What is this Disease?
Huntington’s disease (HD) is a rare, inherited brain disorder. It slowly changes how a person moves, thinks, and feels. Usually symptoms occur in adult age, between the age of 30-50. They can occur both much earlier and much later in life. The symptoms worsen gradually over many years. HD is not contagious. It affects both men and women. If one parent has HD, each child has a 50% chance of inheriting the condition. HD can impact the whole family, not just the person with the condition.
What does HD do to the Brain and Cells?
Huntington’s disease gradually affects nerve cells in specific areas of the brain, especially those involved in movement, thinking, behaviour and emotions. The abnormal huntingtin protein damages the way brain cells function and communicate with each other. Over time, some nerve cells become less efficient and eventually die. This leads to the progressive changes in movement, thinking and mental health that are characteristic of Huntington’s disease.
Genetic Basis
Huntington’s disease is caused by a change in the HTT gene. The gene contains a repeated section of DNA called a CAG repeat. In people with Huntington’s disease, this section is repeated too many times.
The condition is autosomal dominant, which means that a person who carries the disease-causing gene change has a 50% chance of passing it on to each child.
A genetic test can confirm whether a person carries the Huntington’s disease gene change. Genetic counselling is strongly recommended before and after predictive genetic testing, especially for people who do not yet have symptoms.
What are the Symptoms? When do they Start and how do they Progress?
Symptoms most often begin in adulthood, commonly between the ages of 30 and 50, although they can start earlier or later.
Huntington’s disease can affect people in several different ways:
- Movement: involuntary movements called chorea, stiffness, slowness, problems with balance, walking, speech and swallowing.
- Thinking: difficulties with planning, organising, concentration, decision-making and processing information.
- Emotions and behaviour: depression, anxiety, irritability, apathy, changes in behaviour and, in some people, other psychiatric symptoms.
- Daily functioning: as the disease progresses, work, household activities, communication and self-care may become more difficult.
The disease progresses gradually over many years. The combination and severity of symptoms vary considerably between individuals. Not everyone develops the same symptoms or in the same order.
How can I take care of myself along the way?
Although there is currently no cure for Huntington’s disease, good multidisciplinary care can help manage symptoms and maintain independence and quality of life for as long as possible.
It is helpful to:
- have regular follow-up with a healthcare team experienced in Huntington’s disease;
- stay physically active and maintain balance, strength and mobility;
- pay attention to nutrition, weight and swallowing difficulties;
- seek early support for depression, anxiety, irritability or other emotional changes;
- use occupational therapy and practical adaptations to maintain independence;
- keep socially active and connected to family, friends and patient organisations;
- plan ahead for changes in work, finances, driving, legal matters and future care;
- involve family members and caregivers in care planning when appropriate;
- ask for help early rather than waiting until difficulties become severe.
Care should be adapted over time as needs change.
The Ideal Care Team
Because Huntington’s disease can affect movement, cognition, mental health, communication, nutrition and family life, the best care is usually provided by a multidisciplinary team. This may include:
- Neurologist / Huntington’s disease specialist – coordinates medical care and treatment of movement and other neurological symptoms.
- Psychiatrist – supports treatment of depression, anxiety, irritability, behavioural changes and other psychiatric symptoms.
- Clinical psychologist / neuropsychologist – provides psychological support and assesses cognitive and behavioural changes.
- Geneticist and genetic counsellor – provides information and counselling about inheritance, predictive testing and family planning.
- Physiotherapist – supports mobility, balance, strength, posture and prevention of falls.
- Occupational therapist – helps maintain independence in daily activities and recommends adaptations at home or work.
- Speech and language therapist – supports speech, communication and swallowing.
- Dietitian / nutritionist – helps maintain adequate nutrition and weight and adapts food when swallowing becomes difficult.
- Social worker / case manager – supports access to social services, benefits, employment support, care services and practical resources.
- Primary care physician / GP – supports general health and coordinates care for other medical needs.
- Specialist nurses, where available – provide monitoring, education and practical support.
- Patient organisation and peer-support groups – provide reliable information, practical guidance, advocacy and connection with other families.
- Palliative care team, when needed – helps manage complex symptoms and supports quality of life, patients and families throughout advanced stages of the disease.
For Huntington’s disease I would also explicitly add support for family members and caregivers, because HD is a genetic and family condition, and the needs of the family often change together with the needs of the person living with the disease.