Huntington's Disease

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Huntington’s disease or HD is a rare, degenerative brain disorder. The disease causes brain cells to die including those that control movement. HD symptoms worsen as the disease progresses. These symptoms can cause uncontrolled body movements known as chorea. HD is also responsible for behavioral changes and cognitive problems. Victims will also experience slurred speech, impaired coordination, and difficulties swallowing and feeding. According to the Huntington’s Disease Society of America, about 41,000 people in the U.S. live with HD. More than 200,000 others have an increased risk of inheriting the disease. Symptoms usually begin in adult years between the ages of 30 and 50. They can however begin early on in life, a condition known as juvenile HD. Symptoms worsen as the disease progresses. Huntington’s disease has no cure. Medication and treatment options available help manage the symptoms. 

Types of Huntington’s Disease

Huntington’s disease falls into two main categories depending on when symptoms appear. 

Adult-Onset Huntington’s Disease

It is the most common and usually begins when someone is in their 30s and 50s. In the early stages, the disease will cause symptoms like small involuntary movements, depression, irritability, poor coordination, hearing, and learning problems. Most people with adult-onset HD develop twitching and jerking movements referred to as chorea. As the disease continues to progress, the movements are more pronounced. When that happens, the person will have trouble speaking, walking, and even swallowing food. The person will also experience personality changes and reasoning and thinking abilities decline. The life expectancy is usually about 15 to 20 years once symptoms begin. 

Juvenile Huntington’s Disease

Juvenile HD occurs in children and adolescents. The child will experience involuntary movements and emotional and mental changes. Other signs of juvenile Huntington’s disease are clumsiness and frequent falls. The child will also drool a lot, have slowed movements, slurred speech, and rigidity. Their school performance will also decline because of declining reasoning and thinking abilities. 30 to 50% of children diagnosed with juvenile HD will also experience seizures. The disease progresses faster than adult-onset HD. As such, children diagnosed with juvenile HD have 10 to 15 years to live. 

Symptoms of Huntington’s Disease

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Huntington’s disease cause changes in movement. It also leads to psychiatric and cognitive disorders. The symptoms that appear first vary from one individual to another. Some symptoms can also be more prominent than others. Common symptoms include;

  • Involuntary twitching and jerking movements referred to as chorea
  • Muscle rigidity and contracture or dystonia
  • Abnormally slow eye movement
  • Poor balance, posture, and gait
  • Swallowing and speech difficulties
  • Difficulties focusing, organizing, and prioritizing tasks 
  • Tendency to remain stuck on an action, behavior, or thought
  • Loss of impulse control leads to outbursts and risky actions like sexual promiscuity
  • Slowness processing thoughts 
  • Learning difficulties where one has problems comprehending new information
  • Depression
  • Irritability
  • Insomnia
  • Fatigue and lack of energy to perform tasks
  • Suicidal ideations

Diagnosis of Huntington’s Disease

To diagnose HD, the doctor will go through your medical and family history. They will also ask about symptoms like emotional changes. If the doctor suspects Huntington’s disease, they will request a neurologist’s counsel. Imaging tests like CT and MRI scans help rule out brain disorders. Genetic testing also helps diagnose Huntington’s disease. A person with a family history of Huntington’s disease will need genetic testing. Genetic testing helps determine if you carry that specific gene mutation. Asymptomatic people also go for genetic testing to determine if they are likely to develop the disease. Since symptoms of HD usually begin when someone is in their 30s and 50s, going for genetic testing is vital. 

How to Treat Huntington’s Disease

There is currently no cure for Huntington’s disease. Treatment options available only help manage the symptoms. They include;

Medications

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The FDA has currently approved two medications to help treat HD symptoms. These are Tetrabenazine (Xenazine) and Deutetrabenazine (Austedo). Tetrabenazine helps treat involuntary movements caused by Huntington’s disease. The drug, however, can cause side effects like depression, suicidal ideations, and actions. Deutetrabenazine also helps treat involuntary movements but mostly those that occur in the tongue and face. Should you experience any mood changes or depression while on these medications, talk to a professional.

Other drugs help control movements, hallucinations, and outbursts. They include clonazepam (Klonopin), clozapine (Clorazil) and haloperidol. Side effects may include stiffness, rigidity, and sedation. Huntington’s disease can cause obsessive-compulsive disorders and depression. The doctor will then prescribe fluoxetine (Prozac, Sarafem), citalopram (Celexa), or sertraline (Zoloft).

Speech therapy

Huntington’s disease causes one to lose control of certain muscles like those of the mouth and throat. This can make speaking, eating, and swallowing difficult. Speech therapy helps one improve the lost skills. Speech therapy can also help teach non-verbal communication.

Occupational and physical therapy

Physical therapy helps improve muscle flexibility and strength. It is also used to help improve balance and reduce the risk of someone falling. Physical therapy involves using specific exercises that are adjusted after a while. It may also involve using walking aids like a walker or wheelchair. Occupational therapy is all about using supportive devices to improve quality of life. 

How to Live With Huntington’s Disease

Huntington’s disease is incurable and fatal. Adults diagnosed in their 30s and 50s have a lifespan of 15 to 20 years while children have 10 to 15 years. When the symptoms begin, they keep worsening as the disease progresses. There are, however, ways of managing the disease. 

  • Chorea can end up affecting your ability to do certain activities like writing, dressing, driving, and even eating. Involuntary movements can be managed through medications, occupational and physical therapy. 
  • People with HD have problems remembering and comprehending new information. Memories are, however, not affected and someone can continue doing the same job as long as their physical ability is not affected. 
  • Counseling, medications, and psychotherapy can help treat behavioral and mood changes. 
  • Nutritional changes since involuntary movements can leave someone feeling fatigued. A change in diet will help replenish their energy.
  • Joining support groups for Huntington’s disease can help you learn how to cope with the disease. It also provides the kind of emotional support one needs to live with the disease daily. 

FAQ

  1. What is the prognosis of Huntington’s disease? The disease has no cure and symptoms progress over time. Depending on the disease progression rate, most people die within 10 to 30 years after symptoms begin. Most deaths are due to injuries due to falls and pneumonia. 
  2. What is the first tell-tale sign that someone has HD?  The first signs could be personality and mood changes and fidgeting. The person will become clumsy, forgetful and may have difficulties remembering things. 
  3. Who is at risk of getting HD?  You are at risk of Huntington’s disease if you have the Huntington gene mutation. Gene testing can tell you early on if you will develop the disease at some point.

Healthier Me Today is intended for informational purposes only. It is not a substitute for professional medical advice, diagnosis, or treatment. Never ignore professional medical advice in seeking treatment, always consult with your healthcare professional. Stay healthy!

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