Completed 17.11.07
Kitesurfing for Huntington’s disease
The sponsored kitesurf from Tenby to Pendine was in aid of The Paul Murch Huntington’s Disease Trust Fund.
Paul is a local lad from Tenby who has inherited Huntington’s and the monies raised will help Paul in his later stages of the disease.
The sponsored kitesurf from Tenby to Pendine took place on Saturday morning setting off from the south beach Tenby at 9.30am. With two support boats in toe this charity event was performed by Marco Hernandez, Kevin Cheeseman and Nick Merrony.
With strong SSW winds of 20mph increasing to 30mph, the crossing took 1 hour and 40 minutes.
This challenging day under tuff conditions raised approximately £1,500
This event could not have taken place without the support from local businesses such as: Walk on Water, Pembrokeshire on the web, Phonescope, The Hean Castle Pub, The Bush Inn, The Forum Cafe, Five Ways Garage, New Hedges Mini Market and The Normandy Pub.
Also a big thank you to all the local people of Tenby and the surrounding area who were happy to puts there hands their pockets for this worthwhile cause.
Most importantly this event could not have taken place without the two safety boats supplied by Tenby Harbour and Tenby Sailing Club. A big thanks to our skippers and assistants onboard that made it possible.
This sponsored kitesurf will become an annual event with talks of a crossing to Cornwall next year.
You can show your continued support by either donating money or buying t-shirts from Walk on Water (Saundersfoot or Tenby Shop) kindly produced by Sweet T’s Printing and Embroidery with all proceeds going to the charity.
For constant updates on this and future events, please contact one of the following:
Kevin (Cheesemyster)
07960 086 219
Nick Merrony (Fruity)
07973 405 656
Marco (Pedro)
07917 677 595
To find out more about this worthwhile cause, please read the information below.
Huntington's Disease
Published by BUPA's Health Information Team
February 2004
Huntington's disease is an inherited disorder that affects the brain. It results in a progressive loss of the control of movement, changes in personality and mental ability.
Around 4800 people in the UK are living with Huntington's disease. It is also called Huntington's chorea. Chorea means jerky, involuntary movements - a main symptom of the condition.
How it develops
The symptoms usually start between the ages of 30 and 50. Symptoms can then develop over a period of up to 20 years, but the illness invariably causes premature death. Although there is currently no cure for this disease, there are ways to manage symptoms effectively.
Causes
Huntington's disease is caused by a faulty gene and runs in families.
Most genes in the body are present in two copies, one from the mother and one from the father. The gene (which is called "huntingtin") that causes Huntington's disease is dominant. This means that if a person inherits one copy of this gene from either parent, they will go on to develop the disease at some point in their life.
A person who has one parent with Huntington's disease has a 50% chance of inheriting the faulty gene. This risk is 50% for each child and is not altered by gender or whether brothers and sisters are affected. Only people who have the faulty gene are capable of passing it on to their own children.
Very occasionally some people can develop the condition even though there is no family history of it. This is usually because previous generations were not diagnosed as a result of early death from other causes, or loss of contact through adoption.
Huntington's disease causes damage to brain cells in the areas of the brain involved in the control of movement, planning and motivation.
Symptoms
People usually have symptoms for up to 10 years before they find out they have Huntington's disease. Most people are diagnosed between the ages of 30 and 50, although this can happen much earlier or later. Symptoms are often overlooked, as they are mild and commonly experienced by well people. They include:
- mild tremor
- clumsiness
- lack of concentration
- difficulty remembering things
- mood changes, including depression
- sometimes, aggressive antisocial behavior
As the disease continues, the symptoms become progressively worse, and lead to incapacitation.
Symptoms fall into three categories:
Physical symptoms include chorea (involuntary movements of the limbs, face and body). Chorea may lead to difficulty walking, speaking and swallowing. Choking is a particular hazard, due to reduced ability to control the muscles of the tongue, neck and diaphragm. People often lose weight because they have difficulty eating and burn more calories due to the continuous movement.
Emotional symptoms include depression, not only because of the burden of having a progressive disorder, but also as a direct result of the damage to certain brain cells. People often become frustrated at being unable to work or carry out previously simple tasks. They also seem to behave stubbornly, probably due to a reduced ability to react flexibly and to understand the needs and emotions of others. People with Huntington's may also become more irritable and antisocial than usual, or make inappropriate sexual advances.
Cognitive symptoms include a loss of drive and initiative. People with Huntington's may appear to be lazy or uninterested in life, spending days doing little or neglecting personal hygiene. They may also lose the ability to organise themselves, as planning skills and ability to carry out more than one task at once deteriorate. In later stages, people may get memory loss and be less able to understand speech.
The behavioural changes that occur in people with Huntington's disease are often the most distressing for them, their family and their carers. People with previously full and active lives gradually lose their independence and mobility. At the same time, their personality may become gradually more self-centred and apathetic, straining personal relationships.
Juvenile Huntington's disease
A juvenile form of Huntington's disease also exists. Symptoms start before the age of 21 and the condition is usually inherited from the father. Symptoms are similar to those of adult Huntington's, but develop more rapidly and include muscular rigidity and fits.
Treatment and Support
Unfortunately, there is currently no cure for Huntington's disease. However, drug treatments are available to help people manage some of their symptoms. There are drugs available to treat abnormal movements. Antidepressants such as fluoxetine (eg Prozac) can be helpful with depression, and mood stabilisers and antipsychotic drugs can help with some of the emotional disturbances.
The general health needs of a person with Huntington's are also important. Dieticians can advise on adequate calorie and nutrient intake. Social and psychiatric support can help with family relationships and antisocial behaviour.
Scientists have investigated a number of drug treatments to slow the progress of the disease, but these have not yet shown any major benefit.
Some clinical studies have involved using stem cells (the most basic form of cells from which others develop) to grow cells that can be transplanted into the brain of a person affected by the disease. This approach may eventually improve the outlook for those who possess the faulty huntingtin gene.
Genetic Tests
Tests are available to identify whether someone has the faulty gene. These fall into three categories: diagnostic, pre-symptomatic and antenatal.
Diagnostic Tests
These are carried out in people who have symptoms of Huntington's disease who come from families with members who have the condition. The aim is to confirm a diagnosis.
Pre-Symptomatic Tests
These tests are carried out in people who are not showing symptoms of Huntington's disease, but have a family history of it. Taking such a test tells the individual if they will develop the disease, but not when this might happen.
The decision to take a test is a serious one: a positive result can be devastating since it tells the individual that they will one day become severely mentally ill.
There are also issues surrounding testing when the individual's parents have themselves not been tested, since a positive result indicates that one parent also has the faulty gene.
Advice from a genetic counsellor about the implications of taking the test is needed before going ahead. They are not available in the UK for people under 18 years old.
Antenatal Tests
These are carried out early in pregnancy on the unborn children of couples from families affected by Huntington's disease. They can be used to calculate the risk of that child going on to develop the disease in their adult life. Again, the implications of a positive result are serious, and couples need advice and support from a specialist doctor or counsellor to help them in their decisions.
Further Information
Huntington's Disease Association
020 7223 7000
http://www.hda.org.uk/
