of H R Trust founder Paul Kontoyannis, but if you want to adapt it to your own personal story, then please do so. 

In order to achieve our goals of continuing to fund research and supporting patients and their families, we need to continue to raise as much money as we can so that together we can find a cure!

To use, please copy and paste: 

     Today’s Date

Dear 

Nikolas Kontoyannis was just eight weeks old when he was diagnosed with Langerhan’s Cell Histiocytosis - a cancer-like disease which travels through the blood stream causing damage to healthy parts of the body. The possibility of death was very real and his parents were told to prepare themselves. However, against the odds and thanks to the dedication of his doctor and parents, Nikolas survived. Others have been less fortunate. Since then Nikolas’s family have devoted themselves to finding a cure for both Langerhan’s Cell Histiocytosis (LCH) and Haemophagocytic Lymphohistiocytosis (HLH), another Histiocytosis disease. 

Nikolas’s diagnosis prompted the formation of The Histiocytosis Research Trust in 1991. The Trust’s main aims are to reach out to parents and patients seeking support and advice, to fund international research projects and host Parent and Patient Road Shows. For further information, please visit our website www.hrtrust.org

Since Histiocytosis is classed as too rare to be allocated any funding from either the Government or pharmaceutical companies, we are reliant on the donations and fundraising efforts of generous individuals and families affected by the diseases. 

On Sunday May 27^th, some of our families whose children have been affected by LCH and HLH will be taking part in the BUPA London 10,000 and will become part of Olympic history as they experience the Olympic course before the official athletes do. Further information can be obtained from www.london10000.co.uk

I am writing to ask you to pledge your support to them so that we can become even closer to achieving our goal of finding a cure. Your gift will help provide a brighter future for the many thousands of children and adults who are battling these illnesses each day. Their lives depend on it!  Every child and adult with either LCH or HLH is our child and part of our family. We want to give them a fighting chance and a promise of tomorrow. So please give generously. 

To donate, please send your cheque to The Histiocytosis Research Trust, P O Box 435, Leeds, LS17 1GE or you can donate on-line at http://uk.virginmoneygiving.com/team/NikolasKontoyannisYour donation will be gratefully received. 

I would personally like to thank you for taking the time to read this letter and for your support.   

Yours sincerely,

CHAMPION fundraiser Paul Bennett has been picked to carry the Olympic Torch.

Paul was nominated by his employer Sytner Maidenead to be considered for nomination by BMW UK for the London 2012 Olympic Torch Relay Torchbearers. He will now be one of 8,000 Torchbearers taking part and is expected to carry the flame between Bristol and Cheltenham in May.

Paul said: “It is fantastic but a bit overwhelming. Although, I am very proud!”

Dee Soufi of Sytner Sunningdale and Maidenhead, explained: "We picked Paul due to his constant work and dedication to charity since the unfortunate death of his son Declan. He and Sherrie seem to be constantly on the go with fetes, BBQs, marathons etc and they also encourage us as a company to help and get involved. He is a hard working, dedicated Manager and asset to the team here at Maidenhead and who also supports the business 100% with hard work and dedication. Couldn’t think or pick anyone better!"

Paul and his wife Sherrie lost four-year-old Declan to HLH just over two years ago and have devoted their lives to raising money for The Trust ever since through their fundraising group Shining On.

They have held numerous events and taken part in a variety of sponsored activities.

PLACES are still available for this year's BUPA London 10,.000 on Sunday May 27th. You will have a one-off opportunity to experience the Olympic course before the official athletes do.

By entering you can help us achieve our goals of funding scientific research into uncovering not only the causes of Langerhan’s Cell Hisitocytosis and Haemophagocytic Lymphhistiocytosis but also ensuring early diagnosis, effective treatment and a cure.

Buckingham Palace acts as the backdrop to the start and finish of the run and your route will take in some of London’s most famous sights including Big Ben, Westminster Abbey,St Paul’s Cathedral and The London Eye before finishing at Nelson’s Column in Trafalgar Square. 

In return for your guaranteed place, we ask that you raise a minimum of £350. We will give you a free H R Trust T-shirt/vest while the organisers will give you a special tag which can be attached to your shoe to record your time. You will also receive a souvenir kit bag.

To sign-up, we need a deposit of £50. For further information, please do not hesitate to contact Shelley on 07850 740241 or via email Shelley.ross@hrtrust.org.

(L-R) Sinead Healy, Sarah McCormack, Caroline Hird, Dr Kevin Windebank, Dr Venetia Bigley, Dr Vasanta Nanduri and Professor Matt Collin

PARENTS and relatives headed to the Leeds General Infirmary recently to hear the UK’s leading experts talk about LCH, latest trials and research projects.

Twenty-seven people attended The H R Trust LCH Parent and Patient Road Show and met with experts Drs Kevin Windebank, Vasanta Nanduri, Johann Visser and Professors Matt Collin and Peter Beverley.

Dr Nanduri, a consultant paediatrician at Watford General Hospital, introduced the evening by explaining about histiocytes and Histiocytosis and the differences between Langerhan’s Cell Histiocytosis and Haemophagocytic Lymphohistiocytosis.

Senior Lecturer in Adolescent and Child Heath, Dr Kevin Windebank, talked about Langerhan’s cells and dendritic cells and spoke about the types of symptoms experienced by some of his patients.

He gave a detailed explanation about how LCH can affect the body and where it effects.

Professor Matt Collin, Consultant Haematologist at the Freeman Hospital, Newcastle-upon-Tyne, advised that adults suffered quite similar symptoms to children when struck with LCH.

However, they were not able to gain the same access to specialists as children. He explained: “There is a long road to travel in terms of getting access to correct adult specialists.  We need new guidelines to manage the disease and have it more co-ordinated.”

Professor Collin added that at his clinic in Newcastle there is access to a haematologist, a dermatologist and an expert in chest medicine.

He then went on to talk about his latest research project, Cell Autonomous Mechanisms in the Pathogenesis of Langerhan’s Cell Histiocytosis, which is being funded by The Trust.

This project will incorporate the findings of Dr Barrett Rollins which found that over half (57%) of the LCH biopsies examined by his team of scientists had contained a specific genetic change in BRAF called V600E which has also been seen in melanoma, colorectal cancer and thyroid cancers.

Together with Professor Frederick Geissman, of King’s College, London, Professor Collin, is hopeful that new diagnostic and prognostic tests and therapeutic targets for LCH will be established.

 “Our research might even lead to a blood test to screen for LCH. We are very grateful to have this finding and hope to take it further,” he added. 

Dr Vasanta Nanduri spoke about Late Effects and stressed that it was very important that LCH patients did not smoke as this would make their condition worse.

Dr Johann Visser talked about how clinical trials work, the new LCH IV Trial and the  International Rare Histiocytic Disorder Registry.

He advised that clinical trials take a long time to devise and have to be better than the best known treatment on offer before they can go ahead.

LCH IV will be a comprehensive study that will address different aspects of childhood LCH and investigate Late Effects, including the neurological complications that affect some patients.

Dr Visser explained that it would take six years to recruit the 600 patients taking part in the study and involve 23 countries including Germany, the USA and Canada. The study would take a minimum of 10 years to complete.

He added that the aim of the International Rare Histiocytic Disorder Registry is to improve knowledge regarding the natural history, treatment and outcome of children and adults with rare histiocytoses.

Professor Peter Beverley, vice-President of The H R Trust, explained that LCH was called an “orphan” disease because it was so rare and most doctors do not come across it.

He advised that because of the rarity of Histiocytosis, funding was not provided by either the Government or the General Medical Council and therefore the Trust was reliant on donations and fundraising.  

Funds were currently donated by The Artemis Association, Greece, LCH Belgium and from events organised by familes and friends of those affected by LCH and HLH.

Feedback from the Road Show was very positive. Sarah McCormack, who travelled from Ireland, said: “I found the Road Show very informative, helpful and interesting.

“I want to do whatever I can to help the Trust to continue with its amazing work. Overall, I am so glad I came over and it is so reassuring to see such dedicated people involved with LCH.”

The next Road Show will be held in 2012 – the location will be announced in due course.

CIRCLE of Friend's member Sally Scullion has designed and created a wonderful selection of hand-crafted bracelets especially for The H R Trust with all proceeds raised going to the Trust. Our new Christmas cards are also still available, so do check out both items under the Shopping and Recycling section of the website. The bracelets can be found under Clothing and Accessories. 

THE Parent and Patient Road Show on Tuesday December 13^th takes palce in the Littlewood Hall, Leeds General Infirmary, Great George Street, Leeds, LS1 3EX. Please note the Littlewood Hall is based in the old part of the hospital and can be accessed via Great George Street. After entering, please make your way towards the stairase and at the top is the Post-Graduate Centre. The Littlewood Hall and Garland Gallery (where refreshments will be served from 545pm) can be accessed from here.

The nearest car park is The Light, The Headrow, Leeds, LS1 8TL. It can be accessed via Dudley Way off Great George Street on the Leeds City Loop. The hospital is just a few minutes walk away. If you are travelling by train, you will need to take a taxi. 

Should you need to get in touch on the day, please call 07850 740241

IT is with great sadness that we announce the death of Dr Ralph Steinman, a member of The H R Trust’s Scientific Review Board, Nikolas Symposium Steering Committee and 2011 Nobel Prize recipient. 

Canadian-born Professor Steinman was a member of the Scientific Review Board between 2004 and 2011 and played an extremely active role in reviewing the applications made to conduct research into LCH and HLH. His advice and recommendations were immeasurable. 

Despite being unwell during the calls made in 2008 and 2010, Dr Steinman continued to provide perceptive criticisms and greatly helped the SRB to determine which application would be awarded funding. 

He was also a valued member of the Nikolas Symposium Steering Committee and his guidance and support will be deeply missed. 

On Monday October 3^rd2011, Dr Steinman was awarded the Nobel Prize in Physiology or Medicine, just days after his death from pancreatic cancer. He received it "for his discovery of the dendritic cell and its role in adaptive immunity". 

In 1973, Dr Steinman discovered “dendritic cells” which play a critical role in activating the body’s adaptive immune system. His subsequent research led to a new understanding of how they function and laid the foundation for numerous discoveries in the critically important field of immunology. It also led to innovative new approaches in how cancer, infectious diseases and disorders of the immune system are treated. 

The Nobel Assembly at the Karolinska Institute said Dr Steinman had revolutionized the understanding of the immune system by discovering key principles for its activation and had discovered the dendritic cells of the immune system and their unique capacity to activate and regulate adaptive immunity, the later stage of the immune response during which micro-organisms are cleared from the body.

The importance of Dr Steinman’s work was marked by an array of awards throughout his career. He was the recipient of the A.H. Heineken Prize for Medicine in 2010, the Albany Medical Center Prize in Medicine and Biomedical Research in 2009 and the Albert Lasker Basic Medical Research Award in 2007. He received the Debrecen Prize in Molecular Medicine in 2006, the New York City Mayor’s Award for Excellence in Science and Technology in 2004, the Novartis Prize in Immunology in 2004 and the Gairdner Foundation International Award in 2003. He was a member of the National Academy of Sciences and the Institute of Medicine.

H R Trust President Paul Kontoyannis said: “We express our condolences and sincere gratitude to Ralph’s family for his steadfast dedication to our cause and for his many contributions to helping us understand more about Histiocytosis which will enable us to seek a rational cure.

We have lost not only a great and dedicated scientist, but also a wonderful friend.”

Scientists Bobby Gaspar and Adrian Thrasher together with Dr Marlene Carmo are making great strides in the Development of Gene Therapy for Familial HLH which is being funded by the H R Trust at the Institute of Child Health, London.

The team are looking at replacing Perforin, the commonest gene to cause inherited HLH. They have discovered that in order to cure HLH, patients who have an abnormal gene need to be given a normal perform gene. To achieve this, viruses called lentiviruses are used to carry the normal gene into the patients’ cells. To read more, look under our Latest Research section.

THIS year’s Nikolas Symposium has been hailed a landmark as clear targets have been set at which to aim new drug treatments for LCH.

H R Trust Trustee, Professor Peter Beverley, has advised that following the discovery of BRAF V600E, a mutation found now in both some samples of LCH and certain cancers, a huge understanding in LCH has resulted.

The conference, the 21^stto be held since its formation, was entitled Dendritic Cell Genomics and Metabolomics:  Clues for LCH Causes and Cures and was attended by scientists and doctors from all over the world, including H R Trust Trustees Drs Vasanta Nanduri and Johann Visser and Professor Beverley. 

It was Professor Barrett Rollins of Harvard University, who had attended a previous Nikolas Symposium, who identified in 2010 a change or mutation, in the DNA in half of LCH samples. Tellingly the mutation is in a kinase and these molecules are the key to controlling cell growth. 

Mutations in kinases are often found in cancers and the particular mutation found in LCH samples, called BRAF V600E, has already been found in other forms of cancer. Because the discovery of V600E in LCH is a breakthrough in understanding the cause of LCH, much of Nikolas 21 focused on the implications. 

No scientific advance is considered secure until confirmed, so it was important that two independent labs presented data confirming Professor Rollins’s finding. As the mutation is found in only half of LCH samples, it is likely that other mutations are present in the other half. 

Delegates discussed methods for searching for further mutations and another important question that was considered was whether only the mature LCH cells found in LCH tissue samples, contain the mutation. Alternatively the mutation might be present in blood or bone marrow stem cells as well, which would have implications for treatment. 

Though these and many other questions remain, the presence of V600E indicates that LCH is a form of cancer as well as providing a clear target for drug development. Encouragingly, many pharmaceutical companies are developing drugs against kinases for cancer therapy and the meeting heard a preliminary report on a clinical trial of one of these as a treatment for LCH.

A TEAM of volunteers has outlined plans to organise a number of events and raise funds for The H R Trust.

Anne Devenney, Sherrie and Paul Bennett, Lynn and Adrian Oldfield, Samantha and Andrew Terry and Stephen Masser met at a Volunteer’s Day recently in Stoke on Trent to discuss how to raise money to fund vital research and support patients and their families.

The meeting was led by Rachel Walmsley, of Succeed Training and Development and Trust Co-ordinator Shelley Ross. Trust Secretary Jane Ingleby and her husband, Howard, the charity’s Treasurer also attended.

The meeting focused on the role of the Trust and how supportive it was and also looked at what could be improved.

The group advised they were happy with the website and how quickly it was updated as well as being pleased that The Trust now had a new Facebook page as well as a Forum. They also said they received excellent support for publicising their fundraising events and that Dr Vasanta Nanduri was dedicated to helping them with their concerns.

They agreed that more volunteers were needed to move the Trust forward and advised that appointing a patron could also achieve this. It was suggested that The Trust needed to improve its Google listing as well as identify relevant grants.

The discussion then focussed on the type of events that could be organised to raise money. Suggestions included a Charity Ball, taking part in marathons, organising a children’s disco, organising a sponsored Zumbathon and Spin event, a comedy evening and a stall at a car boot sale.

The volunteers were divided into pairs and then worked on compiling a fundraising plan. They were also given a presentation about the importance of publicity and how this can be maximised.

Trust Co-ordinator, Shelley Ross, said: “We are very grateful that our volunteers continue to take time out of their busy lives to help move the Trust forward. They are all very committed to helping the Trust and we are looking forward to helping them with their events should they require it.”

The Trust is always looking for volunteers to help and anyone who is interested should contact Shelley either via email Shelley.ross@hrtrust.org or by calling 07850 740241.

THE BUPA London 10,000 takes place on Sunday May 27^th2012 and will give runners a one-off opportunity to experience the Olympic course before the official athletes do in Olympic Year.

The H R Trust has just 20 places available (minimum sponsorship required) to run this magnificent course which has been designed by the Director of the London Marathon.

By entering you can help us achieve our goals of funding scientific research into uncovering not only the causes of Langerhan’s Cell Histiocytosis and Haemophagocytic Lymphohistiocytosis but also ensuring early diagnosis, effective treatment and a cure. You will also be helping us support patients and their families.

Buckingham Palace acts as the backdrop to the start and finish of the run and your route will take in some of London’s most famous sights including Big Ben, Westminster Abbey, St Paul’s Cathedral and The London Eye before finishing at Nelson’s Column in Trafalgar Square.

In return for your guaranteed place, we ask that you raise a minimum of £350. We will give you a free H R Trust running specific sweat wicking fabric vest while the organisers will give you a special tag which can be attached to your shoe to record your time. You will also receive a souvenir kit bag.

To sign-up, please contact Shelley on 07850 740241 or via email Shelley.ross@hrtrust.org

.

THE Histiocytosis Research Trust's latest research project for 2011 will focus on LCH and improving diagnostic and prognostic tests as well as therapeutic targets. 

The recipients of The Jon Pritchard Research Award are Professor Frederic Geissmann of King’s College, London and Professor Matthew Collin, of the Institute of Cellular Medicine at Newcastle University. 

The duo’s project, entitled Cell Autonomous Mechanisms in the Pathogenesis of Langerhan’s Cell Histiocytosis, was selected from 12 applications received after the Trust invited calls for proposals last May. 

Professor Collin explained:  “We are delighted to receive this award. It will allow LCH research in the UK to take a unified stance against the disease with clinicians and scientists all working together. With LCH IV and the help of all the patients, we hope to be able to look at the cells and tissues of LCH in new ways, to explore exciting new ground related to the recent discovery of the V600E BRAF mutation in the majority of LCH cases. 

“Our project aims to directly test the role of abnormal survival and proliferation in the development of LCH by a number of approaches. In particular, we will generate models to study the role of mutation of BRAF and we will investigate the existence of mutations in BRAF and in related cellular pathways in human patients. These studies are likely to lead to new diagnostic and prognostic tests and therapeutic targets for LCH.” 

To date the causes of LCH and the mechanisms responsible for the disease are not well understood making the establishment of diagnostic and prognostic tests and the design of rational treatment extremely difficult. 

However, after many years of searching for candidate genetic lesions in LCH, a recent breakthrough study has identified a mutation of the gene BRAF in over half of LCH samples. BRAF is a protein involved in cell activation, survival and proliferation and is mutated in numerous human cancers. 

“This suggests that in the majority of patients, LCH is connected to a genetic defect within the cells themselves, although a complex of other factors may still play a role in LCH pathology particularly in patients who do not have a BRAF mutation,” added Professor Collin. 

Those who applied for the Jon Pritchard Research Award were asked to set out the key objectives of their research and provide a brief description of the background, methods to be used and key references. They were also asked to describe the principal investigator and research facilities and indicate the source and availability of clinical samples if these were to be used. 

Applications were scrutinised by the Trust’s Scientific Review board led by H R Trust Trustee Professor Peter Beverley and colleagues Professors Alain Fischer, Ron Jaffe, Hugh Perry and Ralph Steinman. They were also assessed by independent external reviewers. Candidates had to submit proposals linking basic and clinical science but the overriding consideration was scientific excellence. 

The panel faced a difficult task in identifying the best projects as they were all of a high standard but in the end Professor Geissmann and Professor Collin were awarded funding for their project. 

Professor Peter Beverley explained: “We received some very high quality applications and had great difficulty in choosing a recipient but in the end, we felt that Professor Geissmann and Professor Collins’s project matched all of our criteria and will hopefully take forward existing knowledge about LCH.” 

The three-year project will cost just over £327,000 and will be funded by The H R Trust and The Artemis Association of Greece.

 This will be the third project that has been funded by The Trust. The first project, led by Professor Hans Acha-Orbea, aimed to identify genes that are switched on when normal dendritic cells, which are closely related to Langerhans Cells, become tumours. The study took place at the University of Lausanne, Switzerland.

 The Trust’s second project, which is still ongoing, is looking at the Development of Gene Therapy for Familial Haemophagocytic Lymphohistiocytosis (HLH) due to perforin deficiency. Scientists Bobby Gaspar and Adrian Thrasher together with Dr Marlene Carmo are working on replacing Perforin, the commonest gene to cause inherited HLH.


 

THE next meeting of The H R Trust's volunteers will take place on Saturday July 23rd at the Tollgate Hotel and Leisure, Stoke, between 11am and 2pm.

Anyone who is interested in helping the Trust move forward and would like to attend, please contact Shelley either via email at Shelley.ross@hrtrust.org or by calling 07850 740241.

MEMBERS of the Circle of Friends are taking part in a variety of activities to raise money for The H R Trust.

Treasurer Howard Ingleby and friend Jason Miller are taking part in the Skyline London to Paris Cycle Ride between June1st-5^th.

During the five day trip, the pair will be cycling 550km passing through some of history’s most famous landmarks and battlefields.

After beginning their journey in London, the pair headed to Dover and after crossing the Channel to Calais, they will cycle through Abbeville and Beauvais, before finally arriving in Paris.

Howard said:“The Histiocytosis Research Trust is the only charity in Europe funding research into the rare diseases Langerhan’s Cell Histiocytosis and Haemophagocytic Lymphohistiocytosis. We need £380K now to fund some very exciting research projects to help us find a cure.”

Simon Harper is due to take part in the Mazda Blenheim Triathlon on June 4^th and Anne and John Devenney together with Nicola Wilkes will be taking part in the Great Midlands Fun Run on June 5^th.

Nicola is taking part in the run in memory of her boyfriend’s mother, Carol Goodbourn, who died of HLH earlier this year.

To help with her training, Nicola has downloaded an Iphone application ‘Couch to 10K’ to help her train for the fun run. Despite being a 13 week programme, Nicola will have only trained for eight weeks when the run takes place but reveals she has thoroughly enjoyed it.

“After I have completed a run, I feel so good about myself and that feeling cannot be replaced at the moment especially as I feel I am doing some good for not only myself but for The H R Trust. The support has been fantastic and I am very overwhelmed by the generosity there is out there although I still need sponsoring as the more pounds I get, the closer we are to finding out more about this terrible disease and one step closer to a cure.

“I feel very privileged and honoured that I can represent this worthy cause on June 5^th and hope to make a little difference with the sponsor money,” explained the 27-year-old.

Anne and John Devenney will be taking part in the run in memory of their son Cameron. John will be wearing a 6ft Iggle Piggle outfit with the hope of raising more awareness about The Trust and HLH.

Sherrie Bennett has organised a Family Fun Day with special guests Zippy, George and Bungle from children’s TV programme, Rainbow. The Wizard of Oz themed event takes place on Saturday June 11^th at Broomhall Recreation Ground, Broomhall Lane, Sunningdale, between 11am and 4pm. Further information can be obtained by emailing Sherrie at Sherrie150@btinternet.com

Sherrie also organised the Shining On Annual Netball Charity Cup in Woking recently during which 12 professional teams took part.

Emma Krug was so moved after learning that her five-year-old nephew Ben was suffering from LCH,  she has arranged a Spring Fair also on Saturday June 11^th.

She said: “My mum and I want to raise awareness about this particular disease because it is very rare. We also feel that as it is so rare, research is vital. My mum, Lucy (Ben’s mum) and I really want to help raise money for The Trust in any way we can so that diagnosis, causes (if any) and treatment can be quicker.

“We would like to thank The Trust for all the help, support and advice that they have given us as a family and want to make other sufferers and their families aware of this small but wonderful organisation,” added 30-year-old Emma.

The fair will be held at the Church Rooms, Ludham, Norfolk, between 1pm and 4pm. Further information can be obtained by emailing Emma at emmakrug@btinternet.com

Brett Hill will be taking part in the C2C Workington to Sunderland between June 11^th-12^th while employees of YPO in Wakefield will be taking to their bikes and cycling 128 miles between Morcambe and Filey.

On June 19^th, plucky Emma Holloway will be soaring through the skies when she undergoes a parachute jump in Cambridge.

The 22-year-old explained:“Whilst I am nervous about doing the jump, I am also very excited. I would like to raise at least £500 for the Trust which has been very supportive to my future brother and sister in-law.”

And part-time musician Kevin Britt has recorded You Raise Me Up with all proceeds being given to The Trust. 

Kevin explained: “I decided to record the track, not only to raise money but also to give the Trust a helping hand. I know what it is like to have a family member ill and I know how hard it can be. I have always wanted to do something for a charity and raise awareness and thought to myself that now is the best time for me to help out in some way.

“I am hoping this song will help us raise lots of funds and raise awareness and also realise what family means.”

The song can be downloaded from both iTunes and Amazon mp3.

If you are raising money for the Trust please contact Shelley who will help publicise your events with the local media in your area. Shelley can be contacted on 07850 740241 or by email Shelley.ross@hrtrust.org

A MEETING organised to recruit volunteers to help move The H R Trust forward has been hailed a great success.

Anne Devenney, Sherrie and Paul Bennett, Lynn and Adrian Oldfield, Joanna Savva, Hilary Bickler, Samantha Terry and Stephen Masser have all been allocated specific roles as part of an initiative to improve communication, support, administration and most importantly fundraising to help find a cure for LCH and HLH.

The recent meeting was led by Trust secretary Jane Ingleby who explained: “There are several areas of the Trust we need to improve such as seeking out grants, organising marathon packages, overseeing Facebook and organising a forum on our website. These are just a few of the things that we need to improve but as there are so few of us, the only way to achieve this is by recruiting volunteers.

“We surveyed our Circle of Friends last year and one of the questions was would they be prepared to become more involved in the running of the Trust.  I am delighted to say that several people have stepped forward and taken on roles.

“I am now confident that with this support we will be able to move The Trust forward and improve our fundraising prospects.”

The roles allocated are:

Facebook -  Joanna Savva, H R Trust Forum - Anne Devenney, Marthons - Sherrie and Paul Bennett, Grants - Adrian Oldfield, Christmas Cards - Hilary Bickler, Items to Sell - Lynn Oldfield, LCH Befrienders - Samantha Terry, Jane Ingleby and Stephen Masser, HLH Befrienders Paediatric - Anne Devenney and Lynn Oldfield and Collection Tins Co-ordinator - Samantha Terry.             

H R Trust President, Paul Kontoyannis said: “I am delighted this team of people have stepped forward and I would like to welcome them to the Trust. Of course, I must not only thank them, but also the dedicated nucleus of fundraisers amongst our Circle of Friends who raise money on our behalf constantly.  The Trustees are all very grateful.

“All of their combined efforts will help the Trust offer more support and information and be the leader in research to find cures for both LCH and HLH.”

The next meeting of the volunteers takes place on Saturday July 23^rdat The Tollgate Hotel and Leisure, Stoke on Trent. If anyone is interested in joining the team and attending the meeting, please contact Shelley on either 07850 740241 or via email Shelley.ross@hrtrust.org

ATHLETES Andriani Fouseki and Nikos Korras will be travelling all the way from Greece to take part in the BUPA London 10,000 on Monday May 30^th 2011 with the aim of raising thousands of pounds for The Histiocytosis Research Trust.

Both Andriani, aged 37 and Nikos, aged 32, from Loutraki, Greece, were inspired to take part in the BUPA London 10,000 after meeting 29-year-old Nikolas Kontoyannis at a physiotherapy centre in Greece - Nikolas was diagnosed with Langerhan’s Cell Histiocytosis as a baby which has left him with certain disabilities.

Nikolas explains: “I was eight weeks old when I was diagnosed with LCH – a very rare disease. Very little was known about it and I was given a 30% chance of survival. The possibility of death was very real for me and my parents were told to prepare themselves for this.

“However, against the odds and thanks to the dedication of my doctor, the late Dr Jon Pritchard and my parents at this crucial time – I survived. Others have been less fortunate and have died. In my case, the disease is currently inactive but has left me with certain disabilities that I shall carry with me for the rest of my life and the shadow of its recurrence is ever present.”

Nikolas is not alone in this. Langerhan’s Cell Histiocytosis (LCH) is a rare disease which affects 1 in 200,000 each year - in the UK alone, there are 50 new cases and it can strike both children and adults at any time. Very little is known about LCH and doctors are often unable to diagnose it because it presents with many different symptoms and as a result, diagnosis and treatment is delayed.

 People suffering from LCH have too many histiocytes – a histiocyte is a type of white blood cell which normally helps to fight infection. However, for those suffering from LCH, the histiocytes gather together in large numbers causing damage to healthy parts of the body. In some ways, LCH is similar to cancer and is currently treated with chemotherapy, radiation and steroids.

 Nikolas’s diagnosis prompted the formation of The Histiocytosis Research Trust which was set up to promote and fund vital scientific research into both LCH and the closely linked disorder Haemophagocytic Lymphohistiocytosis (HLH)  with the aim of ensuring early diagnosis, effective treatment and a cure.

 Research into both diseases has been very limited due to lack of funds and has been the result of donations and fundraising by a dedicated team of parents and supporters.

 Andriani and Nikos were so moved by Nikolas’s plight they jumped at the chance to take part in the BUPA 10,000 and raise money for The H R Trust.

 Nikolas said: “As soon as the idea of taking part in the race was put to them, and being aware of my life experience with Histiocytosis, without question they agreed to travel to London which has made me very happy, excited and proud. I shall be waiting for them at the finish line!”

 Public and professional awareness of both LCH and HLH are essential if The H R Trust is to fund not only ongoing research, but also implement further research on a larger scale. There are also hopes to fund a dedicated nurse and an adult LCH doctor.

 The H R Trust is closely linked to the Nikolas Symposium, an annual conference attended by leading doctors and scientists from all over the world and also has ties to The Artemis Association, Greece, The Histiocyte Society, Euro Histionet and The Histiocytosis Association of America.

If anyone would like to sponsor Andriani and Nikos, please visit www.justgiving.com/Nik-Kontoyannis

 Nikos, Nikolas and Andriani

THE Histiocyte Society’s latest annual meeting has been hailed a great success after it was revealed that LCH is an unusual type of malignancy or cancer.

The findings were presented in a report by Dr Barrett Rollins’ laboratory in Boston which showed that over half of the LCH biopsies examined by scientists contained a specific genetic change in BRAF called V600E which has also been seen in melanoma, colorectal cancer and thyroid cancers.

As a result, scientists have concluded that this is the first direct evidence to determine that LCH has, in many cases, the same genetic changes as many common types of cancer and is the best evidence to date that the disease is an unusual malignancy or cancer.

Dr James Whitlock, President of The Histiocyte Society, said: “Much work remains to be done to better understand the precise role of this gene in the development of LCH, but it raises the promise of new therapies for patients with LCH, since several pharmaceutical companies have developed new drugs which inhibit, or block, the abnormality caused by the BRAF mutation in cancer lines.

“Work is already underway by members of The Histiocyte Society to develop and open a clinical trial which will test whether a new drug which targets the BRAF signalling pathway will be effective in patients with LCH.”

The meeting, partially sponsored by The H R Trust, was attended by over 170 clinicians from 100 countries and was held in Boston, USA.

Some other highlights included:

• the identification of new genetic mutations leading to HLH
• several reports providing additional information about the effectiveness of cladribine in the treatment of recurrent LCH and other histiocytic disorders
• new information about the effectiveness of clofarabine in the treatment of recurrent LCH
• new data about clinical findings in adults with HLH
• new information about the effectiveness of alemtuzumab (Campath) in the treatment of refractory HLH
• planning of the LCH-IV clinical trial protocol for patients with newly diagnosed LCH
• discussion of new pilot studies to improve the treatment of children with newly diagnosed HLH
• planning of the Society’s International Rare Histiocytic Disorders Registry

Further information about The Histiocyte Society can be found on www.histiocytesociety.org

LCH EXPERTS from around the world will attend the next meeting of Euro-Histio-Net in March.

H R Trust Trustee Richard Price will join clinicians, scientists and members of patient groups at the meeting in Genoa, Italy.

The aim of the two-day event is to review work that has been achieved during the last 12 months as well as consider tasks for the next year.

During the last 12 months, guidelines for the diagnosis, clinical work-up and treatment of paediatric LCH patients for those not formally enrolled in a study have been approved by most members of the working group and will soon be published.

Guidelines for adults are also to be drawn up and these will be overseen by Michael Girschikofsky of the Austrian Working Group for Histiocytic Diseases in Adults.

In addition, guidelines have been produced for tissue banking in LCH and work on the new web portal is proceeding well. The final version is due to be published shortly at www.eurohistio.net

A specific criteria for the appointment of experts in LCH has also been produced as well as a list of frequently asked questions for patients and professionals which will be included on the web.

Project Manager, Eva Schaefer, said: “At the end of the second year of our European project for Langerhan’s Cell Histiocytosis and associated syndromes, we have achieved a lot of important goals.

“The last year of our project will be dedicated to several further important tasks and in order to achieve them, we are organising this meeting.”

The Euro-Histio-Net project was established to increase the knowledge of Langerhan’s Cell Histiocytosis and associated syndromes with the aim of improving patient care.

The projects main objectives are to impro­ve the quantity and quality of knowledge exchange, produce guidelines and set up an international data base. A web portal has also been devised to make any newly acquired information available for professionals, doctors and patients.

It is hoped that the comprehensive collection of patient data and disease courses in an international data base will help histiocytosis experts increase their knowledge about the disease, determine better diagnostic and therapeutic measures with a view to improving patients’ treatment. 

It will also mean that non-specialised doctors will be able to access better information about the disease.

THE recipient of the Jon Pritchard Research Award 2011 will be announced shortly. Consideration was given to proposals linking basic and clinical science although, the overriding consideration was scientific excellence. A shortlist was considered by The Trust's independent Scientific Review Board.         

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THE UK’s leading LCH and HLH specialists turned out in force to meet patients and parents at The H R Trust’s recent Road Show.

Drs Peppy Brock, Vasanta Nanduri, Johann Visser, Kevin Windebank and Professor Matthew Collin headed to London’s Great Ormond Street to talk about LCH and HLH to about 32 patients and parents.

The clinicians were joined by H R Trust Trustees President Paul Kontoyannis, Vice-President Professor Peter Beverley and Treasurer Howard Ingleby. In his address to the audience, Mr Kontoyannis said: “We must work together to find what causes these terrible diseases. I want you know that you are not alone. Every child with LCH or HLH is our child and part of our family.

“We are very small, but we have a big agenda – not only to help the patients but to also solve these diseases. We are working on it and we hope to do it in our lifetime.”

He added that in addition to funding vital research programmes, there was also a need to fund a replacement for Professor Tony Chu, a specialist in Adult LCH, who is due to retire in three years time. The H R Trust also wanted to be able to employ a nurse to advise and support patients and their families.

“We have a responsibility to help, there are many needs besides research,” he concluded.

Professor Matthew Collin, one of the UK’s two specialists in Adult LCH who is based at the Freeman Hospital, Newcastle upon Tyne, advised that treatment of Adult LCH was behind that of paediatric LCH. After explaining how LCH affected adults, he added that an increasing proportion of adults had been diagnosed as children but due to the lack of specialist doctors, they faced a long journey in securing treatment.  

However, he said: “After two years of research, it is an exciting time in terms of our knowledge about dendritic cells – a new horizon is dawning is this field.” He added that a current research programme was now looking at where dendritic cells were coming from.

Dr Kevin Windebank, Senior Lecturer in Child Health at Newcastle University as well as an Honorary Consultant in Paediatric Oncology at Newcastle Hospitals NHS, said there were 50 cases of paediatric LCH in the UK and Ireland each year. 

Dr Johann Visser, Consultant Paediatric Oncologist at Leicester Children’s Hospital, explained about the new LCH4 Clinical Trial which is due to take place. For the first time, paediatric patients with LCH both in the UK and internationally, will be enrolled and monitored over a 10 year period, irrespective of whether they need treatment or not.  He added that at least 600 patients would be needed for the trial to be a success and research was being developed to store tissue samples.

Dr Vasanta Nanduri, Consultant Paediatrician at Watford General Hospital and member of The Histiocyte Executive Board and co-chairperson of the Histiocyte Society Epidemiology/late effects study group, gave an informative talk about the possible late effects of LCH.

H R Trust Vice-President Professor Peter Beverley explained about the two research projects that the Trust has funded since 2005. The first project, which cost £150,000, was led by Professor Hans Acha-Orbea and looked at identifying genes that are switched on when normal dendritic cells, which are closely related to Langerhans’ Cells, become tumours. The study took place at the University of Lausanne, Switzerland.

The second three-year project, currently being funded at a cost of £220,954, is focussed on the Development of Gene Therapy for Familial Haemophagocytic Lymphohistiocytosis (HLH) due to perforin deficiency. Scientists Professors Bobby Gaspar and Adrian Thrasher together with Dr Marlene Carmo are working on replacing Perforin, the commonest gene to cause inherited HLH at UCL.

Professor Beverley announced that the next project to be funded by the Trust would be aimed at LCH and more details would be announced in January 2011. The project is due to start in Spring 2011.

He added: “When we fund research, we make sure that we are going to fund good research carried out by good people. I want you to know that we are making progress.”

Feedback from the Road Show has been excellent. Parent Jane Wynn said:  “The Road Show was interesting and relevant. It was good to hear from different aspects in tackling the disease.”

Parent Gavin Weeden added: “All of the Road Show was useful but in particular late effects, research and clinical trials.”

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April 30th- May 3rd 2010

Thirty-two of the world’s leading scientists and doctors descended on Loutraki, Greece to attend this year’s Nikolas Symposium.

One of the most perplexing questions about LCH is why and how the disease attacks the brain (the Central Nervous System or CNS) and how distressing brain symptoms can be treated.

This year’s conference addressed these difficult questions. Since LCH is so rare and also because it is always difficult to study disease inside the skull, the conference discussed other brain diseases that might throw light on how brain inflammation and damage occurs in LCH.

It is remarkable that infection can take place without any immune response or inflammation. Even more remarkably later inflammation elsewhere in the body can make inflammation in the infected brain occur, leading to brain damage.

Therefore brain damage depends partly on processes occurring in the brain itself but also on events that take place outside the brain, leading to the entry of white blood cells or antibodies into brain tissue. By analogy with other diseases, since LCH attacks particular parts of the brain, it is likely that the targets of the disease are very specific brain molecules or cells.

There was much discussion on how these targets could be identified, as this could lead to new treatments, as it has in other brain diseases.

Professor Peter Beverley

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