is very little medical literature on the care of the Huntington's patient. This partly reflects
the fact that in many countries there are no designated clinics for this
patient group, and within some countries the care provided varies widely
(Simpson, 2004). Systematic clinical guidelines for care have not been
developed since the evidence for any particular best practice is lacking.
studies have shown that simply attending for review as part of a research study
produces an improvement in some areas of the HD disease process. Over the last
15 years, I have been running a management clinic to care for those affected by
HD, and those who have the mutation for the disease. Regular attendance to monitor progression of
the neurological features, drug treatment of movement disorder, chest signs,
swallowing and circumstances of care appear to have improved quality of life
and reduced crisis management. Mutation carriers are seen to monitor for
commencement of disease, and to involve them in research at their request.
of the pathophysiology of Huntington's disease is an essential component of the
research into its cause and prevention. Without detailed clinical information,
these data cannot be collected. Regular assessment and documentation of stage
of disease is essential for this process. Any trial of therapy or preventative
tool will depend on the ability to properly compare progress before and after
intervention. Management review clinics allow for the collection of such data.
addition it is clear that those who are as yet asymptomatic, but who have the
mutation of HD, are rarely offered comprehensive follow up and assessment. This
is an important group and their needs are significant. Information about
research programmes and testing of pregnancies are but two of the issues raised
project is to document practice, and the different approaches around Europe to the management of HD with a view to developing
evidence based guidelines for best practice.