Huntington's disease is a rare, inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington's disease has a wide impact on a person's functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders.
However, almost all people with HD will manifest disease-specific personality and behavioral changes as part of what might be termed a hypofrontal or dysexecutive syndrome, characterized by apathy, irritability, impulsivity, and obsessionality, with potentially severe consequences for the HD person's marital, social, ...
Some of the suggestions I have for living with HD are: Keep life simple - Rest, Exercise, Nutrition, A daily laugh Carry earplugs with you when you are out (Some people with HD are sensitive to noise) Carry calendars with you to write down everything you must do for the day and any information you get from other people.
Psychiatric manifestations in HD include depression, irritability, apathy, obsessions, and occasionally psychosis (4). Psychosis is defined by the presence of delusions and/or hallucinations (5). The prevalence of psychotic symptoms in HD patients is variable, ranging from 3 to 11% (6).
During the early stages of HD, irritability, depression, anxiety and aggression are common behavior symptoms. In the later stages, individuals tend to be less irritable and aggressive, as apathy and a lack of concern become more pronounced.
Huntington's disease also causes a decline in thinking and reasoning skills, including memory, concentration, judgment, and ability to plan and organize. Huntington's disease brain changes lead to alterations in mood, especially depression, anxiety, and uncharacteristic anger and irritability.
Huntington's disease impairs the functioning of the brain, which can result in apathy, trouble organizing, impulsivity, irritability and anger, unawareness, disinhibition, preservation, and other psychiatric symptoms. These emotional and behavioral symptoms can further complicate the caregiver's role.
These patients often display a range of psychiatric symptoms, for example hallucinations or low mood, which coexist with behavioural change and symptoms such as apathy, irritability and aggression.
Stage 5: Advanced Stage
The fifth and final stage is when the disease will be more severe, and it's likely that the individual will require care 24/7. They will likely be immobile, unable to communicate and find it extremely difficult to perform basic motor functions such as swallowing.
Hospice referrals for Huntington's can be challenging because it's difficult to predict decline. Common end-of-life signals include significant weight loss, difficulty swallowing or breathing, recurrent aspiration pneumonia, and uncontrolled pain.
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 bottom line. Being physically and mentally active in life is good for everyone, and particularly for those at risk of developing HD, because it might affect symptom onset.
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.”
Psychosis is a rare symptom complex that occurs in Huntington's disease and has a negative impact on the quality of life for patients. Psychiatric manifestations vary and may precede motor and cognitive changes. Personality changes and depression occur most commonly.
Medications for psychiatric disorders
Possible treatments include the following: Antidepressants include such drugs as citalopram (Celexa), escitalopram (Lexapro), fluoxetine (Prozac) and sertraline (Zoloft). These drugs may also have some effect on treating obsessive-compulsive disorder.
Depression and suicide are common among those with Huntington disease. Antidepressants and antianxiety medications may be prescribed to treat these symptoms.
Pneumonia and heart disease are the two leading causes of death for people with HD.
Palliative care has its origins in cancer care, but is important for all kinds of chronic and progressive diseases, including HD [17]. Its relevance in addressing needs may increase with increasing severity and progression of the disease, with increasing loss of functional capacity.
Huntington's disease (HD) is an autosomal dominant neurodegenerative disorder that usually starts in mid-adult life. The clinical disease progresses to death over an average of 20 years.
The most frequent psychiatric sign occurring in HD patients consists of a depressive symptomatology (DS) [28]. The diagnosis is sometimes difficult because somatic expression of depressed mood (i.e., apathy, inactivity, and weight loss) also occurs in HD patients without psychiatric problems [13].
Medications such as tetrabenazine can be prescribed for chorea,10 and antipsychotic drugs such as haloperidol can be used for psychotic symptoms. Symptomatic management, however small, can improve the patient's ability to cope and quality of life.
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.
HD patients present a broad range of psychological disturbances such as cognitive rigidity, mood disturbances, lack of empathy and breakdowns of social relationships that might also manifest even before the onset of motor dysfunction (Marvel and Paradiso, 2004).
Although not directly related to HD, stress is nevertheless related to the progression of the disease because it adds to the neurodegeneration that is already taking place. Chronic stress can alter nerve cells, brain structure, and brain function.