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.
From the onset of symptoms, people with HD have a life expectancy of 10 to 25 years. HD is not evident at birth. In fact, symptoms will usually not appear until a person is between 35 and 55 years of age, and occasionally even later in life.
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.
Huntington's is a progressive disease. Once the symptoms appear, they continue and worsen over time. In most cases, they become noticeable between the ages of 30 and 50 and typically begin gradually. Although their onset and progression can vary from person to person, they fall into three basic categories.
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.
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 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 is caused by a faulty gene. Cells in parts of the brain are very sensitive to the effects of the faulty gene. This makes them function poorly and eventually die. A parent with the Huntington's disease gene has one good copy of the gene and one faulty copy.
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.
“To date, there is no particular treatment that will actually prevent Huntington's disease or definitely slow down the progression,” Dr. Ross says. “Researchers have found that aerobic intensity can help improve motor function and improve fitness.
(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.
The time when symptoms actually show up varies between people, however. There is evidence that about 40% of this variation is due to other genetic factors, and about 60% is due to unknown influences in a person's life or surroundings - which scientists refer to as environmental factors.
The range of disease duration was between 2 and 17 years, the oldest living to age 91.
There have been reports that juvenile onset Huntington's chorea is almost always inherited from the father, and that late-onset Huntington's chorea is inherited more often from the mother than from the father.
Stage 4: Early advanced stage
Typically lasting between nine and 21 years from disease onset, the early advanced stage is characterized by a need for full assistance in daily living. A person in this stage of Huntington's cannot live independently.
Familial prion disease may produce a diverse range of phenotypes, even within the same pedigree. It may resemble HD with prominent personality change, psychiatric symptoms and cognitive decline, chorea, rigidity, and dysarthria. Limb and truncal ataxia and seizures may be present.
Although anyone can develop HD, it tends to run in people of European descent (having family members who came from Europe). But the main factor is whether you have a parent with HD. If you do, you have a 50% chance of also having 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.”
Huntington's patients are often irritable and angry. They may feel frustrated, become stubborn and lose their tempers, and sometimes these feelings result in violent outbursts.
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.
Mood and behavioral changes
Agitation, irritability, and aggression are other possible personality changes. Some patients may experience hallucinations and delusions that can severely affect their day-to-day interactions. Living with Huntington's can induce feelings of anxiety, depression, apathy, and frustration.
The early symptoms of Huntington disease are often general: Irritability. Depression. Mood swings.