What Is Huntington's Disease?

What Is Huntington’s Disease?

Huntington’s disease is a degenerative condition that affects the central nervous system. As the condition advances, your symptoms worsen. It is also a progressive disease, so it comes on earlier and more severely as it is passed down to the next generation (since it involves a repeat of a defective gene).

Huntington’s disease is a rare condition. It’s even rarer in children and teenagers. Around 5.7 in 100,000 people have been diagnosed with Huntington’s disease in the United States. About 5% to 10% of that number are children. 

Types of Huntington’s Disease

There are two sub-types of Huntington’s disease, they include: 

• Juvenile Huntington’s disease: Children and teenagers can develop this condition in rare cases. They’ll typically exhibit the same symptoms as adults with this condition, alongside stiffness and seizures. 
• Adult-onset Huntington’s disease: This is the more common form of the condition. Symptoms of the disorder typically begin to appear between the ages of 30 and 50. 
  

Symptoms of Huntington’s Disease

Symptoms of Huntington’s disease typically occur between the ages of 30 and 50. In rare cases, children as young as five have exhibited signs of Huntington’s disease. These symptoms occur in stages and affect both your physical and mental well-being. 

Early-stage symptoms of the condition might not disrupt your daily functioning. However, as the disease progresses, symptoms can get so severe that you'll be unable to function on your own.

Early Symptoms 

Early symptoms of Huntington’s disease vary from person to person. In many cases, they go undetected, and a definite diagnosis isn’t made until symptoms have progressed.

Some of the most common early signs of Huntington’s disease include: 

• Mood swings
• Fidgeting 
• Changes in the way you move 
• Memory lapses 
• Trouble making decisions
• Anxiety 
• Trouble learning  
• Slight involuntary movements 
• Depression 

Later Symptoms 

In the later stage of Huntington’s disease, motor and cognitive abilities are severely affected. Some of the most common symptoms in this stage include: 

• Memory loss 
• Difficulty eating and swallowing 
• Worsened involuntary movements (chorea)
• Changes in the way you speak 
• Confusion 
• Personality changes 
• Tremors 
• Rigidity 
• Hallucinations 
• Trouble with coordination 

Complications of Huntington’s Disease 

Huntington’s disease progresses in severity. In its later stage, it’s possible for a person with the condition to develop dementia. Dementia is a condition that causes severe memory loss and changes to your personality. 

Causes of Huntington’s Disease

The exact cause of Huntington’s disease hasn’t been identified. It’s a hereditary disorder, which means that it has been passed down to you from a parent carrying the gene if you have it. If you have a parent with Huntington’s disease, there’s a 50% chance that you could also develop the condition.

However, it’s possible for people with no family history of Huntington’s disease to also develop the condition. People of European descent are also more likely to develop the condition than people of other backgrounds. 

Diagnosing Huntington’s Disease

Huntington’s disease shares many similar symptoms with other neurological disorders, making diagnosis difficult, especially in its early stage. For an accurate diagnosis to be made, your doctor will look at your family history and order for an extensive neurological and physical examination to be done. 

Genetic Testing 

For people with a family history of Huntington’s disease, it is typically recommended for genetic testing to be done, even though you are not exhibiting symptoms of the condition.

After the test is done, a genetic counselor will speak to you on the next steps to take. If you aren’t exhibiting symptoms of Huntington’s disease, learning that you carry the gene can be difficult. However, it can help you put plans in place to manage the condition better if it develops later in life. Other family members might also be asked to come in for genetic testing.

Treatment for Huntington’s Disease

There’s currently no cure for Huntington’s disease. However, several treatments make coping with the condition more manageable. These treatments are targeted at reducing the severity of your symptoms and improving your quality of life.

Medication 

Different types of medication targeted at the various symptoms that characterize this condition are prescribed to people with Huntington’s disease.

Doctors usually first seek to get chorea under control, especially in the later stage of the condition. Austedo (deutetrabenazine), Haldol (haloperidol), and Xenazine (tetrabenazine) are most commonly prescribed for chorea.

Other types of medication usually administered include: 

• Antipsychotics: These are prescribed to manage symptoms like hallucinations and aggression. 
• Antidepressants: An early symptom of Huntington’s disease is depression; however, this can persist in later stages. Antidepressants help to ease depression. 
• Mood-stabilizers: Mood stabilizers help with symptoms like mood swings and anxiety, especially when they are severe. 

Coping With Huntington’s Disease 

Living with Huntington’s disease can be difficult. Also, if you’ve just been diagnosed or found out that you are at risk of developing the condition, that can also be painful to deal with.

In that case, it’s essential to take care of your mental and physical health. Exercising regularly has been proven to help improve the quality of life of people living with Huntington’s disease.

Other ways you can take care of yourself to enhance the quality of your life include: 

• Eating healthy: A healthy diet is essential to keep your mental and physical well-being functioning as optimally as it can. 
• Join a support group: Connecting with other people going through a similar experience can help boost your morale. Support groups are also great resources to learn new tips to help you cope with living with the condition. 
• Think of the future: As the condition progresses, it will become increasingly challenging for you to function daily. If you’ve just been diagnosed, it’s essential to appoint a trusted person who will be in charge of your care, finances, and other important decisions that will need to be made.