Bulletin No. 3 – July 2005

WORKSHOP REPORT

What a great morning!  led by Prof. Garth Nicholson, Prof. of Neurogenetics, at the Molecular Medicine facility at Concord Hospital, prior to our AGM.

Garth proceeded to lead a discussion on genes, with diagram examples of recessive, dominant and X linked, and explained the complexity of  pinpointing the actual gene that applied to various families.  He also explained that HSP damages the nerves (which have the key role in muscle control) to cause the muscles to become over active. 

Garth, together with Dr. Andrew Ellis, Orthopaedic Surgeon from Royal North Shore Hospital, and Peter Lumb, Podiatrist, covered two different surgical procedures (illustrated by slides and then by the patients who had benefited).  Three HSPers took to the stage, barefoot to illustrate their physical disabilities, the first two having had different operations and physio, the third HSPer not having had any remedial attention.  Discussion interacted here between all parties, the medics and HSPers, with all parties gaining information one way or another.  While it was pointed out that the operation/phsyio will in no way cure HSP it was evident that it did give sufferers benefit.  Various types of footwear and the importance of flexibility was also discussed and illustrated with different types of footwear.

Thank you to all those who gave of their time at this workshop, to help HSPers understand, assist, etc in their condition.

A sandwich lunch was provided.  A special thank you to those who provided the goodies.

At 2pm we proceeded to our first AGM  (minutes attached to this Bulletin) and then progressed to our Charter.
   

OPEN DISCUSSION ON HSP CHARTER / STRATEGIC DIRECTION

The original draft Charter was prepared very early in the formation of the HSP Research Foundation and hence without membership involvement.  There is a need for the membership  to develop its Charter.

The discussions were led by Frank McKeown who is an organisational change consultant and has recently returned to Australia after living in the USA for many years.  He followed the formation of the Spastic Paraplegia Foundation of USA (formed in 2002) and understands HSP.  He is working with us on a pro bono basis (professional services free of charge).

Frank opened the discussion by explaining that the meeting was to make a start on setting a direction for the organisation and that this meeting will be followed by the inclusion of people who did not attend the meeting (mostly Victoria & South Australia people) in the process.

Following the introductory getting to know each other session, the work groups discussed and charted the outcomes of discussion on –
*where we are, and
*where we want to be
and then the groups began to develop the draft charter within a framework of mission, vision, core competence and values.

The output of these discussions is enclosed with this report.  As mentioned, developing the strategic direction of the Foundation is something to which we want everyone to have the opportunity to contribute.  So for those not at the meeting, we welcome and value your thoughts, reactions and input as this work-in-progress is refined, developed and improved.  Please read  ‘Developing our HSP Charter / Strategic Direction – Stage 2’ also enclosed and take this opportunity to have your say.

THE PAUL NEWMAN FOUNDATION

The Paul Newman Foundation invited submissions for funding support from charities including health support groups.  A joint HSP Research Foundation and the Genomic Disorders Research Centre application was submitted with the aim of obtaining a grant of $21,000 for the detection of gene variations which cause Hereditary Spastic Paraplegia in individual patients.  The objective of this grant is to determine a protocol for the detection of gene variations that cause HSP disorders and to achieve this within a cost to the patient or health fund of $1,000 or less per patient, making it affordable to all.  A successful outcome for this project would impact on hundreds of rare diseases in Australia and world wide.

The need for the funding of the project was based on the fact HSP causes the ends of the nerve cells which control lower extremity muscles to deteriorate.  This results in a loss of function in these muscles and the need for walking assistance or a wheelchair.  It progresses over several years from slight gait impairment and onset and usually occurs in early adulthood but varies widely.

For an HSP sufferer in Australia to benefit from any therapy that arises from overseas research or for them to participate in an approved pre-implantation genetic diagnosis programme, it is essential the gene variation causing their particular disorder is known.  At present no Australian laboratory has a cost effective diagnostic protocol that can identify HSP gene variations.  Indeed no protocol is offered by anyone.  Local research is needed to meet that need, hence, if this funding application is successful, there would be a substantially increased capability to fight this debilitating disease in the years ahead.
 
The submission was made under the Genomic Disorders Research Centre deductible gift recipient status prior to 30 Jun 2005.

Robin Bligh
President