Huntington's disease

An inherited condition affecting movement, thinking and mood. Every child of an affected parent has a one in two chance.

What it is

Huntington's is unusual among the conditions here because the genetics are unambiguous and the consequences reach the whole family before anybody is ill.

A single faulty gene causes progressive damage to specific parts of the brain. Symptoms typically begin between 30 and 50, and there is currently no treatment that stops the progression.

It is autosomal dominant, which means each child of an affected parent has a 50% chance of inheriting it. Predictive testing is available, and around 15% of people at risk choose to have it. Both choices are reasonable.

Signs you might notice

Movement: involuntary jerky movements called chorea, clumsiness, difficulty with balance, and later with speech and swallowing.

Thinking: slowed processing, difficulty planning and switching tasks, poor insight.

Mood: depression, irritability, apathy, and sometimes obsessive or aggressive behaviour. These often come first, years before the movement problems.

The psychiatric symptoms are usually what disrupts life earliest, and they are the part families are least prepared for.

How it can affect day-to-day life

Apathy is regularly mistaken for laziness or depression, and it responds to neither reassurance nor pressure. It is a symptom of the damage, and treating it as a choice damages relationships badly.

The family dimension is unlike anything else in this library: somebody may be caring for a parent while knowing they have a one in two chance of the same illness, and while deciding what to tell their own children.

Dysphagia becomes a central risk in later stages, and aspiration pneumonia is a common cause of death.

Suicide risk is elevated, particularly around the time of diagnosis and testing.

Supporting someone well

Treat apathy as a symptom. Start tasks alongside the person rather than asking them to start.

Keep calories high. Chorea burns an enormous amount of energy, and weight loss is common and harmful. Fortified, frequent food beats three meals.

Get speech and language therapy involved early for swallowing, not once there is a problem.

Simplify choices and reduce time pressure. Ask one question and wait.

Plan legally and financially while capacity is clear.

Refer the family to genetic counselling, and do not push anybody towards testing. It is a decision with lifelong consequences for insurance, family and identity.

Fiducia Guardian holds the whole picture, medicines, therapy plans, consents and the care team, which matters in a condition where the care team grows steadily over fifteen years.

Where to get help

The Huntington's Disease Association have specialist advisers across the UK and are the best first call for families.

Ask for referral to a neurologist and to a regional genetics service.

Care Act and carer's assessments from the local authority, and do them early rather than in crisis.