Huntington’s Disease: The Facts

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Huntington’s disease

Huntington’s disease (formerly known as Huntington’s Chorea) is a degenerative condition. The disease damages brain cells. It causes involuntary movements, speech disorders and progressive dementia. As the brain is irreparably damaged, sufferers experience a range of symptoms.  These also include mood swings, a change in behaviour, problems with cognition and being able to make judgement calls. Paranoia can become a problem, and sufferers may resist help at times, believing people’s intentions are coming from a bad place. Delusions can be common, too.

Huntington’s is caused by the presence of an inherited affected gene. Humans have 46 chromosomes. It’s a problem with chromosome number four that causes this debilitating disease. A normal chromosome number four makes a protein called Huntingtin, but a faulty one malfunctions and causes problems. It has a harmful amount of something that Doctors call CAG repeats. This region is bigger than normal and produces a mutant version of Huntingtin. What this results in the healthy cells are attacked by the defective gene. It effectively kills off brain cells over time.

How is Huntington’s Treated?

At present, there is no cure for Huntington’s. It’s a cruel, debilitating disease that can’t be slowed down, halted or reversed. Studies continue to try and find better treatments and cure, but as it stands, symptoms can only be managed to an extent.

Taking regular exercise is a way to help combat the effects of Huntington’s. It has a positive impact on a sufferer and someone who manages to get moving out and about will feel much better. If no exercise is taken, the effects may be more pronounced.

Occupational Therapy and Language and Speech therapy are used to help sufferers. This means they can retain use of their vocal skills and swallowing abilities for longer.  Breakthroughs in technology mean that online speech therapy has become useful in managing symptoms. It’s flexible and can be helpful for people who find getting out a struggle.  Someone with Huntington’s may need help as the disease progresses in managing their everyday lives.

Medication prescribed for Huntington’s usually comes in liquid forms to ease swallowing. This can become a problem as the condition advances. The side effect of the medications that are effective in managing Huntington’s is that they usually have a range of harsh side effects. These include extreme fatigue. This can be problematic; it is often near on impossible to tell if this tiredness is part of the condition or a side effect of the vital drugs.

Other Complications

If you have Huntington’s disease and want to have children, it can be a difficult decision to make. There is, of course, a big chance that you could pass the condition on to your child. The nature of the disease and ethics combined mean that you can’t generally test a child before the age of 18. Many people who have the condition choose not to have children because of the risk of passing it on to their offspring.

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