The Royal Hallamshire Hospital
Glossop Road
Sheffield
South Yorkshire
S10 2JF                        
Switchboard: +44 (0)114 2711900
To speak to one of our team, call Kay on: 01142 711 848

The Royal Hallamshire Hospital is situated a mile from the city on the Trust's central campus. Nearly 4,500 staff work at the Royal Hallamshire in a wide variety of clinical and non-clinical roles.
For more information about the Royal Hallamshire Hospital and how to get there click here.

Nature of service:
A multidisciplinary HD clinic was established at the Royal Hallamshire Hospital, Sheffield, UK in 2002. The main aim of this clinic is to improve the care of HD in South Yorkshire by bringing together the expertise of different specialists. The clinic was set up by Dr Oliver Bandmann (Consultant Neurologist) and Dr Oliver Quarrell (Consultant in Clinical Genetics).  We are now fortunate enough to have a DeNDRoN research nurse, and a genetics nurse as well as established links with Neuropsychology and Liaison Psychiatry (Dr Paul Gill).

Clinic Times:
The multidisciplinary HD clinic runs on Tuesday Afternoons twice a month.
 
Patients should be referred to this clinic by their GP or a specialist. The referral should be sent to:
 
Dr. Oliver Bandmann
The Royal Hallamshire Hospital
Glossop Road
S10 2JF
 
For enquiries about hospital appointments, please contact Dr Bandmann’s secretary Marilyn Bestall (0114 2713005) 

Genetics clinics:

In addition to the symptomatic clinic at the Royal Hallamshire Hospital, the clinical genetics service offers home visits by genetic counsellors and clinic appointments at the Sheffield Children’s Hospital and the District General Hospitals in Barnsley, Doncaster, Worksop (Bassetlaw) Chesterfield, Rotherham and Grimsby.
We see families for the following reasons:

• Following a new diagnosis in a family
• Concerns about being at risk for HD
• Predictive testing
• Pre-natal tests
• Pre-implantation genetic diagnosis (PGD) for HD as part of a satellite service of Guy’s Hospital.

1. The Euro-HD registry Study NOW RECRUITING
 
We are participating in the Euro-HD registry study. This is a natural history project which also allows us to develop a cohort of patients who are interested in research and can be involved in future drug trials.
 
The Euro-HD registry study clinics are currently run twice a month, usually on the first and third Tuesday of every month. Three participants are invited to come and see Dr Oliver Quarrell, Kay Fillingham, Kirsty O’Donovan and either Alyson or Helen on that afternoon.
 
The clinics are carried out on the Neuro Day Care Unit (M-Floor) of the Royal Hallamshire Hospital. Since 2008,  families who would like to participate in REGISTRY can also  be seen at the Sheffield Children's Hospital and at the District General Hospitals in Barnsley, Doncaster, Worksop (Bassetlaw) Chesterfield, Rotherham and Grimsby in addition to the Royal Hallamshire Hospital.  This has the potential to reduce travel times for patients.  Please let us know your requirements.  
 
If you would like to participate in the REGISTRY study or would like any further information, please contact:

Kay Fillingham
DeNDRoN Clinical Research Nurse for HD
Room 125d, Floor N
Royal Hallamshire Hospital
Glossop Road
Sheffield, S10 2JF
Phone: 0114 2711848
Email:  
FAX: 0114 2713158                                                
                                                                          
2. Drug Trials
In 2006-2007 we collaborated on a drug trial which aimed to test a possible treatment for improvement of motor symptoms associated with chorea, dystonia and Bradykinesia in patients with Huntington’s Disease. Unfortunately, the study showed that this particular treatment (Ethyl-EPA) did not improve motor symptoms in Huntington’s disease patients. However, the trial was useful in giving a clear answer and showed that such trials can be successfully carried out in the future.

We are currently participating in the ACR16 drug trial. The main aim of this drug trial is to assess the ACR16 test compound for its beneficial effect on overall mobility and walking in patients with HD.

3. Gastric Biopsy Study
We are co-ordinating a study of patients having feeding tubes inserted; an option for patients is to have gastric biopsies taken.  The purpose is to study the effects of the HD gene on tissues outside the nervous system.
There is an additional research option within this study looking at the decision making process for patients who are tube fed.

4. Disease-modifying factors and biomarkers
Every patient with HD is different to the next both in terms of the speed at which the illness progresses and also in terms of the specific symptoms and problems that a particular patient develops. Dr Bandmann and collaborators have now begun a study looking at the presence and levels of particular antibodies in the blood of HD patients which might be useful to monitor the disease progression and possibly also to explain specific symptoms, in particular weight loss.
 
5. Skin biopsies to investigate mitochondrial function in HD
Abnormal function of cells in the body is not limited to the brain in HD – distinct abnormalities are also present in particular skin cells (fibroblasts) of patients with HD. Dr Bandmann and co-workers are now taking skin biopsies from HD patients to better characterize these abnormalities. Any abnormality we find in the skin cells is likely to be present in the brain as well. We are therefore assessing the function of the “power generators” (mitochondria) in HD skin cells in detail. We hope to subsequently be able to use the HD skin cells with the abnormal mitochondrial function to identify drugs which will help us to make these power generators work normally again in HD.

6. Juvenile Huntington’s Disease
Dr Quarrell is  lead facilitator for the juvenile Huntington’s Disease Working Group of the European HD Network.  The projects being developed include:

• Developing new rating scales: Only a few patients with HD have very early onset but the current rating scales used in drug trials are not suitable for those with this form of the condition. Currently, there is no medication to slow down the disease process. We want to use this time to develop appropriate rating scales so that young people can be included in future studies of such treatments.
• An international study of the needs of carers: We are collecting interviews from carers of juvenile HD patients from a number of different countries and will compare their experiences with the information we have already gathered from families within the UK. We want to know if the experiences are the same or whether there are important differences within the countries of Europe and North America.
• We have summarised information on juvenile HD in a book called ‘Juvenile Huntington’s Disease and other trinucleotide repeat disorders’, published by  Oxford University Press 2009.
• There are no guidelines available for the management of juvenile HD so we are conducting a survey of the medications currently used to help manage these young patients.

7. Information for families
Dr Quarrell has  published a second edition of ‘Huntington’s Disease – the facts’. published by Oxford University Press 2008.  This book contains information about HD for families and carers. The new edition contains quotes from families and professionals to supplement the information about HD. For more information please go the HDA website.

About the Dementias and Neurodegenerative Diseases Research Network (DeNDRoN):

The Dementias & Neurodegenerative Diseases Research Network (DeNDRoN) provides a world-class health service infrastructure to support clinical research and remove barriers to its conduct. DeNDRoN does this through investment in regional research networks.
 
Clinical Studies Groups have been set up to represent Motor Neurone Disease, Huntington’s Disease, primary care, Parkinson’s Disease and Dementia. The groups are multidisciplinary and aim to involve patients, carers and UK charities in their area of research. The DeNDRoN network brings together expertise and enthusiasm to drive forward research and facilitate patient participation in trials in the dementias and neurodegenerative diseases community.
 
For further information or to find out how you can become involved, please visit www.DeNDRoN.org.uk