Staten Island Dementia Care for Huntington Disease
At The Brielle, we provide residents with exceptional dementia care and attention to their individual needs. This includes meeting the challenges of Huntington’s disease. Our staff is highly trained in memory impairments, including dementia and Huntington’s disease, to ensure every resident has the support they need.
If you or someone you know is struggling with this diagnosis, we’re here to help you. Be sure to reach out to learn more about how we can help you.
What is Huntington’s Disease?
Huntington’s disease is a rare condition in which there is a continuing breakdown of the nerve cells located in the brain. This degeneration of nerve cells isn’t fully understood and physicians don’t know why it happens. The condition, just one of many types of dementia, is less common than Alzheimer’s disease. The Alzheimer’s Association states that Huntington’s Disease impacts 1 in every 10,000 people, with about 30,000 people in the U.S. with symptoms of it at any given time. About 200,000 people in the U.S. are at risk of inheriting this condition at some point in their lives.
Huntington’s disease can be difficult for both the individual with the disorder and their family, causing problems with memory, movement and the ability to function independently. Because of how difficult it can be to handle Huntington’s disease, it’s important for people dealing with this condition to understand what they are facing and, whenever possible, to be proactive in preventing its onset.
What Causes Huntington’s Disease?
Huntington’s disease is a genetic disease, meaning it is often inherited. It is caused when a single defective gene changes up a person’s genetic code.
There are a total of 23 chromosomes that provide the detailed print or genetic code for each person. Researchers have found that a defective gene on chromosome 4 carries one of the main risk factors for Huntington’s disease. Doctors also know that this gene is considered dominant, which means anyone who inherits the defect on chromosome 4 isn’t likely to develop the condition right away. However, they are very likely to develop the condition at some point in their lives.
When the gene and its defect change from dormant to active, it will cause changes in a normal protein known as huntingtin. Huntingtin is present in everyone, but when the defective gene goes active, it causes changes in the blueprint and creation of the protein, creating an abnormal version of Huntingtin which causes Huntington’s disease.
When the defect begins to cause changes, the disease’s symptoms will start presenting themselves. This often begins with involuntary and abnormal movements. It may include declining thinking and reasoning skills. Many people begin to experience intense mood changes. Depression is a common factor. All of this occurs in the area of the brain that controls movement, thinking skills and mood.
Symptoms of Huntington’s Disease
It’s possible for Huntington’s disease symptoms to begin at any time in life. However, they typically begin to occur in a person’s 30s and 40s. It is possible, but less likely, for symptoms of Huntington’s disease to develop prior to the age of 20. If that occurs, it is considered juvenile Huntington’s disease. Most often, when it presents at an early age, the symptoms tend to be amplified, and the disease moves rapidly.
There are many potential Huntington’s disease symptoms. Symptoms can be psychiatric, cognitive and movement related. Here are some of the most common symptoms.
Movement changes such as:
- Jerking or writhing movements
- Trouble with swallowing and speaking
- Difficulty maintaining posture and balance
- Slow eye movements, sometimes abnormal eye movements
- Muscle spasms, rigidity or contracture
Psychiatric changes, such as:
- Sadness and depression
- Fatigue and a loss of energy
- Thoughts of suicide or dying
- Social withdrawal
Cognitive changes, including:
- Inability to focus on or prioritize tasks
- Trouble with organization
- Lack of awareness of one’s behavior
- Difficulty learning or understanding new information
- Slow thought processes
- Trouble finding the right words
Some people will experience mania and obsessive-compulsive disorder. Bipolar disorder may present in some people with Huntington’s disease as well. Most often, the condition progresses over a period of months and years, and not all of these symptoms are present at the same time.
Stages of Huntington’s Disease
Huntington’s disease is a progressive condition, meaning it will get worse over time, especially as the condition begins to move into more severe levels. There are several notable stages.
Stage 1 Huntington’s Disease
In stage one, a person can be diagnosed with the condition, generally from a genetic test. He or she is still able to function normally with limited or mild symptoms. Most people at this stage are not likely to experience more impairment in motor systems, but there are likely to be some psychiatric symptoms and mild cognitive changes.
Stage 2 Huntington’s Disease
Called the early intermediate stage, stage two typically begins three to 13 years after the disease’s onset and is when a person is likely to see more widespread changes. They may still be able to work and function in most areas, but they will have a lower capacity than other people their age. They may need some support for daily functions. A person may also begin to experience chorea, a condition in which irregular involuntary movements occur, especially as the condition progresses.
Stage 3 Huntington’s Disease
In this stage, which generally begins 5 to 16 years from the start of the condition, a person is struggling to manage normal household activities and work tasks. This is also when the impairment of a person’s ability to think occurs. They may be more irritable, impulsive and anxious. Motor impairments, cognitive changes and psychiatric symptoms tend to worsen.
Stage 4 Huntington’s Disease
In this early advanced stage, which begins nine to 21 years after initial onset, individuals with Huntington’s disease are no longer independent, but may still be able to live at home with a caregiver. They almost always need additional help to complete daily activities. They may still be aware of activities that need to happen, but they cannot do them on their own.
Stage 5 Huntington’s Disease
In this advanced stage, which occurs 11 to 26 years after disease onset, the condition will worsen to the point of a person needing 24-hour care. They are likely to develop stiffness, abnormal limb postures and slowness in all functional aspects. They are likely to fall. Many will see advanced speech problems, difficulty with swallowing and changes in body temperature.
Progression of Huntington’s Disease
The progression of Huntington’s disease follows its own path. However, most often, this process of moving from stage one through stage five takes between 15 and 20 years from the first sign of symptoms.
At some point in the progress, typically between stages three and five, the condition will progress further to a point where an individual is unable to function on their own. They may suffer from heart failure, infection or pneumonia in the latest stages as their body struggles to manage functions.
Risk Factors of Huntington’s Disease
Since Huntington’s is a genetic disease, the biggest risk factor is having a close family member or relative who has had the disease. Because it is a dominant gene, it is a very high likelihood for a child to inherit this condition if their parent has it. Keep in mind that every child in a family has about a 50 percent chance of getting this genetic disorder.
If someone in your family, especially a parent, has had the disease, you should speak to a physician about being tested for the defective gene. Knowing if you have the gene can help you better prepare for Huntington’s disease, including finding the right support options.
Aside from the defective gene, researchers are not fully sure what causes Huntington’s disease to occur, so they are unable to pinpoint many additional risk factors. It’s also important to know that doctors are not fully sure how the huntingtin protein works normally. They also do not understand how even a small number of genetic defects can cause such an incredible response from the body leading to the devastating symptoms of this condition. Researchers continue to work to better understand the cause and risk factors of the condition as a result.
Diagnosis of Huntington’s Disease
The diagnosis of Huntington’s disease is often made by looking for symptoms and genetic factors in a person’s family history. If a close relative has been diagnosed with Huntington’s disease, you can get a genetic test that can be used to help determine if you’re likely to develop the condition. This test can be done well in advance of symptoms presenting.
If someone does not have a known family history of the condition, then diagnosis typically occurs when symptoms are obvious. Once someone presents with symptoms doctors can run a genetic test to determine if it is Huntington’s disease or if another condition that is causing symptoms.
Treatment for Huntington’s Disease
There is no cure for Huntington’s disease. However, there are medications that can help reduce symptoms and provide for a person’s needs more effectively. In addition, these treatments may work to slow down the progression of the disease.
Medications are one of the most important treatment options for those with Huntington’s disease, although they are likely to change as the condition changes. Some may have their own side effects. As the condition progresses, each person is likely to need a new set of treatments and interventions to reach a stable level.
Medication for Muscle and Movement Problems
There are medications that can improve muscle and movement disorders. Some drugs can help individuals gain more control over movement while others are designed to be antipsychotic drugs, but can also suppress movement issues.
Medications for psychiatric disorders are commonly necessary, as well. This often includes mood-stabilizing drugs, such as those to help control bipolar highs and lows, and antidepressants, used to help with depression symptoms.
Other Huntington’s Disease Treatments
Psychotherapy can help provide support for behavioral problems that often develop, and can help a person learn to cope with their condition and manage the progression of the disease. Speech therapy can help keep the speech muscles in the mouth and throat working longer. Speech therapy can later progress into using tools to help improve communication when it is no longer effective in helping speech muscles function.
Physical therapy is also a treatment option for Huntington’s disease. Individuals can learn how to keep muscles strong and flexible, reducing the risk of falls and posture concerns. Over time, this type of therapy may also focus on learning how to use a walker or a wheelchair, which can give a person more independence over time. Occupational therapy can help with improving function, such as support for using assistive devices to help with dressing and bathing.
Care for Those with Huntington’s Disease
A person with Huntington’s disease is likely to grow more and more dependent on other people. In the final stages, it is often very important for family members to ensure their loved one remains safe, in a secure location that provides 24-hour support in an environment that’s still relaxing and feels like home. The quality of care a person receives is often a determining factor in their quantity and quality of life. Huntington’s disease care is always specialized but needs to be constant, especially in stages four and five.
Over time, there is a need to use care settings for a person, including round-the-clock support. These types of environments are safe and have the tools to help a person to continue with necessary occupational and physical therapy.
At The Brielle, we provide just this type of a supportive environment. Here, residents with Huntington’s disease receive the type and amount of care they need to live the highest quality of life possible. With enrichment programs, a holistic wellness philosophy and support services to meet their individual needs, many people facing the challenges of Huntington’s disease are happier and healthier here. With specialized dementia care services, your friend or family member with Huntington’s disease will get the best possible care available. Contact us at The Brielle for more information on how we can support you and your family.