Huntington's disease in children

Huntington's disease in children

Huntington's disease in children

Huntington's disease is a hereditary and fatal disease that causes the destruction of nerve cells in the brain. It weakens the physical and mental faculties and worsens over time. There is no medicine. If it starts at an early age, it is called juvenile Huntington's disease.

The diagnosis of Huntington's disease can be a shock. There are many things to accept.

But using a support system like a social worker, therapist, or support group can make the journey a little less intimidating. With the help of a medical team, people with Huntington's disease can live independently for many years.

Symptoms of Huntington's disease

Although the first symptoms may appear in middle age, Huntington's disease can affect anyone from childhood to old age. Symptoms often first appear when people are in their 30s and 40s. Over the course of 10 to 25 years, the disease gradually kills nerve cells in the brain. It affects the body, mind and emotions. If symptoms appear before the age of 20, this is called juvenile Huntington's disease and may progress more rapidly.

Symptoms can vary greatly from person to person. And stress or anxiety can exacerbate symptoms.

Some symptoms are easier to notice than others. Abnormal movements may be the first thing you notice. Weight loss can be a challenge at all stages.

The symptoms of Huntington's disease tend to develop in stages.

Early stage symptoms

  • Changes may be quite subtle in early stages, making it possible to keep driving and working. You may just require a little extra help.
  • Common early symptoms include:
  • Trouble learning new things
  • Trouble making decisions
  • Memory lapses
  • Mood swings
  • Clumsiness
  • Slow or abnormal eye movements
  • Muscle problems (dystonia)
  • Trouble sleeping (insomnia)
  • Loss of energy and fatigue

Symptoms of the intermediate stage

Over time, the symptoms begin to interfere with your daily life more. For example, you may start dropping things or falling. Or you may have trouble speaking or swallowing.

Staying organized can be difficult. And emotional changes can create tension in a relationship.

Common symptoms of the intermediate stage include:

  • Common middle stage symptoms include:
  • Uncontrolled twitching movements (chorea)
  • Trouble walking
  • Confusion
  • Memory loss
  • Personality changes
  • Speech changes
  • Decline in thinking abilities
  • Swallowing problems
  • Breathing problems
  • Thoughts of death, dying, or suicide
  • Weight loss
  • Development of obsessive-compulsive disorder, bipolar disorder, or mania

Late stage symptoms

During this stage, people with Huntington's disease must depend on others for care. Walking and talking is impossible. Most likely, you are always aware of loved ones around you. Restless movements may become severe or may disappear.

Symptoms of juvenile Huntington's disease

In children or adolescents, Huntington's disease may progress more quickly and cause symptoms such as:

  • Stiff or awkward walking
  • Increased clumsiness
  • Changes in speech
  • Trouble paying attention
  • Sudden drop in school performance
  • Behavior problems
  • Tremors
  • Seizures

Diagnosis of Huntington's disease

Family history plays an important role in diagnosis. Your doctor will ask you about your medical history and perform a physical examination. If you and your doctor suspect Huntington's disease, a neurologist will perform additional tests.

A neurologist may check:

  • Reflexes
  • Muscle strength
  • Balance
  • Sense of touch
  • Vision
  • Hearing
  • Mood and mental status
  • Memory
  • Reasoning
  • Thinking skills
  • Speech

Treatment and home remedies for Huntington's disease

For now, treating Huntington's involves managing symptoms:

  • Medications can help control restless movements. Your doctor can work closely with you to manage side effects and change medications if needed.
  • Some antipsychotic drugs have a side effect that controls movement and helps some people.
  • Antidepressants can also help with obsessive-compulsive disorder.
  • Mood-stabilizing medications can relieve symptoms of mood disorders, but may cause other side effects.
  • Speech therapy can help with any problems with speech or swallowing.
  • Occupational therapy or physical therapy can help you learn to control your movements better. And assistive devices like handrails can help you cope with changing physical abilities.
  • Nutritional support ranges from the use of special utensils to tube feeding in later stages.
  • Exercise can be very helpful. People with Huntington's disease who stay as fit and active as possible do better than those who don't.
  • Psychotherapy can teach you how to deal with changes in your emotions and the way you think. Strategies such as breaking tasks down into simpler steps can make these changes much easier for you and your family.

Family members can help by making some home changes:

  • Serve extra meals and add high-calorie supplements to help you stay at a healthy weight.
  • Limit distractions during meal times.
  • Choose foods that are easier to chew and swallow.
  • Use forks and other utensils made for people with limited motor skills.
  • Use covered cups with straws or drinking spouts.
  • Keep a regular routine.
  • Use phone or computer reminders for tasks.
  • Keep life as calm, simple, and low-stress as possible.
  • For children, work with the school counselor to make an education plan.
  • See friends and maintain social interactions as much as possible.
  • Add wheelchair ramps and elevators to the home if possible.
  • Add safety bars in bathrooms, next to the bed, and at stairs.
  • Use voice-controlled lights and other “smart” home features.
  • Use electronic speech programs or picture charts to aid communication.

Causes of Huntington's disease

In 1993, researchers discovered the gene that causes Huntington's disease. Everyone carries the HD gene, but in some families, an abnormal copy of the gene is passed from parent to child. If you have a relative with Huntington's disease, you have a 50% chance of being a carrier of the gene and getting the disease.

Also:

  • Men and women are equally likely to inherit the abnormal gene.
  • If you do not have the abnormal gene, you cannot get Huntington's disease or pass it on to your children.
  • The disease does not pass from generation to generation.

If you or your family members are considering getting tested for Huntington's disease, it is recommended that you first seek professional genetic counseling. Counselors can help explain what to expect from the test results.

By knowing the gene for Huntington's disease, scientists have been able to learn a lot about how the disease affects the brain. More importantly, this discovery could help pave the way for future treatments. 


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