How does HD affect people?
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If you have read the ‘What is Huntington’s disease?’ section, you will know that the symptoms experienced by people with Huntington’s disease can be divided into three main types: involuntary movements, cognitive problems and behavioural symptoms. This section will look into what type of symptoms fall under these three categories and how they affect a person with Huntington’s disease.
Before we start it is really important to realise that a person with Huntington’s disease may not develop ALL the symptoms mentioned in this section. Most people only get some, and each individual experiences different symptoms. The symptoms also change as time goes on.
What do the three categories mean?
Huntington’s disease affects people in three main ways (involuntary movements, cognitive problems and behavioural symptoms), but what do they all mean?
Involuntary movements: The first category, involuntary movements, means that a person has less control over some of the movements they make. Sometimes people with Huntington’s disease will find it hard to keep still because of ‘involuntary’ or unwanted movements. Many people find that movements they want to make are harder, too.
Cognitive problems: The word ‘cognitive’ means a person’s thinking abilities. People with Huntington’s disease may find it hard to think clearly, answer questions or concentrate. This is because of the cognitive effects of the disease.
Behavioural symptoms: The third category, behavioural symptoms, means that a person’s behaviour may change when they have Huntington’s disease. For example, someone with the disease may get angry a lot or get frustrated, At times it can seem like someone’s personality has even changed.
The three categories
People with Huntington’s disease usually develop symptoms from these three categories. They can happen in any particular order, and people don’t necessarily have to be impacted by all three categories. For example, someone may get behavioural symptoms first, and then develop the involuntary movements. Another person may have cognitive issues first, then the involuntary movements, but never develop behavioural symptoms. It is quite unpredictable. Now let’s look at these categories individually and check out some of the symptoms from each.
Often a person with Huntington’s disease will have involuntary movements where they find it hard to keep still. This is also known as ‘chorea’, which is the Greek word for dance. In the old days, people used to call the disease ‘Huntington’s chorea’ because the movements were so obvious. It was renamed to remind people that there’s more to the disease than movement problems. Medical professionals also tend to use the term ‘motor symptoms’, which is another term for problems with a person’s movements.
The involuntary movements caused by Huntington’s disease can affect people in various ways throughout the progression of the condition. Usually people appear to be restless, or fidgety in the beginning. Then gradually these fidgets turn into more obvious involuntary movements which can result in a whole host of situations when out in public with the person with the condition.
Surprisingly, many people with Huntington’s disease aren’t aware that the involuntary movements are happening, or don’t notice them as much as other people do. That’s because Huntington’s disease can affect people’s awareness of their own bodies. This can be difficult, because it can cause people not to take enough care when walking. But on the other hand, it can also mean that people with Huntington’s disease often feel better than they look.
There’s another side to movement problems in Huntington’s disease. It’s less obvious than unwanted movements, but can cause big problems. Doctors call it ‘loss of voluntary movement control’. It means that movements that a person wants to make become more difficult. Walking can get slow, balance gets less reliable and fine movements like fastening buttons or writing can become uncoordinated. The problems caused by loss of movement control can be more severe than the unwanted extra movements.
‘My dad would cause chaos when out food shopping. There was never a week that went by without some sort of incident, usually involving him knocking over a beautiful display some poor staff member had spent hours putting together!’ Katy
As Katy highlights, keeping your balance when you have movement problems is not easy, and it only becomes more challenging as time goes on. The same goes for walking in general: people tend to get slower and slower, with increasing involuntary movements thrown in.
At some point people with Huntington’s disease usually have to stop driving as well. This is because of the involuntary movements and changes to the thinking processes. This can be a very difficult decision that has to be made, and often the person with Huntington’s disease may not think that they have a problem with driving. It is important to voice any concerns about someone’s driving though as it can be quite dangerous.
Speaking relies on the brain being able to control the lips, tongue and mouth. So, speaking can also become difficult for people with Huntington’s disease. Often people’s speech will begin to slur and other people may have trouble understanding what they are saying. Speech generally deteriorates as the disease progresses and some people lose their speech completely as a result of the disease. It’s important to remember that people with Huntington’s disease can recognise loved ones and usually understand what’s going on and what’s being said, even if they have trouble speaking.
‘Mum lost her speech, I forget when it happened, and it seemed to gradually fade away. First she was slurring her words, and then she was down to sentences, keywords and eventually no words. But we still communicate with her as best we can, we use cards with words on them and she points to tell us what she wants.’ Anthony
Swallowing, burnt calories and weight loss
Swallowing can also be affected for people with Huntington’s disease, which causes a problem because we all need to eat.
Weight loss can be made worse by the fact that people with Huntington’s disease need to eat a lot more than normal. We don’t fully understand why this is. Partly it’s caused by involuntary movements, which make people burn an awful lot of calories each day. But partly it’s because the bodies of people with HD are less efficient at using energy.
So it’s very important people with Huntington’s disease eat lots of food or get lots of calories in them. This is why people with Huntington’s disease sometimes eat high-calorie foods or supplements, or have a feeding tube inserted, to make sure they are getting enough calories each day.
As you can see, movement problems can cause a lot of different problems for someone with Huntington’s disease. But remember, people with the disease won’t necessarily have all the symptoms mentioned in this section.
The second category is cognitive problems. Cognitive means anything to do with ‘intellectual activity’ or our process of thinking. Basically, anything that you have to put some thought into (like Sudoku… or maybe that’s just me).
Decisions, organisation and concentration…
As the disease progresses, people with Huntington’s disease sometimes have difficulty organising things or making decisions. That’s because the ability to concentrate becomes more difficult as a result of cognitive problems. A little bit of patience from people around them, or extra help explaining the options of a decision can go a long way.
Memory can also be affected by the cognitive changes caused by the disease, although often it is not memory itself that is affected but the ability to recall something (which is explained in our what does it feel like to have Huntington’s disease section).
‘My mum was unable to speak and communicating in general became almost impossible. I would often wonder if she knew who I was anymore, she would look at me but there was never really any emotion or expression, it was hard. I felt she didn’t remember me at all.’ Anthony
Unlike Alzheimer’s, those with Huntington’s disease do recognise people and places. So it is very likely that Anthony’s mum does know exactly who he is. This is an important point to remember because some people may start to question it at times, as the disease progresses.
Communicating: Wait for an answer…
Communicating, as was highlighted already, can become an issue. But not just because of the person having difficulty talking, there is also an issue when the person can talk, but takes longer to answer because the person with Huntington’s disease is often unable to think of a reply right away. A neat trick to get past this is when you ask a question, to wait for an answer to your question without interrupting or saying anything yourself (otherwise you will disrupt their concentration on answering your original question), and after a while the person with Huntington’s disease will usually respond to your question. It just takes people with Huntington’s disease longer to process what was asked and what answer to give. That tip might not work for everyone, but it has shown to be quite effective.
The third and final category is the behavioural aspect of Huntington’s disease. The condition can cause people with Huntington’s disease to behave unusually and do or say things they don’t mean to (without thinking). The behavioural changes do not happen to all people who have the condition, but they can be difficult changes to cope with, because it can be hard to remember that these actions or words are a result of a disease, rather than the person.
Depression is very common in Huntington’s disease. Researchers know that depression is actually a symptom of the disease, rather than people just feeling sad. But depression can often be treated and when someone’s depressed, it’s important that people around them try to keep them involved in activities. If you think someone with HD is depressed, try to get them to see a doctor.
People can also suffer from apathy, which means that they have difficulty motivating themselves to do anything. This can be a difficult problem because it is very important that people with Huntington’s disease exercise and be active, it can help delay symptoms. However, often if prompted, and encouraged they will be more successfully motivated to do things. Apathy can be difficult to treat, but getting a regular routine of activities can often help.
Denial is also a behavioural symptom of Huntington’s disease. People may even deny that they have any symptoms at all, which can obviously be quite a problem when trying to help the person. Your Huntington’s disease clinic, local HDA, or community care team will be able to offer advice on coping with the problems caused by denial.
‘My mum denies that she has Huntington’s disease, or any other condition for that matter. It doesn’t make my life easy when I want her to help me fill out forms for her care plan!’ Sophie
Although this is very frustrating it is important to remember that this is often due to the cognitive changes that are taking place in the brain, and the person does not have insight into the difficulties they are having.
Perhaps one of the more difficult symptoms to deal with can be the aggression caused by the disease. Some people with Huntington’s disease may get angry a lot, be irritable, get annoyed and can generally be difficult to reason with. This is all a part of the behavioural aspect of the disease - try to be understanding when somebody is behaving this way towards you. Try to remember it’s not the fault of the person with Huntington’s disease, and learn to back down from the arguments. Make a cup of tea, leave the room or go for a walk. There are also medications which can often be effective for aggression.
People with Huntington’s disease can also become ‘stuck’ on particular ideas or thoughts and repeat them over and over again (similar to Obsessive-Compulsive Disorder). This is why people with the condition get very much into routines and find it difficult to adjust if their routine is changed.
‘My dad got fixated on washing his hands all the time. He would also advise us to wash ours regularly as well! It was becoming quite ridiculous and fortunately a health care professional was able to help him get rid of this fixation, otherwise we would all still be washing our hands repeatedly right now!’ Jason
Everyone is different
Although Huntington’s disease can affect movements, cognitive function and cause behavioural symptoms, each person is impacted individually and it is impossible to predict how each person will be affected. For example, one person with Huntington’s disease may have severe involuntary movements (chorea), but very few behavioural symptoms. Another person with the condition may have a lot of behavioural symptoms but only slight movements. Even two people from the same family, who are both affected by Huntington’s disease, may have completely different symptoms of the same disease.
Remember, too, that some symptoms may go away on their own, while others can be treated effectively using medicines or other therapies. The key is to seek help from a professional if a symptom is causing problems.
Progression and stages
Although symptoms of Huntington’s disease vary from person to person, even within the same family, the progression of the disease can be roughly divided into three stages: Early stage, middle stage and late stage. In early stage disease, a person has difficulties but can usually still look after themselves and walk, and may be able to work and drive. In the middle stage, people usually need to use a frame or wheelchair to get around, and may need help at home. In the advanced stage, there are serious problems with getting around and people need a lot of help, often needing to be looked after most or all of the day. As the disease progresses through these stages, the disease generally becomes more severe, and presents new challenges. Professionals with experience of Huntington’s disease can usually come up with useful suggestions and help at all stages of the disease.
There is no cure or effective treatment which can slow down the progression of Huntington’s disease yet. There are treatments that can help control many of the symptoms of Huntington’s disease, and there is a lot of research being done at the moment - so there is great hope for the future! Visit our research section to find out more about advances in research.
Overall, Huntington’s disease has an impact on people in many different ways, and it is impossible to predict how each individual with the disease will be affected. If you have any more questions about how Huntington’s disease affects people why not try the Ask a question section. Alternatively, if you want support or advice in dealing with some of the issues discussed in this section, you can contact HDYO directly or speak to your local HDA.