Huntington's disease is an illness caused by a faulty gene in your DNA. If you develop symptoms of Huntington’s and are diagnosed with the disease before the age of 20, this is known as Juvenile Huntington’s disease.

It's a rarer form of Huntington's disease

Juvenile Huntington's disease is rare, and less than 10% of people with the disease will have Juvenile-onset Huntington's disease.

 

The symptoms differ from the adult form

People with Juvenile Huntington’s often experience symptoms that are different from adult Huntington’s.

 

Epilepsy

Epilepsy is also more common in Juvenile Huntington’s disease, particularly in younger people and children

 

Symptoms

As with adult-onset Huntington’s, Juvenile Huntington’s symptoms can vary from one person to another. They mostly affect three main areas:

  • Movement
  • Thinking
  • Behaviour

In some ways, symptoms of Juvenile Huntington’s are similar to those of the adult disease, but there are some key differences.

Children and young people affected by Huntington’s are less likely to experience the involuntary movements, known as chorea, that often characterise the adult illness, and more likely to be affected by muscle contractions and stiffness.

Key facts:

  • Children and young people with Huntington’s can remain undiagnosed for some time, as doctors and specialists may be reluctant to give a diagnosis or refer them for genetic testing before physical symptoms appear.
  • Challenging behaviour tends to be more common in people who develop the disease in their teens, but many young people/children are not affected in this way.

There are many ways that children and young people living with Juvenile Huntington’s can get help and support with their symptoms to help them cope in school and greatly improve their quality of life.

How to know if it's Juvenile Huntington's disease?

Different types of symptoms generally occur at different stages of the illness. Often the first indication that someone has Juvenile Huntington’s is a change in their thinking or behaviour. For example, they may experience difficulty concentrating and following instructions, and there may be a noticeable drop in their performance at school, college or work. Family members and teachers may not initially interpret these changes as an illness at all.

Not all behavioural changes, however, may be directly caused by the disease. Children of families affected by Huntington’s may also be experiencing disruption and difficulties in their home life, which might also impact their behaviour, or they may be facing other challenges in their lives.

The stages of Juvenile Huntington's disease

We understand that after finding out about Huntington's or after a diagnosis, some people may want to know everything they might expect, but others might not.

Click on the below tabs - each of which will explain in further detail about symptoms in the different stages of Juvenile Huntington's disease.

Early stages

Changes in behaviour or a drop in school performance are often the first noticeable symptoms of Juvenile Huntington’s disease.

 

  • It may become harder to concentrate, learn new things, follow instructions and remember things.
  • It may take you longer to respond to questions and perform tasks.
  • It might be harder to start things, even things that you can do once you get going. Sometimes this can be misinterpreted as lethargy or laziness.
  • You or your loved one start to feel more frustrated, impatient, irritable or angry than normal.
  • Experiencing stiffness in your limbs. Your movements may slow down and you might find that you start to stumble or walk unevenly.

 

These are just a few of the possible symptoms - you may not experience them all or you may experience different ones. It is usually the case that the earlier the onset of symptoms, the more rapid they are likely to progress.

 

Many symptoms can be greatly helped with extra support at home and in class. As Juvenile Huntington’s is so rare, it’s important for parents and carers to work with a child’s school to help them understand the disease and how best to support you. For example, it may be that you need longer to eat or to have frequent snacks throughout the day to keep your weight up.

 

Living with Huntington’s disease can be very difficult for you if you have it and for your family. The right support can make a big difference, and we’re here to help.

Middle stages

Symptoms of the middle stage of the illness often revolve around changes in muscles and movement, although changes in behaviour may also become more significant and challenging too.

 

  • Muscles are likely to start making involuntary contractions and become stiff and rigid.
  • This will cause your movements to slow down and your arms and legs may become clumsy.
  • You may start to experience changes in how you speak as forming words becomes more tricky. Speech therapy and certain equipment can help a lot with this.
  • It may become difficult for you to swallow, making eating difficult. Support from a dietician can help to make sure they keep their weight up.
  • Your behaviour may change as feelings of anger, frustration or depression become quite intense. This can be quite challenging for you and those around you. It doesn’t happen to everyone but it’s more likely to happen if you start to develop symptoms in your teens.

 

Everyone’s journey through Juvenile Huntington’s is unique. You may not experience all these symptoms or may experience different ones or experience them at different stages. This means your care will need to be tailored to your specific needs and to change and evolve as you do.

 

The right support at every stage can make a big difference, and we’re here to help.

Later stages

The nature of Huntington’s is such that gradually, often over a period of many years, the disease progresses until the end of life.

 

Later on, you may experience difficulties with:

  • weight loss and nutrition
  • speech and swallowing
  • movement and stiffness
  • communication

 

These are just a few of the possible symptoms - you may not experience them all or you may experience different ones. It is important to pay attention to the symptoms that are causing you and your family the greatest difficulty, emotionally or practically, at the time.

 

Sometimes the psychological and emotional sides of living with the disease are more of a problem than the physical side. It can be mentally difficult to cope with having a serious illness, and extremely frustrating not to be able to do more of the things that you could easily do before.

 

However just because things are likely to be difficult, this doesn’t mean there is nothing to be done. There are many different kinds of support and help that people with Huntington’s can try, to manage their symptoms and live as well as possible. Your carer and family may also need additional support, practically and emotionally.

 

In the later stages of the disease more care and support will be needed.