November 1999 Newsletter


For those who were unable to attend the meeting in early October, this edition necessarily contains a substantial article describing the day, which, I hope, is a fair representation. If I have missed anything please let me know and I will include amendments or omissions in the New Year edition.

Further information on the newsletter, including some ideas for the future, is set out on the back page.

A copy of the current list of members is attached. You might find a fellow sufferer nearer than you think so please take advantage of it. If there are any mistakes in the list, or you would like to add your phone number, please let me know.

1999 Meeting

The meeting was held in Birmingham on 2nd October and was hosted by Tony Glas. Following an opportunity to meet over coffee, a warm welcome was extended by Tony to all those who attended the fourth annual meeting with many new faces among the regular attendees, including people from as far afield as Devon, Scotland & Germany. Sadly, as Tony explained, a group member Ian Jenkins had recently passed away and our thoughts are with his family.

Tony reminded us that the function of the group is principally self-help and that we bad not adopted the formal structure of becoming a charity although we bad a general constitution (copies of which are available from Ian on request).

Contributions are set at £10 per working family and £5 for non-working families although we are happy to support families who are unable to make a contribution.

Reports were provided by:

Mike Fawcett, the treasurer, reported that the group had a surplus for the year of £622.42 giving rise to a surplus carried forward of £1,862.27. There had been no specific fundraising during the year but we had received donations from Dr. Griffiths and Ian Jenkin's church. Tony added that members could seek assistance from the funds should the need arise. Mike has established a web site for the group (and there are other sites around the globe). In recognition of the invaluable role Tony has performed over the last 4 years in running the group, Mike presented Tony with a book token.

Stephanie Pengelly, a trained social worker who runs the help line and has had a somewhat difficult year herself, reported another busy year with calls from new members and those with problems from time to time. Stephanie has details of holidays for people with disabilities.

Sally Langton, who is responsible for Group Awareness, reported that she had regularly been in contact with external bodies to publicise the group. Following an article in Woman's Weekly, enquiries were received from 36 readers, 20 of who have FSP and 13 of who joined our group.

Tony then introduced David Pearce, the new secretary and Ian Smith the newsletter editor. Ian said that the newsletter would remain a quarterly publication and asked for any input that might be of interest to the group. Each of us has unique experiences and sharing of information is critical to a self-help group. David reiterated this point and said that he would endeavour to expand the group over time.

In the general discussion that followed the formal introduction, the meeting agreed that we were not catering fully for the younger members with early onset of FSP and that we would seek ways to help them further. The possibility of local meetings was discussed and it was agreed that this was really down to individual members of the group following the publication of the address list, which accompanies this newsletter. A particular difficulty was raised regarding access to experienced medical staff and Stephanie agreed to offer individual advice as necessary.

A number of presentations then took place. Firstly Paul Clayton from Worldwide Mobility (Products) spoke on the range of mobility aids that might be of use to members of the group and had a display of such aids in an accompanying room.

A question was asked regarding adaptations to help pull wheelchairs up hill or slow them down going down hill and Paul said that such a device, controlled from the handlebars, was available for use on some chairs. Paul also confirmed that there was a range of "modern" chairs for children and said that the voucher scheme could help people select an appropriate chair (see page 4).

Stephanie then introduced FRODO, her canine partner. Canine partners are available, following assessment, to disabled people over the age of 18 who can exercise the dog and generally look after it. Costing £5,000 to train, each dog is able to respond to a number of commands and can, for example, retrieve items from the floor or seek help from neighbours if their owner has difficulties. They are also, of course, company and Stephanie has further details.

Before lunch, those of us who were prepared to brave the cooling weather then posed for a photo and, if technology permits, a future edition of the newsletter may contain the results.

Following lunch, there were presentations from two doctors. Dr Tom Warner described the wide range of causes of spasticity, including multiple sclerosis, strokes and FSP and explained that there are two forms of FSP, pure and complex. In the former case, the primary symptom is stiffness in the legs with possible bladder dysfunction, and in the latter case there may be additional complications perhaps affecting the skin, sight, hearing and hands. Within individual families, there would generally be consistent symptoms.

The condition is generally slow but progressive and can arise in childhood or later in life (although there would probably be early indications). It is caused by a faulty gene leading to the premature death of nerve cells and resulting deterioration in the messages getting from the brain to the legs. A feature of FSP, when compared to other causes of spasticity is that the strength in the legs is preserved.

Dr Warner then described the treatments (but not cures) currently available. These include:

  • Physiotherapy where stretching in a controlled way, under the guidance of a neurological physiotherapist, can help delay contractions.
  • Drugs, including baclofen and more recently zanaflex, which can ease the stiffness in the legs although they have side effects such as causing drowsiness. These drugs can be delivered orally or directly to the base of the spine using an internal pump (which has not been commonly used in FSP)
  • Operations on the affected muscles which is not common
  • Botulism toxin, which temporarily paralyses the muscle although it can only be administered in small doses due to its toxicity and cannot be used to treat some of the larger muscle groups in the leg.

Dr Warner said that genetic research is the way forward and that a £40,000 research grant has recently been provided. To take the research forward, the team need to investigate individual families, within which the same faulty gene will operate, and look at healthy and afflicted individuals to isolate the relevant gene. They are particularly interested in large family groups and their address is Dr Tom Warner, Dept of Clinical Neuroscience's, Royal Free Hospital Medical School, Rowland Hill Street, London NW3 2PF with a phone number 0171 830 2951.

Dr Paul Hart from the Royal Free Hospital then described the current genetic research and explained that it was a complicated process with many steps that necessarily take time to complete. He explained that the faulty gene appeared to result in the cells being unable to generate energy resulting in their death and showed that there were several suspected sights for the faulty genes. In one particular rare instance of FSP, the gene has been isolated and there are indications that another UK research group might have isolated another FSP gene.

However, even if the genes are found, there are still substantial difficulties in offering a treatment that will replace faulty genes with their healthy counterpart. Paul was unable to offer any indication as to when treatments might become available although modern computer techniques were helping the process. At present, it is impossible to test for the faulty genes.

Dr Warner reassured the group that the various research groups around the globe share their results, to avoid duplication, and that the frequency of the condition is of the order of 1 in 10,000 people.

The Wheelchair Voucher Scheme

At present anyone referred to a Wheelchair Service will be assessed by a NHS therapist or other qualified professional and offered a wheelchair, which meets their clinical needs. This will be provided free on loan for as long as it is needed and the NHS will meet any maintenance and repair costs.

If, from this assessment, the prescribed wheelchair is not acceptable to the client the voucher scheme may then be offered. The therapist will discuss and agree with the client the most appropriate alternative wheelchair, which may be purchased, under the voucher scheme, from an appointed supplier. The voucher will be of equivalent value to the NHS wheelchair with the user paying the balance of the cost and maintenance costs; alternatively, a voucher of lower value could be offered with the NHS meeting maintenance costs.

PLESASE NOTE THAT THE VOUCHER SCHEME DOES NOT RELATE TO THE PROVISION OF POWERED WHEELCHAIRS which are available from the NHS to severely disabled people who are unable to walk or self propel a manual wheelchair within their home environment but who can show that they are able to operate such a chair safely inside and outside their home.

The above is necessarily a summary. Please ask your local NHS wheelchair service for more information.

The Newsletter - Our Future Together

This is the first newsletter I have produced and future editions will evolve over time with some ideas I have based on my own experiences and those I know you will have from your experiences. As a key aim of the group is self-support, I would encourage you to let me have any information or personal histories that might be interesting to our colleagues.

You can of course contact me by conventional means and, at some stage, by e-mail. Some historic copies of the newsletter are on the World Wide Web, for those of you with access, but more recent issues have yet to be added.

I am keen to develop a Q & A section and have asked Stephanie, who mans the help desk, to let me have any such points we can share. They will of course be anonymous. Again, if you would like to throw questions at us please do and we will attempt to find answers for you.

I have a range of really fun pictures that will fill some space so watch out!!

Other News

Family Records

Thank you for the family records received to date. Every- ones family history is valuable and, whilst we may not benefit personally from current research, we must do everything in our power which may benefit younger relatives and future generations.

There is no deadline so please return your form to Audrey Town if you have not already done so. It does not matter if there are gaps in the information you can provide; together we can achieve 100%. Audrey's phone number is above if you need help.

FSP 1999 Survey Results

Those of you who completed the 1999 survey will find any updated results enclosed.