Information for schools
What is Juvenile Huntington’s disease?
Huntington’s disease (HD) is a hereditary condition that causes abnormal movements and other physical signs as well as emotional, cognitive and other physical problems. It usually presents in middle life, although it can present at any age. Juvenile Huntington’s disease (JHD) is the name given to any affected person who has signs or symptoms before the age of 20 years.
This group makes up about 5-10% of all people with HD, and so it is a relatively rare condition in the general population. This can make dealing with JHD an isolating experience for the young people with HD, their families and also professionals involved with them. Given their age, teachers are likely to be one profession that they have a lot of contact with. This page has been written for schools to help them have a better understanding of JHD and hopefully to give them some ideas about what they might be able to do to support a child with JHD.
The young people with JHD are likely to be attending a school or college of some kind and as a result will have specific educational and care needs. Some of the needs they may have are discussed below, but it is important to remember that each child will be unique and certain support may, or may not, be a problem and therefore may, or may not, be needed.
Movement disorder at school
The features of the movement disorder in young people with JHD are complicated and often involve a mixture of rigidity, an irregular, staggering gate and myoclonus (brief, strong muscle contractions). The chorea (involuntary movements) often seen in adults with HD is less common in young people with JHD, although it can still appear in some cases. These movement problems, both rigidity and chorea, put the child at a high risk from falls and they will probably find it difficult to get to parts of the school without easy access (e.g., up-stairs rooms). They may also find it difficult to carry their own books and equipment that they need at school and need help doing this. As the condition progresses, it may be necessary for them to be accompanied between classes, especially at times when the corridors are very busy and crowded.
It may be useful to think ahead about what specific parts of the schools may cause problems, so that there is time to find funding and establish any changes that need to be made. Often, however, all that is needed to overcome these difficulties is to make a few simple changes (e.g., switching the child’s classes to classrooms with easier access).
Cognitive disorder in schools
Young people with JHD may also have cognitive (or ‘thinking’) changes that cause them difficulties in the school environment. In terms of changes to cognition (or ‘thinking’) in JHD there are a number of things that are fairly common in young people with JHD. For example, they may have a slowed response time and/or a lessened ability to take things in.
The problems they have in taking information in tend to get worse as the condition progresses, but often remain fairly intact in the early stages of the condition, even when their responses may be somewhat slowed. They may also find it harder to concentrate and can have memory problems. It can be harder for them to initiate actions that they may be capable of doing, which may make them appear to be lethargic and lazy.
However, all of this is part of the condition, and is easily helped if they have extra help in getting started in activities. Also, they may be more impulsive and have difficulty waiting for things, which is again a part of the condition. Finally, these difficulties with thinking tend to cause them to have problems with organisational skills (e.g., having equipment and books for homework, for example). They may also mean that they need extra help in classes. Sometimes, this can be given by a friend or the class teacher, although at other times they may require a Learning Support Assistant in some, or all, of their lessons.
Challenging behaviour in schools
Challenging behaviours are also fairly common among young people with JHD, in particular where symptoms begin when they are in their teens. In some, this can be the main obvious symptom with much fewer movement problems than is normally seen in HD patients. For some years, it may be uncertain whether their behaviour is due to the Huntington’s disease or the environment, particularly as many specialists would be reluctant to give a diagnosis of HD (or refer the young person for a genetic test) without obvious physical signs of HD.
It is important to remember that simply because a child has HD, it does not follow that every other problem is related to it and changes to behaviour can have many different causes, particularly in children / young adults. For a child with HD the family situation may be disrupted and the school may be the most, and sometimes only, stable environment that the child has.
However, it is equally important to remember that the challenging behaviours could be due to the HD and that they should therefore be managed sensitively. When trying to deal with changes in behaviour both in a child with JHD and in at-risk children and siblings it is important to bear in mind the implications that labelling the child (either as difficult, or as affected by HD) may have in any particular context. In some cases, being labelled as ‘difficult’ may be more stigmatising than having a diagnosis of HD.
People may be more sympathetic to a child when they believe there is a medical reason for the child’s behaviour, but when there is not, there may be a temptation for people to place the blame for the young person’s problems on the family. It is important to bear in mind that a mentally or emotionally disturbed child in an HD family needs special attention regardless of whether they have the disease or not.
Whether or not their problems are directly caused by HD, they may have had some very serious issues to deal with indirectly related to HD and staff should equally be sympathetic to this. Finally, although challenging behaviours may appear in some children with JHD, in many children they don’t appear at all. Further information can be found in the leaflet ‘Challenging Behaviour in JHD’ and in ‘Understanding Behaviour in HD’ (see ‘Further Information’).
Epilepsy can be a problem in youngsters with JHD, although less so when symptoms begin in the teens. Having to deal with epileptic seizures worries many teachers. However, there are many sources of support that can be used to get more information and advice on epilepsy and there is therefore no reason why it should be an insurmountable problem.
Epilepsy Action (see www.epilepsy.org.uk) and the National Society for Epilepsy (see www.epilepsysociety.org.uk) produce a range of information booklets and leaflets, including advice for teachers (see www.epilepsy.org.uk/info/education/epilepsy-in-schools-in-england). Epilepsy Action also have a free-phone helpline (0808 800 5050), which can provide staff with more specialist advice. In addition, it would be a good idea to organise staff training (e.g., from a specialist epilepsy nurse) for members of staff who have contact with the child, as this will give them an opportunity to ask questions about issues not covered in the written information and be given up-to-date and specific advice about their type of epilepsy.
A young person with JHD may also have problems with speech and with swallowing. Adults and older children with HD need a high calorie intake. The reason for this at present is unclear, as it is not thought that this problem is wholly caused by the involuntary movements often seen in HD. However, it is important that people with HD receive the correct number of calories as it can affect their physical and mental well-being.
Given the problems that a person with JHD often has with swallowing, and that they may not be able to go home to their parent/guardian at lunchtime, it is important that they are given help to get as many of these calories as possible. To be able to get enough calories, the young person may need to eat small, frequent snacks in-between meals. They may also need more time to eat, because they will have to eat slowly, and will need a calm, quiet environment as they need to concentrate on eating.
The young person also needs help getting enough fluids sometimes, as they may also have problems drinking and there is a risk that they may become dehydrated. This is particularly true during the summer months, but care should be taken throughout the year.
Speech problems in school
Speech problems usually start off with only mild slurring, but this typically develops into indistinct and virtually monosyllabic speech as the disease progresses. Speech and communication problems are one of the aspects of the condition that can make it most difficult for the child with JHD to maintain good relationships with peers.
In class, the young person may need more time to speak, receive the message and reply. Speech aids can be of help, but it is important that an assessment is made early on by a speech and language therapist and that any suggested solutions (e.g., flash cards, computer aids) are put in place as early as possible to allow the young person to get used to using them. It is also important that the school is aware of what aids are being used at home, so that a consistent method is being used.
There are a number of simple things you can do to help communication with the child. More information about these can be found in the leaflet, ‘Communciation Skills’ available from the HDA.
General points about JHD in schools
A number of general comments can be made about the support needs of a young person with JHD. Firstly, it is very important that these support systems that have been discussed are put in place as early as possible. An assessment of the young person’s needs should be made early to identify any needs, and it needs to be remembered that these needs are likely to change as the condition progresses, and so regular re-assessment is important.
The earlier changes are made, the more time the person will have to adapt to these changes, meaning that they are likely to be more useful for longer. For example, if a computer aid is given earlier, the young person will have time to learn how to use it so that they are more likely to be able to use it as their cognitive abilities decline. Also, these changes should be implemented quickly. Having a degenerative condition, the needs of a person with JHD changes and a long delay in meeting their needs can mean that the person can no longer get the best benefit from the support that was needed at the time a request was made. However, considering what a young person might need in the future (which will involve assessing how their condition will progress in the future) should also be done sensitively and with an awareness of how discussions about the young person’s degeneration may affect them and their family.
Although young people with JHD can degenerate quickly from the onset of their condition, it is important to remember that this can be very variable and a person with JHD can live a full life for many years. It has not been unknown for people with JHD to live for a long time after the onset of their condition. The engagement involved in school activities, the process of learning and the social environment of the school may also play a very important role in the progress of the condition and may help to maintain the current abilities of the person for as long as possible, as well as helping them to develop new ones. It is therefore very important to support the young person so that they are able to remain at school as long as possible if they wish to do so.
The family and siblings
The child with JHD has been discussed at length, but for a school it is also important to consider the wider family. Huntington’s disease is a condition caused by a dominant gene. This means that when one parent has Huntington’s disease, their biological children have a 50% risk of developing the condition. It is therefore important to remember that any siblings, who may be at the same school as the affected child, may be at-risk and have concerns about that. Even if they are not at-risk (e.g., they are not a biological sibling or they already know their genetic status), they still have to cope with a sibling who has HD. They may be being bullied (this may also be true for the young child with JHD), or they may feel a responsibility to look after their brother or sister. It is important therefore that the school is also aware of how JHD in the family may be impacting on them and their education and development.
It can be very useful for staff, and perhaps also students, at the school to gain a basic knowledge of HD and the genetics of HD as misunderstandings and misinformation can cause many problems.
The supported school
With knowledge about HD, support and advice on how to cope with the problems of HD in young people, it can be possible to keep the child at school for a long time. Although the young person may have additional needs, with support there are few things that they cannot do. The school is in a unique position in being able to support the child and enabling them to achieve. Equally, seeing how the young person copes with a progressively disabling condition can be of untold benefit to everyone in the school, both children and adults.
If a school has concerns about a young person affected by HD or their family, they are welcome to contact the Huntington’s Disease Association and their team of Specialist HD Advisers, who can offer them support and advice, and answer any questions they may have.
Glendinning, N ‘Huntington’s Disease in Children and Teenagers: A Guide for Professionals’, available from the Huntington’s Disease Association. Please contact Head Office for a copy. Chapters include: Definitions of HD, Genetics, Genetic Testing in Children, Pathology, Prevalence, Age of Onset, General Remarks on JHD, Clinical Picture, Diagnosis and Differential Diagnosis, Speech and Communication Problems, Swallowing and Alternative Feeding, Physiotherapy, General Management with flow chart, Management of Mental Disorders, Treatment, Schooling, Social Services and Further Reading.
‘Challenging Behaviour in Juvenile Huntington’s Disease’ available from the HDA website.
Paulsen, J ‘Understanding Behaviour in HD’, available from the Huntington’s Disease Association. Please contact Head Office for a copy.
Nance, M ‘The Juvenile HD Handbook: A Guide for Families and Caregivers (2nd edition)’, Huntington’s Disease Society of America (HDSA) website. This guide has chapters on: The Diagnosis of HD in a child, After the Diagnosis, Medical Care, Daily Life, The Late Stages, Financial, Legal and Social Service Issues, Caring for the Caregivers and Hope for the Future.
‘Juvenile Huntington’s Disease: A Resource for Families, Health Professionals and Caregivers’. This guide is available from the Huntington’s Society-Canada website at www.huntingtonsociety.ca. Chapters include: About Juvenile Huntington’s Disease (Overview, The genetic basis of JHD, First Symptoms, Diagnosis), Living with JHD (Schooling, Physical and Occupational Therapy, Speech Therapy, Swallowing, Behaviour and Judgement, Seizures, Other Drug Treatment, Juvenile HD and Family Life), and Later Stages of the Disease (Leisure and Social Time, Institutionalisation, Death, Hope for the Future).
Frequently Asked Questions
How long can we expect the young person to live for after they develop symptoms?
Unfortunately, there is no answer to this question. There is some evidence to suggest that people who develop HD symptoms when they are younger have a shorter life expectancy from the onset of their symptoms than people who develop symptoms as an adult. However, how long people live for after they develop HD symptoms is very variable. At the moment, it is very difficult to predict the prognosis.
How long can we expect the young person to be with us at the school?
Again, this is very variable. Some young people do very well and manage to stay in a school for a long time, even in a mainstream school. It depends largely on their individual symptoms, the support at the school (both staff and students) and the facilities the school has.
Is a mainstream or special school better for the young person?
More detailed information about this can be found in the book ‘Huntington’s Disease in Children and Teenagers: A Guide for Professionals,’ by Neil Glendinning and available from the Huntington’s Disease Association. However, influencing factors are:
- The type and degree of disability
- The availability of schools locally
- The response of schools to the problems posed by the disease
- Schools that will meet the physical and emotional needs of the child
- the opinions of the parents and the affected youngster
- the opinions of the siblings
- the opinions of the main carers and professional advisers
Home schooling is not generally recommended, as the parent is placed under additional pressure and the child is deprived of social contact. Mainstream schooling can become difficult as the disease progresses, but this very much depends on the particular situation. Moving to a special needs school can be a big step for everyone. Therefore, careful thought needs to be given about the timing of such move. A school for children with physical disability may be more appropriate than a school for children with severe learning disabilities.
We are having real problems managing the challenging behaviour in the child. Is there anything we can do?
This can sometimes be a real problem for schools, who are torn between being sympathetic to the child with HD and the welfare of other students at the school. However, this is a very difficult question to answer as the challenging behaviours could be caused by so many different things and take many different guises. The problems could be due to the HD, or they could be due to a disrupted environment, or both. It will be worth consulting an educational psychologist or a paediatric psychiatrist who know something about HD. For example, it could be that certain drugs could help, or drugs that are being taken need to be changed (they could need to be stopped, reduced or increased). Also, there may be some behavioural modification techniques that could be tried. Using these two methods together has sometimes had some success. However, it should be remembered that challenging behaviour in HD can sometimes be caused by very small problems that can be resolved quite easily. Is the child finding the busy school environment too overwhelming? Are they having difficulty expressing what they want? Your Specialist HD Adviser may help you consider some of these things and the best way to resolve them.
No-one seems to know much about Juvenile Huntington’s disease. Where can I go for help?
Being relatively rare, it can sometimes be difficult to find someone to talk to who knows about Juvenile Huntington’s disease. Probably the best source of support in these situations is the Huntington’s Disease Association. They have a team of Specialist HD Advisers, who can offer advice and training on JHD. In some cases, they may also be able to put schools in touch with other schools or professionals who have had similar problems in dealing with JHD.