Huntington's Disease Awareness Month
Huntington's Disease Awareness Month
May is Huntington’s disease awareness month, promoted by the Huntington's Disease Association. Huntington’s disease is a rare genetic disease that causes the progressive degeneration of nerve cells in the brain. Damon Butcher, who was diagnosed at only 23, sat down with us to talk about what life is like with Huntington’s disease and how he manages his symptoms. Damon works for the NHS and is also an ambassador for Campaign For My Brain. Damon told us about the stages of Huntington’s disease and his experiences with it.
What is Huntington’s Disease?
Huntington’s disease is an illness caused by a faulty gene in your DNA. If affects the body’s nervous system, resulting in a wide range of symptoms. As the disease progresses it has an impact on a person’s functional abilities, resulting in movement, cognitive and behavioural changes. Symptoms can present at any age, but for most people they develop when the person is in their 30s or 40s. If the condition develops before the person reaches their 20s it is referred to as Juvenile Huntington’s. When the disease develops early, the symptoms are often slightly different, and it can progress faster.
Everyone’s journey through Huntington’s is unique. People living with Huntington’s may not experience all these symptoms or may experience different ones or experience them at different stages. Changes usually occur within three main areas: movement, cognition and behaviour.
Scientists around the world are researching ways to slow down or prevent Huntington’s disease.
Very early symptoms, often pre-diagnosis may include slight, uncontrollable muscular movements; stumbling and clumsiness; lack of concentration and short-term memory lapses; depression and changes of mood and personality. Many people can normally carry out all of their usual day-to-day activities and work without requiring assistance. One of the most common symptoms to appear first is mood problems, and issues with the person’s mental health. This was the case for Damon. He told us how the first thing he noticed was the decline in his mental health,
"I was probably the most abrasive I’ve ever been in my life.”
This is when the symptoms become noticeable enough to make a diagnosis. More symptoms will present such as tremors, though these are usually subtle at this stage and may be mistaken for nervous tremors. Although most people will be able to carry on with the majority of their activities of daily living without issue, some may now become more difficult and require assistance and support either via assistive technology or support from others. For Damon what was most noticeable was his increasingly poor memory.
“One time I put a pizza in the oven and Ian (his now husband) came home to the fire alarm going off because I’d forgotten about it and gone to take a nap.”
When a person reaches the middle stages of Huntington’s disease it becomes much harder for them to carry out their normal daily activities unaided. Help is needed both physically, and for cognitive chores, as there can be a drastic worsening in the ability to think and remember clearly. Speech may also become affected and the ability to swallow may become difficult. As Damon has experienced, mobility can be affected at this stage.
“I use a walking stick any time I go out for a walk anywhere as my balance is really bad now.”
Many people may use a stick, walker or a wheelchair.
Another symptom that can become evident at this stage is the nerve damage that prevents the brain perceiving pain the same way.
“Because my brain can’t feel the pain, I was always burning myself on cigarettes and hot drinks.”
Products such as protective clothing can help prevent your loved one from hurting themselves.
In the later stages of the disease more care and support will be needed. Movements can become extremely slow and rigid, and people with HD are often unable to communicate because of difficulty speaking. Difficulty chewing and swallowing can also necessitate the use of a feeding tube. Difficulty falling asleep or staying asleep is often experienced, amongst other symptoms.
There are a range of things you can do to support or help someone with many of these later symptoms.
It can be difficult to know what pieces of Assistive Technology could be helpful to someone living with Huntington’s disease. As is often the case with degenerate conditions, finding out what aids or adaptations might be helpful really depends on what you can do and where you are facing barriers. Use AskSARA can help guide you to the right advice and information on equipment by selecting an area of daily life and answering a few questions about the areas that you might be finding difficult. AskSARA then provides OT written advice to guide you to the categories of products that might be helpful to you depending on your answers.
For example, communication devices, such as communication boards, some of which can even function using eye movement alone or apps for the person’s phone can assist when communicating has become an issue.
“You manage the symptoms not the condition”
There is no cure or treatment for HD, so, as Damon describes, you have to treat the symptoms that a person experiences rather than the condition as a whole. There are many organisations out there that can help with this, such as the Huntington’s Disease Association, which supports people living with Huntington’s Disease in England and Wales.
If you or someone you care for is living with Huntington's disease or has recently been diagnosed, the Huntington's Disease Association has resources to help support you, including specialist Huntington's disease advisors.