Huntington's disease

Overview

Huntington's disease
Huntington's disease

Huntington's disease is a condition that stops parts of the brain working properly over time. It's passed on (inherited) from a person's parents.

It gets gradually worse over time and is usually fatal after a period of up to 20 years.

Symptoms

The symptoms usually start at 30 to 50 years of age, but can begin much earlier or later.

Symptoms of Huntington's disease can include:

  • difficulty concentrating and memory lapses
  • depression
  • stumbling and clumsiness
  • involuntary jerking or fidgety movements of the limbs and body
  • mood swings and personality changes
  • problems swallowing, speaking and breathing
  • difficulty moving

Full-time nursing care is needed in the later stages of the condition.

How it's inherited

Huntington's disease is caused by a faulty gene that results in parts of the brain becoming gradually damaged over time.

You're usually only at risk of developing it if one of your parents has or had it. Both men and women can get it.

If a parent has the Huntington's disease gene, there's a:

  • 1 in 2 (50%) chance of each of their children developing the condition – affected children are also able to pass the gene to any children they have
  • 1 in 2 (50%) chance of each of their children never developing the condition – unaffected children can't pass the condition on to any children they have

Very occasionally, it's possible to develop Huntington's disease without having a history of it in your family. But this is usually just because one of your parents was never diagnosed with it.

When to get medical advice

Speak to your GP for advice if:

  • you're worried you might have symptoms of Huntington's disease – especially if someone in your family has or had it
  • you have a history of the condition in your family and you want to find out if you will get it, too
  • you have a history of the condition in your family and you're planning a pregnancy

Your GP may refer you to a specialist for tests to check for Huntington's disease.

Treatment and Support

There's currently no cure for Huntington's disease or any way to stop it getting worse.

But treatment and support can help reduce some of the problems it causes, such as:

  • medicines for depression, mood swings and involuntary movements
  • occupational therapy to help make everyday tasks easier
  • speech and language therapy for feeding and communication problems
  • physiotherapy to help with movement and balance

Further infomation and advice

Living with Huntington's disease can be very distressing and frustrating for the person with the condition, as well as their loved ones and carers.

You may find The Huntington's Disease Association a useful source of information and support.

They offer:

Symptoms

Huntington's disease can cause a wide range of symptoms, including problems with mental health, behaviour, movement and communication.

The symptoms usually start at 30 to 50 years of age, but can begin earlier than this (juvenile Huntington's disease) or much later.

Once they start, the symptoms usually get gradually worse.

Early symptoms

The first symptoms of Huntington's disease often include:

  • difficulty concentrating
  • memory lapses
  • depression – including low mood, a lack of interest in things, and feelings of hopelessness
  • stumbling and clumsiness
  • mood swings, such as irritability or aggressive behaviour

See your GP if you're worried you might have early symptoms of Huntington's disease, especially if you have a history of the condition in your family.

Lots of things can cause these symptoms, so it's a good idea to get them checked.

Your GP may suggest having a test for Huntington's disease.

Later problems

Over time, someone with Huntington's disease may develop:

  • involuntary jerking or fidgety movements of the limbs and body
  • difficulty speaking clearly – eventually they may find all communication very difficult
  • swallowing problems – they may choke on food and get lung infections (pneumonia) from food going down the wrong way
  • increasingly slow or rigid movements
  • personality changes – sometimes they may change so they don't seem like their former self at all
  • breathing problems
  • difficulty moving around – they may eventually lose the ability to walk or sit up by themselves

In the later stages, people with Huntington's disease find daily activities increasingly difficult and will need full-time nursing care.

Tests

A test to look for the genetic change that causes Huntington's disease can be used to diagnose the condition or check if you or your child will develop it later in life.

Test to find out if you'll get Huntington's disease

If you have a history of Huntington's disease in your family (especially if a parent or grandparent had it), you can have a test to see if you'll also get it.

It's up to you to decide if you want to have the test. Lots of people at risk of Huntington's disease decide they'd rather not know until any symptoms appear.

If you do want to know, ask your GP for a referral to a genetic counsellor. You'll have several appointments with the counsellor. It's only done once all the benefits and risks have been explained.

The test involves checking a sample of your blood for the genetic fault that causes the condition. It can take a few weeks to get the result.

The Huntington's Disease Association has more information about genetic testing for Huntington's disease.

Tests before or during pregnancy

Speak to your GP if you're planning a pregnancy and:

  • you have a family history of Huntington's disease
  • a test has shown you have the genetic fault that causes the condition

This might mean your child is at risk of developing Huntington's disease.

Your GP can refer you to a genetic counsellor to discuss your options.

These may include:

  • having a child with a donor egg or sperm
  • adopting a child
  • having a test during pregnancy (chorionic villus sampling) to see if your baby will get Huntington's disease
  • pre-implantation genetic diagnosis – where eggs are fertilised in a laboratory and tested to make sure they don't have the Huntington's disease gene, before being implanted into the womb

Tests to diagnose Huntington's disease

If you have symptoms of Huntington's disease, your GP may refer you to a specialist for tests.

The specialist will ask about your symptoms to see if it's likely you have Huntington's disease and rule out similar conditions.

They may examine you and test things like your thinking, balance and walking ability. Sometimes you might also have a brain scan.

A blood test to check for the Huntington's disease gene can confirm if you have the condition.

Treatment

There's currently no cure for Huntington's disease or any way to stop it getting worse. But treatment and support can help reduce some of the problems caused by the condition.

In many areas, there are Huntington's disease clinics run by a specialist doctor and nurse, who can offer treatment and support and refer you to other specialists if needed.

Research into new treatments is ongoing and there have been some promising results recently.

Medicines

Medicines can help reduce some of the problems caused by Huntington's disease, but they don't stop or slow down the condition.

These include:

Some of these medicines aren't licensed for Huntington's disease, but have been found to help relieve the symptoms.

Most of these medicines can cause troublesome side effects. Speak to your doctor about the possible benefits and risks of taking them.

Help with everyday tasks

Daily tasks such as getting dressed, moving around your house and eating can be frustrating and exhausting if you have Huntington's disease.

An occupational therapist can look at activities you find difficult and see if there's another way you can do them.

They can also recommend changes that could be made to your house and equipment you can use to make things easier for you.

These can include:

  • putting in ramps so an area can be accessed in a wheelchair
  • fitting a stairlift
  • installing grab rails – for example, by the stairs or beside the bed
  • using electric can openers, electric toothbrushes and kitchen utensils with large handles that are easier to hold
  • voice-controlled lights or voice-controlled software on a computer

Read more about how occupational therapy can help and how to get it.

Help with eating and communication

A speech and language therapist and a dietitian can help if you have difficulty communicating and eating because of Huntington's disease.

For example, they can advise about:

  • alternative ways of communicating – such as electronic speech devices or picture charts
  • a high-calorie diet to help prevent weight loss
  • ways to make food easier to chew and swallow

At some point, a feeding tube that goes directly into your stomach may be needed.

If you don't want to be fed in this way, you may want to consider making an advance decision that outlines how you'd like to be cared for in the later stages of your condition.

Help with movement and balance problems

If you have Huntington's disease, it's important to try to stay as active as you can. This can help you feel better both physically and mentally.

Getting around can be difficult if you have problems with co-ordination and balance, but even regular walking with the use of aids like walking sticks can be beneficial.

A physiotherapist can also help with movement problems.

They may recommend things like:

  • an exercise plan
  • moving and stretching your joints (manipulation)
  • massages

Read more about how physiotherapy can help and how to get it.

Research into new treatments

Research is underway to find new treatments for Huntington's disease.

Progress has been made in identifying possible ways of slowing down or halting the condition by "switching off" the faulty gene that causes it.

Several treatments are now going through clinical trials. If they're found to be safe and effective, they might be available in several years' time.

You can find out more by visiting the European Huntington's Disease Network, HD Buzz, and the Huntington's Disease Association. You can also ask your specialist doctor or nurse.

More information about care and support

The Huntington's Disease Association has more information about getting help for Huntington's disease, including advice about:

  • behavioural problems
  • communication skills
  • sexual problems
  • diet, eating and swallowing
  • seating, equipment and adaptations
  • your options when full-time care is needed
  • benefits you may be entitled to

Driving

You should discuss any concerns about driving with your doctor.

If you hold a driving licence and have symptoms caused by Huntington's disease, you're legally required to contact the DVLA.

The DVLA will ask you for details of your doctor to seek further information. Many people are still allowed to drive, but this will be reviewed regularly.

There's no requirement to contact the DVLA if you haven't developed symptoms. If in doubt, discuss this with your doctor.

Find out more about driving with a medical condition



The information on this page has been adapted by NHS Wales from original content supplied by NHS UK NHS website nhs.uk
Last Updated: 12/06/2024 11:22:09