SIF - Sheffield Institute Foundation

SIF have been working tirelessly over the last few years to raise money and awareness of Motor Neurone Disease (MND). The foundation's aim has been to raise the funds needed to build the Sheffield Institute of Translational Neurosciences (SITraN), and finance it for the first 5 years. It will be the first of its kind in the World, and will continue to grow and develop in to a pioneering Institute attracting first class. Scientists and Professors from around the World, such as Professor Pam Shaw.

About MND

Motor Neurone Disease is the name given to the group of diseases which are caused by the death of motor neurones. These are the nerve cells that link our brain to our muscles, sending electrical impulses to control muscle movements. Also known in some countries as ALS or Lou Gehrig’s disease, MND is a fatal condition with no cure and virtually no form of treatment. From diagnosis, sufferers live on average for only two years and only about 10% of sufferers live beyond five years.

MND attacks and destroys motor neurones, disrupting these vital links within the body; without the stimulus of electrical impulses, the muscles cease to function and waste away. Eventually this muscular atrophy leads to acomplete loss of mobility, speech and the ability to swallow; death typically comes from paralysis of the breathing muscles. MND is devastating, usually leaving the sufferer with full retention of their mental faculties and trapped in a living hell. This is compounded by the speed at which the disease takes hold, giving little opportunity for clinicians to help patients and their families adjust to the condition. For these reasons many doctors regard MND as the worst disease known to medicine.

We know very little about the causes and development of MND. There is little understanding as to what relationship exists between a sufferer’s genetic make up, environment or life style. Men are twice as likely to be affected as women. Only around 5% of cases appear to be hereditary in nature. Furthermore, researchers believe that there is a link between high levels of physical activity and MND. Incident rates of MND are materially higher for people who have served with the armed forces; studies amongst professional footballers show incidence rates nearly seven times that of the general population. However researchers do not know if the onset of the disease is triggered by high physical stress levels only when combined with a certain genetic characteristics in an individual, or whether other environmental factors are at work.

Our lack of knowledge is largely due to the fact that, despite its horrific impact on thousands of people in the UK alone, MND has not benefited historically from the levels of funding directed at many other areas of disease research. Between 1999 and 2004, six thousand people in the UK died from MND. This compares with just over four thousand from HIV/AIDS and less than one hundred from CJD. Over the same period £45million was spent on HIV/AIDS research, £33million on CJD and just £8 million on MND.

This low level of funding can partly be explained by the sheer pace at which MND advances. The low life expectancy amongst MND patients means that the disease area is not a priority for the pharmaceutical industry – quite simply, the industry would not be able to recover its investment given the limited time frame available to treat patients. This low level of investment in research to date means that there are almost no therapies available to alleviate the symptoms of MND. Only one drug, Riluzole, has ever been successfully developed and brought to market. Riluzole prolongs life for, at best, just a few months. So to be diagnosed with MND today is a sentence to a bleak and brutally short future.

We now have a chance to play an important role in changing this future. The creation of the Sheffield Institute for Translational Neuroscience will establish the world’s first stand alone institution dedicated to fighting MND. Through supporting this expedition in aid of SITraN, you will not only be giving us much needed encouragement, you will be making a vital difference in helping to achieve a major leap forward in bringing an end to the devastation caused by MND.

Case Studies

Neil Platt's Story

Louise and I were overjoyed at the birth of our son Oscar in August 2007. Things were shaping up nicely, we had just managed to become property owners and I was excited at the prospect of new career challenges. Everything was pretty normal really, but in this day and age,it feels like quite an achievement.

It is now September 2008 and we no longer live in our own house. I gave up work when I could no longer speak on the telephone and the voice recognition software stopped recognising my voice. I’ve not been able to hold my son for months.

The first symptom was a pain in my foot. I thought it might have been the pair of shoes I had been wearing. That was October, when Oscar was just two months old. By November, I couldn’t carry him because of the walking stick. My wife who had only just recovered from a traumatic birth suddenly had to become the packhorse for our family, and I had to watch her. In December, on Christmas Eve, I had an MRI scan and I remember calling my wife with the result and making her cry. We had travelled to relatives that evening, everyone keen to share baby’s first Christmas, but it was overshadowed by devastation. In January I spent two nights away from Louise and Oscar while I underwent more tests, and in February we went to the hospital for formal diagnosis. March was spent saying goodbye to our life in London, and in April we moved away from our first home to a rented bungalow with wheelchair access and nearer the help of family. I walked into this house in April with a walking stick. It’s now September and I only have control of my head and neck and two fingers on my right hand.

Our little unit of three no longer exists. We can’t function without at least two other people at all times. I watch friends push Oscar on his swing and hold his hands while he literally tries to run before he can walk. Louise has a daily battle with when to spend time looking after her husband and when to spend time looking after her baby. It’s an either/or situation. Our home has been invaded by varying degrees of mobility equipment, battling for space with baby equipment, and there’s a constant stream of health professionals through the door. We’ve only been married for three years and already we’ve lost all sense of privacy. I now spend my days being hoisted from a chair to the commode and back again and my nights are spent in a bed that’s plugged in to the mains with a ventilator mask over my face and a nurse watching over me while my wife has to sleep on a blow-up bed in the living room. She knows that I have great difficulty sleeping because of the pain and anxiety and she tries her hardest to stop Oscar from waking me with his crying when he’s teething. I can do nothing to help. We tried to rest during the day together last week and she lay her head on my chest, but found it too upsetting, I can’t even put my arm around her, she has to put it there herself.

I think of the things that I would have loved to have done with Oscar and Louise, things that my father had time to share with his family, things that I had been looking forward to sharing with mine. I think of who will do these things in my stead. My wife wanted to have a sibling for Oscar, but we were too late. She now worries about bringing him up without a father or a sibling, and wonders how she can possibly compensate for both. But most of all, she worries about how to tell Oscar his family history, and how it may affect him. His great grandfather died of MND aged 60. His grandfather died of MND aged 50. His father is 34 and there is still no treatment, farless a cure.

The only consolation of history repeating itself this time is that he won’t have the memory of seeing my decline.

SITraN

The Sheffield Institute for Translational Neuroscience

The University of Sheffield is already home to the UK’s leading clinical research department for MND. Established in 2000 by Professor Pamela Shaw with initial funding from the Welcome Trust and the MND Association, today the department has more than 80 clinical and science researchers.

Professor Shaw has dedicated her career to the care of MND patients and is regarded as one of the world’s leading MND specialists. She has been recognised for her work through numerous awards from international bodies, including the 2007 Forbes-Norris Award from the International ALS/MND Alliance, of which she is Chair of the Scientific Programme Committee of the International ALS/MND Symposium.

Dedicated institutes are a critical force in the fight against disease. Institutes bring the best teams of multi-disciplinary clinicians and researchers together under one roof, enabling personal interaction and ideas generation to be maximised. In the past, institutes have played leading roles in achieving important breakthroughs in disease research. Notable examples include the Institute of Cancer Research in London and the Pasteur Institute in Paris. The Sheffield Institute will be the world’s first stand alone institute dedicated to MND. The scale and skills of the team already assembled by Professor Shaw represents a world class cluster of MND scientists and researchers. However not only are they are currently scattered across multiple University and NHS sites in Sheffield, but there is no further room available for the team to expand. A dedicated, stand alone building will create a unique focus for MND researchers, giving the existing team the room it needs to continue its research, enhancing personal interaction and providing a first class working environment to attract the world’s best scientists and clinicians.

The Sheffield Institute will be focused on translational neuroscience, an approach where scientific developments in the laboratory will be rapidly translated into effective therapies in a clinical care environment. By combining both leading edge science and clinical care, the Sheffield Institute for Translational Neuroscience (SITraN) will become a real force in accelerating the pace of therapeutic development and improving the lives of those suffering from MND across the world. Creating SITraN will cost £20 million, of which £12 million is needed to build the Institute itself with the remaining £8 million needed to create an endowment to fund necessary additional researchers and clinicians. The University of Sheffield has donated the site on campus and £5 million. A further £10 million has been raised to date from a panel of patrons, led by the Duke of Devonshire as Honorary Patron, and individual contributions. With £15 million already raised, SITraN is close to becoming a reality. However we urgently need your help it cross the finishing line.

Through our Talisker Bounty Boat expedition, we hope to raise awareness of MND and SITraN, and through your support, help the fundraising effort for SITraN cross the finishing line.