Contents. 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.
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.
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.
Research on the evolutionary genetics of this disease suggests that there are two main reasons for the persistence of Huntington's in human populations: mutation coupled with weak selection.
Huntington's strengthens the immune system during most fertile years allowing them to produce more offspring. Symptoms associated with Huntington's occur later in life, after peak reproductive age.
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 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.
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.
Common end-of-life signals include significant weight loss, difficulty swallowing or breathing, recurrent aspiration pneumonia, and uncontrolled pain.
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.”
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.
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.
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.
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 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.
memory lapses. depression – including low mood, a lack of interest in things, and feelings of hopelessness. stumbling and clumsiness. mood swings, such as irritability or aggressive behaviour.
The disease attacks areas of the brain that help to control voluntary (intentional) movement, as well as other areas. People living with HD develop uncontrollable dance-like movements (chorea) and abnormal body postures, as well as problems with behavior, emotion, thinking, and personality.
(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.
Surprisingly, many people with Huntington's disease aren't aware that the involuntary movements are happening, or don't notice them as much as other people do. That's because Huntington's disease can affect people's awareness of their own bodies.
Myth 4: HD can skip generations. Fact: The HD gene mutation never skips a generation.