Dr Eddie Murphy Huntington’s Disease
My first career was as a general nurse and I remember nursing a man with Huntington’s Disease in the UK. I had little understanding of the disease but was always curious and aware of the ripple effect and the immense anxiety that it provokes. Nursing this man always stuck with me. A diagnosis of Huntington’s Disease (HD) is a devastating experience.
Huntington’s disease is a relatively rare, degenerative disease of the brain, first described by George Huntington in 1872. Symptoms affect motor ability (e.g. posture, uncontrolled voluntary and involuntary movements, sustained muscular contractions, and difficulties with speech and swallowing): behaviour (e.g. anxiety, depression, irritability, anger, OCD, sleep disturbance): cognition (e.g. the gradual impairment of comprehension, reasoning, concentration and memory.) HD robs the individual of the self which vary from person to person and at different stages. Life expectancy is approximately 20 years after onset. In the later stage of HD people will need total support.
Each child of an affected parent has a 50% chance of inheriting the illness. Approximately 750 people in Ireland live with HD with a further 3000 at risk. Symptoms usually start between 35 and 50 years old. About 5-10% of people have symptoms before age 20 (Juvenile HD) and 10% have a late onset after 60 years of age.
The Huntington’s Disease Association of Ireland offers information and support to people impacted by Huntington’s Disease: National helpline 1800 393939, www.huntingtons.ie.
For families living with this disease, a new diagnosis is heart-breaking. In families with no previous history, the diagnosis is difficult to comprehend. This transpired for Anne who was pregnant with her third child when her motherin-law was diagnosed. When Anne’s husband received his predictive test result three years later, their young family faced a stark reality. “Prior to his test I was shocked by sudden out of character changes in his behaviour. My once considerate and loving husband appeared ‘selfcentred’ at a time when our children required priority. The diagnosis was hard-hitting but looking back there were signs before this test result.”
Initially Anne and her husband needed time to process the diagnosis and the implications for their children, all under 10 years of age. “We contacted the Huntington’s Association and got as much information as we could. Next began the process of dealing with the shock of the diagnosis. Taking time to come to terms with it allowed us to move forward. Meeting other families through the HD Association events was educational, helpful and empowering and also gave us a feeling that we were not alone. You cannot underestimate the power, when in crisis, of talking and being with people who are living a life similar to yours. People who are further along the HD road offer advice and wisdom. The Association also informs and guides you about access to services you will need.”
A few dedicated health professionals in Ireland are liaising with international experts to enhance care and progress research here. Bloomfield Hospital Services, www.bloomfield.ie, offers a multi-disciplinary care service.
If you have concerns, contact the Huntington’s Disease Association of Ireland, www.huntingtons.ie. National helpline 1800 393939