Huntington’s Disease in the Elderly
Huntington’s disease has been a recognized disorder since the Middle Ages. At that time, it was simply referred to as chorea for the jerky dance-like movements associated with the illness. The first thorough description of it was made by George Huntington (for whom the disease was later named) in 1872.
What is Huntington’s Disease?
Huntington’s disease is a rare and progressive brain disorder that causes nerve cells in the brain to break down over time affecting the body, mind and emotions. It’s characterized by uncontrolled movements, emotional disturbances and cognitive difficulties.
In the U.S., it’s estimated the disorder affects approximately 30,000 individuals. Due to its genetic component, thousands of these individual’s relatives have a fifty percent chance of developing the disease at some point in their lives.
What Causes Huntington’s Disease?
Huntington’s disease is a genetic disorder caused by a defect of the Huntington disease gene located on chromosome number 4. Only one parent is required to have this gene for a child to inherit the disease; but, if a parent has the gene, their child has a fifty-fifty chance of inheriting it.
Huntington’s disease generally affects people of European ancestry and is less common in some populations including Japanese and African Americans. It is equally prevalent to both genders, and it doesn’t skip generations.
Although the disorder is primarily genetic, a small percentage of Huntington’s disease cases, one to three percent, occur even when there is no previous family history of the disease.
Signs and Symptoms of Huntington’s Disease
Typically, Huntington’s disease causes progressive movement, psychiatric and cognitive symptoms. Excitement and stress can cause symptoms to worsen.
In the early stages of the disease, change will be very subtle. Abnormal movements are likely to be the first symptom noticed causing slight changes in coordination and balance. The person may become clumsier. During the early stages, a person may be able to continue driving and working, although some help may be required. Mood may be affected causing irritability and depression.
As the disease progresses, symptoms begin to interfere with day-to-day activities. A person becomes even more clumsy, dropping things or falling more frequently. At this stage, medication to control the involuntary movements (chorea) may be considered. A person may begin to have more problems with talking or swallowing and seeing a speech-language pathologist would be beneficial. Emotional changes may begin to affect interpersonal relationships, but medications may be able to help with these symptoms as well. Staying organized may become difficult.
In the later stages of Huntington’s, care will need to be provided by others. Speaking and walking become impossible. Choking and aspiration become a major concern. The involuntary movements may become quite severe or may totally disappear. Although cognitive problems will worsen, a person is likely to still be aware of family and friends around them.
Symptoms will vary greatly from person to person. Individuals may experience the following:
Muscular symptoms of Huntington's disease :
- Fidgety involuntary movements (chorea)
- Increased muscle activity
- Abnormality walking
- Problems with coordination and balance
- Muscle spasms
- Loss of muscle
- Slowing and stiffness
- Stiff or awkward gait
- Increased problems with swallowing
Psychiatric/Psychological symptoms of Huntington's disease :
- Mood swings
Cognitive symptoms of Huntington's disease :
- Memory loss
- Mental confusion
- Lack of concentration
- Difficulty thinking and understanding
- Slow thought processes
- Trouble learning new things or loss of previously learned information
Behavioral symptoms of Huntington's disease:
- Compulsive behavior or lack of restraint
Weight loss is always a major concern. Tremor and changes in speech may also be common.
Treatment for Huntington’s Disease
Huntington’s disease requires a medical diagnosis which generally requires diagnostic imaging and laboratory tests. The condition is chronic, typically manifesting when a person reaches middle age in their 30s and 40s. Individuals with the most common form of Huntington’s usually live 15 to 25 years after onset of the disease. The most common causes of death are not the disease itself, but complications arising from the disorder including infection (generally pneumonia), heart failure due to a weakened condition, choking and fall-related injuries.
Treatment can help, but Huntington’s disease cannot be cured or reversed. Treatment, which usually consists of prescription medications, physical therapy and counseling or talk therapy to help manage the symptoms, will depend on the stage of the disease.
Various specialists can help control the symptoms related to Huntington’s. Using medications, a psychiatrist will address the mental components of the disease. A neurologist treats the symptoms related to the nervous system. A medical geneticist, a doctor who diagnoses and manages hereditary disorders, may be consulted. Physical therapists will work to restore muscle strength and function.
Treatments can include:
- Prescription medications to help control involuntary and fidgety movements and to control psychiatric and behavioral symptoms such as depression, irritability and anxiety
- Language or speech therapy to deal with speech difficulties and trouble with swallowing
- Physical and occupational therapy to restore muscle strength/function and to help the person learn how to control some of their body movements.
- Nutritional support beginning with a focus on nutrient-dense foods and, as the disease progresses, tube feeding
- Assistive devices such as special eating utensils, handrails and walkers help to manage changing and declining physical abilities
- Routine exercise has shown to be beneficial
If you or a member of your family intends to be tested for Huntington’s disease, it’s best to seek professional genetic counseling beforehand. The counselor can help you understand what to expect from those test results.
Care Options for Seniors with Huntington’s Disease
In the early stages of the disease, the person can live at home with some assistance or live in an assisted living community.
As the disease progresses, and as more assistance is required, the person may continue to live in an assisted living community or memory care facility, but at some point, the care necessary will be more than the facility can provide.
In the last stages of the disease, full care will be necessary. This often means moving into a skilled nursing facility or a hospice facility. A residential care home or adult family home may be an option as well, with the help of home health care agencies and hospice.
With enough assistance from family and friends, and with the help of home health care agencies and hospice, in-home care may be possible.
Various organizations are available to assist you as you make decisions about caring for someone with the disease or living with Huntington's yourself.
- Huntington's Disease Society of America offers online support groups
- Huntington's Disease Foundation
- International Huntington Association
- We Have A Face offers online support groups and other online resources
- Huntington Disease Lighthouse Families has a message board
- Huntington's Disease Association in the UK has a message board
- Huntington's Disease Treatment & Diagnosis Guide
- Inspire offers an online support group
- HOPES is a student-run project at Stanford University dedicated to making scientific information available