The mutant protein huntingtin causes neurodegeneration in the brain, particularly in the caudate nucleus and putamen. The mean age at onset is in the third and fourth decade of life and the disease duration about 15-20 years. Death usually results from respiratory complications.
Huntington's Disease (HD) is not fatal in itself. People with HD have a shorter life expectancy and die of other life-threatening complications related to this disease. Pneumonia and heart disease are the two leading causes of death for people with HD.
Huntington's disease (HD) is one of the most devastating diseases of mankind. It incapacitates patients by affecting their ability to move, think, and behave normally. It causes uncontrollable and disabling movements of the face, neck, trunk and limbs, loss of balance, and uncoordinated movements.
The deterioration causes uncontrolled movements, emotional disturbance and loss of intellectual faculties. Advanced stages bring total reliance on caregivers. While the cause of the disease is known — a single mutated gene — there is no cure.
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.
Common end-of-life signals include significant weight loss, difficulty swallowing or breathing, recurrent aspiration pneumonia, and uncontrolled pain.
On average, individuals with Huntington's disease live for 10 to 30 years after the onset of symptoms. However, it is essential to note that this is an average estimate, and some individuals may live longer or shorter than this range.
People can start to show the symptoms of Huntington's disease at almost any age. Most will develop problems between the ages of 30 and 50. The condition gradually gets worse for around 10-25 years, until the person dies.
Summary. Although no disease-modifying therapies currently exist to slow or halt the progression of Huntington's disease, many new types of treatment are under investigation that may offer hope for the future.
People with Huntington's may also be less able to control their emotions, possibly leading to outbursts of screaming, swearing, slamming doors, hitting walls, or the like. Patterns of behavior can change as the disease progresses.
Huntington's disease is a progressive brain disorder caused by a single defective gene on chromosome 4 — one of the 23 human chromosomes that carry a person's entire genetic code. This defect is "dominant," meaning that anyone who inherits it from a parent with Huntington's will eventually develop the disease.
The most painful conditions in Huntington's, said Achterberg, are limb pain, headache, abdominal pain and fractures. “I think we should do more work in validation of pain tools, including observational pain instruments,” he said. “And for clinicians, I suggest you be especially aware of abdominal pain.”
For the purpose of this fact sheet, the beginning of end-of-life in HD is determined as the stage when the person affected has little control over movement, is bedbound, unable to communicate, unable to eat and drink on his/her own and experiences severe chorea or extreme rigidity.
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.
The range of disease duration was between 2 and 17 years, the oldest living to age 91.
After Huntington's disease starts, a person's functional abilities gradually worsen over time. The rate of disease progression and duration varies. The time from the first symptoms to death is often about 10 to 30 years. Juvenile Huntington's disease usually results in death within 10 years after symptoms develop.
Stage IV (Late Stage HD)
The progressive breakdown of nerve cells in late-stage Huntington's disease results in severe motor symptoms, including extreme difficulty with voluntary movements, severe involuntary movements, abnormal slow movements, rigidity, and speech impairment.
(For more information on Complications of HD, click here.) It is important to know that stress can lead to complications and the worsening of symptoms in people with HD.
While treatments exist to manage the symptoms of the disease, nothing has proven to be curative – yet.
The youngest symptomatic child documented had an age of onset of 18 months,3 with other children developing symptoms in their teens, meaning that some individuals with JHD may now be in their twenties.
Once the symptoms start to affect your daily life, you will be diagnosed with active disease. The disease is staged based on your motor function and ability to complete everyday tasks. Typically, HD progresses for 10 to 30 years.