Science Interviews


Thu, 20th Feb 2014

Living with Huntington's Disease

Richard Price

Part of the show Naked in a Brain Bank

In last month’s podcast, I took a trip to New Zealand to find out about Huntington's disease or HD.

Richard -   My wife now Heather is in residential care.  She has what has turned out to be a very aggressive form of Huntington's that has affected not only her cognitive side and her mobility, but also her mental state.  She started off with not being able to handle stress, even the smallest of the issues can make her quite upset.  Also, in terms of depression, she suffered from that a little as well.  But just in the last 6 months, she’s been declining in terms of her mental state and where she currently resides there as between 4 and 6 people with Huntington's and they all are so different.  You've got one person that, you just wouldn't realise.  In fact, the GP probably wouldn't realise that they have HD.  You've got other people that have the movement issues and you've got people with the emotional and the cognitive issues.  Unfortunately, Heather is ahead all three.  Of course, Heather, having watched her mother go through what she’s going through, it’s been really quite tough on her.

Hannah -   Thanks to Richard Price who’s talking about how his wife Heather has been affected by Huntington's.  It’s a complex disease that affects one in every 30,000 people worldwide.  We found out how changing a single gene is responsible for the myriad symptoms associated with Huntington's.

Russell -   I was one of the members of the group that found the gene, now 20 years ago.  On average, if you inherited the sort of tract, you can imagine that as like a bicycle chain that gets longer.  If the repeat is longer, then the age of onset is lower.  So, you can draw a curve.  So, if you're unfortunate enough to inherit a very long repeat say, over 60 or 70 units then the age of onset tends to be younger than 20.

Hannah -   How a genetic test can affect how people affected lead their life.

Richard -   She’s 43 now.  She was tested when she was 18.  At that stage, they'd only just discovered how to actually discover the gene and test for it.  Heather is a very positive and proactive person so she wanted to know whether she was going to have the symptoms later on in life.  It was quite tough, but she got through it and in fact had helped her knowing rather than not knowing.

Hannah -   Did she ever mentioned that as a result of having this test and knowing that she was going to develop Huntington's down the line, do you think she led her life a Holding Handslittle bit differently because of that?

Richard -   Differently.  In fact, that was her purpose.  Everything from deciding to get a diploma rather than a degree, so rather than going to a university and really, just getting into the work force and really, just building up a nest egg when the symptoms really started to appear.  She was able to manage that, understand it, and then make some decisions around it.  So, she always had planned on travelling.  She sold her house and went travelling and I met up with her in London.

Hannah -   Do you have children with Heather?

Richard -   No, that was another sort of decision that she made quite early on because it seems that her family may be more prone to passing it down the line.  Her brother has it as well so yeah.  Heather had a lot of time to sort of process and accept that and we’re both at the same mind.  So, we had dogs instead of children.

Hannah -   And I met brain scientist studying sheep to try to understand the disease.

Jenny -   Sheep in a flock can be very silly.  You get a sheep by itself working under controlled conditions.  They perform very well.  They're very intelligent, a little bit moody, but they understand things, they learn very quickly.

Hannah -   I've heard that you've even managed to get some of your sheep playing games of football.

Jenny -   The sheep don't play football with each other, but I did teach sheep how to kick a ball into a goal because I'm looking for tasks of skill and motor coordination and also wanted something that sheep didn’t normally do.  Now, sheep don't normally kick a football.  If they see a football on a field, they would be afraid of it because it was weird.  But I taught a sheep how to kick a football because I know that it’s a good test of balance, coordination and skill.

Hannah -   And how they're using genetically modified flocks of Huntington sheep to help develop new treatments for patients. 


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