Huntington's Disease

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Huntington’s disease is a rare hereditary condition that causes the degeneration of brain cells over time. Progressively, the condition worsens and, subsequently, is usually fatal after approximately 20 years.

The condition is linked to a faulty gene which, if passed on from parent to child, causes a person to develop Huntington’s disease.

In rare cases, a person can develop Huntington’s as a new mutation, although often, a parent has had it, but it was undiagnosed, or the family history is unknown.

Huntington’s disease has broad implications on a person and their ability to function in terms of both physical and mental health.

There is currently no cure for the condition, or treatment, although it is possible that some symptoms can be managed.

Huntington’s disease has two forms that can be diagnosed in (1) adulthood and (2) childhood.

Adult-onset Huntington’s disease is the most common type of Huntington’s effecting 1 in every 20,000 in the UK. Both men and women are equally affected by the condition. This form of Huntington’s disease affects people from any adult age, but commonly between the age of 30 to 50 years old.

Juvenile Huntington’s disease is a type of Huntington’s which affects those under the age of 20. It is incredibly rare, effecting approximately 6 out of every 100 persons.

Photo by Anand Dandekar from Pexels

Symptoms amongst those living with Huntington’s disease can vary from person-to-person. Symptoms usually present themselves at around the age of 30 to 50 years old.

Early symptoms of adult-onset Huntington’s disease include:

• Stumbling and clumsiness
• Memory lapses
• Depression
• Difficulty in concentrating
• Mood swings.

Later symptoms of adult-onset Huntington’s include:

• Difficulty moving – possibly losing the ability to walk, or sitting up unassisted
• Increasingly slow, or rigid movements
• Involuntary movement of the body and/or limbs (chorea)
• Issues with swallowing
• Difficulty speaking – it is possible that someone with Huntington’s disease may find verbal communication very difficult as the condition worsens
• Breathing problems.

Symptoms of juvenile Huntington’s disease can be slightly different to the symptoms of adult-onset Huntington’s. It is widely thought that the symptoms of juvenile Huntington’s are similar to the symptoms of Parkinson’s disease.

Juvenile Huntington’s symptoms to be aware of:

• Behavioural problems
• Rigid muscles affecting the gait, especially in younger children
• Difficulty in concentrating
• Seizures
• Significant decline in quality of schoolwork
• Tremors, or slight involuntary movements
• Clumsiness, or frequent falls.

Photo by JZhuk on iStock

Living with the condition poses numerous difficulties for a person, both physically and mentally. As the disease progresses, the implications of it grows and people are less able to live their life the way they once did.

There are medications and treatments available for some symptoms, so that they can be managed, and a person can live their life as unaffected as is possible, despite the fact that there is no cure for the disease.

One of the later symptoms to develop, Chorea, makes a person unpredictable in terms of their body movement, causing them to struggle with day-to-day tasks, such as driving, writing, eating, dressing and washing. In addition to this, the disease can cause cognitive changes to a person which may make them struggle to retain information after watching TV, or having a conversation with someone.

Speech can be affected by Huntington’s disease by making a person’s voice more hoarse, or slurred. Additionally, control of the throat muscles can be impacted by Huntington’s, making it harder to swallow certain foods.

Eating softer foods can make it easier for a person to eat and ensure they get the nutrition that they need from their diet. Good nutrition is vital for those living with Huntington’s, as they require specific diets which consist of high-calorie foods due to increased levels of movement. In some cases, it may be necessary for a person, in the later stages of Huntington’s, to have a feeding tube, if the changes in throat control have deteriorated to the point where they can no longer eat.

Moreover, it is possible that a person living with Huntington’s disease may have issues with their mental health and therefore suffer with low moods, depression, or anxiety. This can happen following a diagnosis, or during the progression of the condition. 

Labelled Key Fobs may be a good assistance when it comes to the procedural memory loss associated with Huntington's disease. We have a wide range of Dressing Aids, which can help with any dexterity issues that impact day-to-day activities. A Fluted Grab Rail will also be a household essential for any issues with balance when navigating tricky areas of your house.

 

There is a wide range of support available for those living with Huntington’s. Usually, a medical team will support each person in the way that is best suited to their needs. Health care professionals can access support groups and other medical services which may be required, or of use. 

For mobility related problems, it might be suitable for a person to see an occupational therapist and/or a physiotherapy team.

Speech therapists can support people who are struggling with their speech, as well as using talking aids, such as cards, or other visual aids.

Other forms of support for a person’s mental wellbeing is available via counselling, cognitive behaviour therapy, or possibly medication.

For any further support, or information, please visit:

NHS

Huntington’s Disease Association

The Brain Charity

Medical terms are often baffling and difficult to fully understand. To help, we have listed some frequently used terms below.

Chorea – is a neurological disorder which causes involuntary jerk like movements in the body, especially the shoulders, hips and face

Degeneration – in medical terms, this means the decline and loss of function of cells, or tissues, in the body

Gait – is the term used to describe the way in which a person walks

Terminal – in medical terminology, it means that a condition is expected to lead to death