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.
The advanced stage lasts between 11 and 26 years from disease onset. Patients with Huntington's at this late stage will need total support in daily activities from professional nursing care.
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.
During late-stage Huntington's disease, or stage V, individuals experience speech difficulty, weight loss, and loss of bowel and bladder control. They will have dementia and severe limitations in voluntary movement and will be fully dependent on assistance.
Common end-of-life signals include significant weight loss, difficulty swallowing or breathing, recurrent aspiration pneumonia, and uncontrolled pain.
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.
Stage 3: Late Intermediate Stage
Generally, the disease will have affected them enough that they will require assistance with everyday tasks. This stage will generally carry on for around five to sixteen years. By now, symptoms will be more pronounced and include those such as: Issues with walking.
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.”
Frustration, Irritability, and Anger
Your loved one with HD may become easily upset, angry, and stuck on an action or idea. It is important to remember that these outbursts of anger are commonly the result of HD, and the person with HD may not understand that you are trying to help them.
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.
The most common sleep problems reported by HD patients include insomnia, difficulties in falling asleep, frequent nocturnal awakenings, and excessive daytime sleepiness.
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.
Weight loss can make symptoms worse and weaken the patient's immune system, making them more vulnerable to infections and other complications. Huntington's disease itself is not usually fatal, but it can lead to choking, pneumonia, or other infections that can lead to death.
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.
When alcohol becomes a coping mechanism for Huntington's disease, that's when it becomes a problem. “With any neurodegenerative or medical disease, drinking a lot can certainly affect your balance and your gait,” says Dr. Oguh.
Symptoms of Huntington's disease usually develop between ages 30 and 50, but they can appear as early as age 2 or as late as 80.
People with Huntington's disease usually die within 15 to 20 years of their diagnosis. The most common causes of death are infections (such as pneumonia) and injuries related to falls.
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.
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.
Huntington's disease (HD) is an inherited disorder that causes nerve cells (neurons) in parts of the brain to gradually break down and die. The disease attacks areas of the brain that help to control voluntary (intentional) movement, as well as other areas.
(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.