The Teen Years
Huntington's Disease (HD) is a hereditary illness that causes damage in a certain part of the brain. This stops the brain and body from working as well as they used to, and affects a person's movements, thoughts and behaviour. People generally start to develop symptoms of HD between the ages of 30 and 50.
The part of the brain that is affected is called the basal ganglia (say: bay-sul gang-lee-ah). This is a very important part of the brain that co-ordinates a lot of the information that's whizzing around, and makes sure it gets to the right part of the brain. HD causes brain cells in the basal ganglia to deteriorate and die. This means that it can no longer co-ordinate information as efficiently, so the messages being passed through the brain don't always get to the right place, or get lost along the way.
Think of your mobile phone, sometimes you get a signal, no problem. But at other times, the signal gets cut off halfway through, just when you were getting to the good part of the gossip! At other times, you cannot get a signal at all.
Having HD is a bit like this, sometimes the messages get through and other times they only get a little of the way through. Other times they just don't get through at all. So some days are better than others. Some things affect the messages getting through, for example, if the person is feeling stressed this makes it harder for the messages to get through and things may become worse at times of stress.
The brain cells in the basal ganglia deteriorate over a long period of time, 15-20 years, and during this time the affected person slowly loses different skills and abilities. This happens gradually and they can be reasonably well for a long time.
For more info on how the brain works, check out www.kidshealth.org
How does HD affect people?
When someone has HD they may change and behave differently. They may not be able to walk and talk as well as before, and they may be more forgetful. Sometimes they may be more bad-tempered and get angry about little things. These changes take a long time and you might not notice them at first.
How do you get HD
You might have more than one person in your family who has HD. This is because HD runs in families – it is hereditary. This means it can be passed from parents to their children. People might say that you look like other people in your family – this is because of your genes. Genes are passed to you from your parents – that’s why you might have freckles like your dad, or brown eyes like your mum. You have about 30,000 different genes in your body.
Genes are sections of DNA carried on your chromosomes. These genes are all different, and are programmed with different information. They decide things like the colour of your hair and your eyes, how tall you are, etc. They are like a recipe book - your genes are the ingredients that make you uniquely you. Everyone has a different recipe - that’s why we’re all different shapes and sizes. Most cells in your body have 23 pairs of chromosomes. Each parent gives you 1 chromosome in each pair. Our genes are strung out along our chromosomes and there are 100s of genes in each chromosome. We have 2 copies of each gene as well – one from each parent.
People can be identified because of the differences in their genes and the DNA that makes them. DNA can be extracted from one cell of our body, such as a single skin or blood cell, or even a hair root. That’s why on your fave crime dramas, they search the crime scene for DNA evidence. People can then be identified by their DNA.
Sometimes genes don’t work properly. In people with HD,a faulty gene causes things to go wrong in the brain. If a parent has HD, they have 1 copy of the faulty gene and 1 good copy. They may pass either of these on to their children. Their children therefore have a 1 in 2, or 50 chance of inheriting the faulty gene. You may hear people saying you are “at risk” – this just means you have the 50chance of inheriting the faulty gene. If your grandparent has HD that means your parent is at 50risk. If they do not have the faulty gene, then you are no longer at risk.
The specific gene that causes HD was identified in 1993. It is located on chromosome 4. No-one knows exactly how this faulty gene causes HD and there is a lot of research going on all over the world to try and find out. Somehow having the faulty gene causes the build up of a protein (a type of body chemical) called Huntingtin in some of the brain cells which makes them deteriorate and die. Once we know how this works, then we can try and find a way to stop it happening or at least slow it down. We know a lot more about the HD gene now than ever before and different medications and approaches are being tested. So the future looks positive!
Development of HD
No-one is one hundred percent sure about what causes HD. We are fairly certain that the damage in the brain is caused by a build up of a protein (a type of body chemical) called Huntingtin. The cells in a certain part of the brain slowly become sick due to a build up of this protein over a long period of time - it is when the build up of this protein becomes too much that the cells can no longer work as well as they used to and become sick and start to die. This damage to the brain cells is what causes all the changes in HD.
This is why most people only start becoming unwell when they're older - it takes that long for the protein to build up in the brain cells and make them sick. This means that people are well for a long time before any signs of the disease start to show. So they live normal lives like everyone else - they have careers, they get married, travel - do all the usual stuff basically!
Living with HD
How do I cope with living with HD?
It can be hard when someone you love has HD. There can be lots of changes to cope with. Because of the damage to the brain, people with HD can change a lot as they become more unwell. Everyone in the family may have to do things they aren't used to doing. Most people have to give up their jobs at some stage when they become too unwell. This can have a big impact on everyone, there might not be as much money around and you may have to help out a bit more.
The person with HD may live at home and need lots of looking after. You might help look after them - helping around the house, doing the cooking, looking after younger brothers and sisters, etc. This means you are a young carer - you help to care for someone in your family who is not well. It's good to help out at times, but make sure you still get time for yourself to do the things you enjoy. Even though you may worry about the person with HD when you're not there, you still need a break and some fun.
The person with HD may also go away to a special home sometimes to give you all a break and have some time-out. This is called 'respite'. It can be exhausting looking after someone all the time and everyone needs a break. After a while, they may move into the special home because they need looking after all the time and need specialist care. You can visit them in the home if you want to and it means they are being cared for all the time.
Other people might not understand the changes in the person with HD. You can explain it's because they aren't well, maybe you could show them this website and help them understand HD better.
You might also worry a lot about whether you have the faulty gene. Maybe you've been a bit clumsy one day, or a bit absent-minded. Everyone gets like this sometimes so it doesn't mean you have the faulty gene. We all have off days, but it's hard not to worry when you're living with HD. If it's on your mind, talk to someone about your concerns.
It is important for you to talk to people you trust about how you feel. People in your family, friends and teachers need to know what's going on so they can try and help you. The more people you have around you who understand what you're dealing with, the more help you can get. It's good to have someone to talk to!
Looking after Yourself
It's very important to take good care of yourself when difficult things are happening.
Make time for things you enjoy - hanging out with your pals, a steaming bath with loads of bubbles, shopping, going for a run, playing your computer, remember to still have fun!!
Talk to people about how you feel, good friends, family, people you can trust. Don't keep your worries to yourself!
Make sure you get the information that you need - ask questions if you're not sure what something means.
Remember you are not alone, there are lots of other people living with HD. If you want to chat on-line with other young people who have HD in their family, register for our chatroom which will be running soon.
If you help out a lot at home, see if there's a Young Carer's Group in your area. You can meet regularly with other young people who help to look after a family member, they won't all have HD in their family but they'll still have some similar stuff to talk about. Plus you get some time out and the chance to have some fun! See the links page for info for young carers.
Being at Risk
Having a parent or grandparent with HD makes life more complicated at times. You have to think about things most of your friends never have to. Being at risk doesn't mean that your life is over, it just means that you have another factor to think about when making important decisions about your life. You can still go to college, have a career, go travelling, get married - do whatever you want to do in fact!
It can be difficult to decide when to share this information with important people, and particularly when you are in a serious relationship. Maybe show them this website so they can find out more about HD and what it's all about. It's often better to be open with the important people in your life as they can support you when things are rough. Keeping things secret is never easy and can make you unwell and stressed out. Even though it's difficult to talk about, you owe it to yourself to get the support you need, and you might be surprised how understanding people can be! Make sure you choose a good time to bring it up, when you've got time to talk and are feeling calm. Remember though, it's up to you who you choose to tell and when. Trust your judgement, if it feels right for you, go for it!