Guide to Huntington’s Disease

Huntington’s disease (formerly known as Huntington’s chorea) is an inherited condition causing progressive brain damage.

The disease is caused by a faulty gene, which causes a protein called huntingtin to be produced in the brain. This protein damages and ultimately kills off brain cells.  As the disease progresses, it leads to depression and psychiatric problems, uncontrolled movement (from ‘chorea’ – the Greek word for dancing), problems with eating and swallowing, behaviour changes, memory loss and poor cognition.

Facts & figures

Around 12 people out of every 100,000 in the UK have Huntington’s. The disease affects both men and women and it’s possible to develop Huntington’s at any age. If symptoms develop before someone is 20 years old, the disease is referred to as Juvenile Huntington’s disease (JHD). Thankfully, this is pretty rare, representing only 5-10% of people affected with HD. Typically, most people who develop problems are diagnosed between the ages of 35 and 55 years old. The condition generally progresses for around 10-25 years.

Symptoms and diagnosis

Although symptoms of HD can vary, the progression of the disease is fairly predictable. The NHS Choices website has a good summary of the various features of HD. Early symptoms, such as personality changes, mood swings and unusual behaviour, are often subtle and can be overlooked.  Patients can often alternate between excitement and apathy, and depression and excitement. A general lack of coordination becomes more apparent. Patients can be unsteady on their feet and develop jerky body movements. Loss of motor control can make eating and swallowing difficult. Behavioural changes become more pronounced and there is a gradual decline in communication and mental abilities. Some patients may develop dementia.

Although a genetic test for HD had been available for people with a family history of the disease since the early 1990s, some people prefer not to know if they are carriers.However, finding out may give people more time to get appropriate treatment and come to terms with the disease.

How is Huntington’s treated?

Unfortunately, there is no cure as yet for Huntingdon’s, but research proceeds worldwide and there have been some exciting developments recently. In the meantime, some aspects of the disease can be managed successfully with medication, specialist therapies, and keeping as active as possible.

Medication may be prescribed to control involuntary movements, depression and mood swings. Speech therapy can help with communication and swallowing issues, physiotherapy can help maintain balance and mobility.

Local authority social services can provide occupational therapy to help can make daily living much easier with home adaptations and equipment.

Potential good news: Breakthrough gene silencing research

 In October 2015, the first human trial of a drug that may turn off the faulty gene that causes HD was begun, involving 36 HD patients in Canada, the UK and Germany.

Known as ISIS-HTT, the experimental drug is a type of ‘gene silencer’ and targets the huntingtin gene in the patient’s DNA to stop it making the destructive protein that causes HD. Previous trials of the drug in mice resulted in significant improvements of symptoms.

The first human volunteers will be studied over the next year to help plan for future HD gene silencing studies.

Help and support for people with Huntington’s

As well as your GP and local social services there are some specialist organisations which offer specialist advice and support.

The Huntington’s Disease Association (HDA) is a national charity, which supports people affected by the disease and also works with health professionals to provide information and advice.

The HDA website has a wealth of information about the disease, including a wide range of really useful and detailed Factsheets.

In addition, the HDA operates a Central Care Information, Advice and Support Service  (available Monday to Friday during normal office hours) via their head office on 0151 331 5444.

As well as offering information and advice to families, the HDA advisors can answer crisis calls, liaise with other professional service providers, and help identify suitable respite and residential care.

To help HD patients and families help and support each other, the HDA has local branches and support groups throughout England and Wales.

For people north of the border, The Scottish Huntington’s Association offers great support and advice.

The Royal Hospital for Neuro-disability in Putney, London, provides a wide range of treatments both at the hospital and in the wider community. They also offer support for patients of Huntingdon’s and their families.In particular, the hospital provides a team of healthcare professionals who can provide Electronic Assistive Technology (EAT) for people with neuro-disabilities including Huntingdon’s.  Examples of such equipment include IT hardware and software, communication aids, environmental controls, and powered wheelchair controls.

Other useful links:

NHS Choices: Huntington’s Disease

Huntington’s Disease Association

The Scottish Huntington’s Association

Royal Hospital for Neuro-disability


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