THE UK AND IRELAND HUNTINGTON'S DISEASE ASSOCIATIONS NEW ALLIANCE - WORKING AS ONE

The president of Ireland launches new alliance in Belfast on MONDAY 12TH OCTOBER 2009

The four Huntington’s disease charities throughout the UK and Ireland have come together to form The UK and Ireland Huntington’s Alliance’. The aim of the collaboration is to make as many people as possible aware of this rare disease and its devastating effect on whole families. The UK and Ireland Huntington’s Alliance aims to:

• Shape the debate on the care and support of people with HD and those affected by it
• Work with young people from Huntington’s families in order to educate and prepare them for the years ahead.
• Provide opportunities to share information and intelligence
• Act collectively where appropriate

Mrs Pat McKay, Chair of the Huntington’s Disease Association Northern Ireland (HDANI), welcomed President of Ireland, Mary McAleese who is Patron of the Huntington’s Disease Association Ireland, Sarah Winckless, Olympic Medallist, Double World Rowing Champion and Patron of the Scottish Huntington’s Association, along with senior executives of Huntington’s Disease Association (England/Wales) to a reception in the Ormeau Baths Gallery Belfast on October 12th, 2009.

In a message to the Alliance, HDANI Patron, Baroness Blood applauded the initiative of the four Huntington’s Associations, in uniting to formthis Alliance of common purpose saying that, “I strongly believe that the Alliance have got it right in focussing on tackling ignorance about Huntington’s at all levels, from the health sector itself to the street.  Ignorance breeds prejudice, and leads to isolation, bullying, stigma, and great suffering.  We should not tolerate this. We must use every means to provide information and foster understanding, and work together to shape a better, more caring and inclusive world.” 

President of Ireland, Mary McAleese,gave the keynote speech at ther launch event and referred to the positive aspects of building bridges, not just across the border in Ireland, but across all borders that represent a barrier to supporting families everywhere with Huntington’s disease. 

Sarah Winckless, World Gold and Olympic Bronze medallist also attended the press conference to talk about the importance of providing young people living with, or at risk of, Huntington’s disease with the information and strength they'll need in the years ahead and how the Alliance will help to achieve that aim.

Sarah Winckless, who has herself tested positive for the Huntington’s gene, believes: “An important focus for our fundraising efforts is to support activities for children and young people from Huntington’s families, particularly those which aim to strengthen self esteem and self confidence, promote participation and engagement and build connections both within and outside the Huntington’ s community.”

What is Huntington’s Disease?

Huntington’s disease is a neurological disorder, a disease of the brain. It is a hereditary disease which is caused by a faulty gene on chromosome 4. In some way, which is not yet fully understood, the faulty gene leads to damage of the nerve cells in areas of the brain, including the basal ganglia and the cerebral cortex. Since so much of the brain’s activity passes through these areas, the death of these cells affects virtually everything about a person, including movements, moods and thinking processes.

Symptoms usually begin between the ages of 30 and 50 years, though they may occur at anytime from childhood to old age. An individual may have the disease for around 15-20 years however some people may have the disease for a longer or shorter period of time.

Who is affected by Huntington’s Disease?

For those whose lives are touched by this disease the effects can be quite devastating.

In the UK and Ireland, there are about 6,500 people with Huntington’s disease at any one time. Every child of a parent with Huntington’s disease has a 50% risk of inheriting the defective gene. It is not just the individual with Huntington’s disease who needs support and help, but often other family members too. Family members may also play a major part in caring for the person with Huntington’s disease.

Some of the ways in which the disease affects the individual

Each individual has complex and unique needs with no two people being affected in the same way. For example, one person may have a rapid deterioration in thinking processes or behaviour and less decline in the control of movements. Another person may have rapidly increasing difficulty with movements, but no significant change in thinking processes or behaviour during the same period of time.

Sometimes the symptoms of Huntington’s disease are present for a long time before a diagnosis is made. This is especially true when people are not aware that Huntington’s disease is in their family.

The early symptoms can be subtle at first and may include:

• Small changes in co-ordination and some minor involuntary movements.
• Lack of concentration and short-term memory lapses, together with some difficulty thinking through problems and adapting to changing situations.
• Changes in mood could include depression, irritability or aggressive outbursts.
• Various mental health problems may be experienced, such as obsessive compulsive thoughts and behaviour, hallucinations and delusions and a significantly increased risk of suicide.

As the disease progresses movement, balance, co-ordination and speech production becomes increasingly troublesome and frustrating for the individual and the carer. The individual with Huntington’s disease sometimes suffers from a lack of insight or self awareness, and is unable to recognize changes occurring in him/herself. The person eventually requires more and more help and support in managing the activities of daily living such as washing, dressing, eating, drinking and mobilising. In the later stages the person may become completely dependent on others to meet their basic needs. Despite problems with cognition continuing to advance the person usually retains a strong sense of self-identity, is oriented to where and when they are and retains the knowledge and awareness of loved ones and familiar faces.

Genetic Testing for Huntington’s Disease

There are complex ethical issues for families living with Huntington’s disease including whether to be tested for the gene. A test has been available since 1993, but less than 15% of people across the UK, who are at risk of the disease actually have the test.

The principal reasons for this are that:

• there is no cure yet for Huntington’s disease
• knowledge of having the gene does not remove uncertainty over when symptoms may begin

• a positive test or a family history of the condition may make it difficult for people to purchase life insurance, secure a mortgage or pursue certain career options

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