Huntington’s Disease (HD) devastates a person’s ability to control thoughts and movements, usually at a much younger age than more common dementias like Alzheimer’s. HD is an inherited genetic disorder, passed down through parents, that has symptoms sometimes described as a combination of Alzheimer’s, Parkinson’s, and ALS. Motor skills and mood are profoundly affected by HD, while understanding and memory loss are less of a problem than for people who have Alzheimer’s disease. Caregiving for loved ones with HD has unique challenges, and finding a long-term-care community to serve their particular needs might also be more difficult than for someone with a different dementia.
Huntington’s Disease comes from a faulty gene within the genetic instructions we inherit from our parents (DNA) that determine how our cells develop and how our bodies grow and function. The gene defect affects nerve tissues in the brain and spinal cord that control thinking and movement.
The Huntington gene is rare, with about 41,000 cases in America. Unfortunately, every naturally-conceived child of a parent with this faulty gene has a 50% chance of inheriting it. Everyone who inherits the gene will eventually develop symptoms. Carrying the gene is the only risk factor for Huntington’s disease, which affects all races and genders.
Genetic testing can determine if a person has the faulty gene. Someone who tests positive can live without symptoms for years (HD usually becomes a problem between ages 30 and 50).
These are the symptoms of Huntington’s disease:
– Changes in personality, moodiness, depression
– Social withdrawal
– Difficulty sleeping despite fatigue
– Suicidal thoughts
– Loss of focus
– Loss of impulse control
– Impaired judgement
– Difficulty walking
– Involuntary movements known as “chorea”
– Muscle stiffness or contractions known as “dystonia”
– Slow eye movement
– Slurred speech
– Difficulty swallowing
– Significant weight loss
These symptoms typically appear between the ages of 30 and 50. The first symptoms that develop are memory lapses and difficulty concentrating. Someone should see the doctor if experiencing a dramatic change in the ability to move or think. People with a family history of HD are highly at risk, but the same symptoms could signal a different serious health condition so diagnosis should be sought as soon as possible.
Diagnosing Huntington’s requires physical and neurological tests. An examination of blood determines whether someone carries the deformed gene that causes Huntington’s, and a computed tomography (CT) scan shows how much damage has occured to cells in the brain. Other tests may also be necessary.
Huntington’s disease is fatal. Someone diagnosed with HD typically lives for between 10 and 20 more years. The cause of death is usually one of these issues:
– Pneumonia or similar infection
– Injuries from a fall
– Problems related to the inability to swallow
Alzheimer’s disease is the most common form of dementia, accounting for between 60 and 80 percent of dementia cases, which translates to about 5.5 million Americans. Huntington’s is much rarer, affecting about 30,000 people in the U.S.
Inheritance is one of the risk factors for developing Alzheimer’s, but a person whose parents or grandparents had Alzheimer’s is only slightly more likely to get the disease than someone with no family history. Alzheimer’s has also been linked to head injuries, cardiovascular disease, and a sedentary lifestyle. Huntington’s, on the other hand, is directly inherited from parents, with no other causal factors.
Huntington’s disease also begins to show symptoms much earlier. A person as young as 30 can begin to show the effects of the disease, while Alzheimer’s overwhelmingly impacts people over 65. And while someone with Alzheimer’s typically begins having difficulty remembering events and recognizing faces in the middle stages of the illness, someone with Huntington’s can usually recognize people and places until very late in life. Huntington’s has a greater impact on someone’s planning, organizational skills, and mood than on memory. The loss of bodily control in a person with Huntington’s comes on sooner as well, usually as involuntary jerks and movements called “chorea” that are similar to the physical symptoms of Parkinson’s disease.
No treatment can cure or even change the course of Huntington’s disease. People with HD can alleviate their physical and mental symptoms, however, with medications and interventions including psychotherapy and physical therapy.
The only drug approved specifically for Huntington’s disease symptoms is Xenazine (generic name tetrabenazine), which suppresses the involuntary jerks and movements known as “chorea.” It is important for someone on Xenazine to mind the more powerful side effects that can impact mood and worsen depression.
Other drugs that might help with symptoms include antipsychotics like risperidone, but these also have strong side effects and should be taken only after careful consideration. Antidepressants and mood stabilizers similarly might help but could worsen other symptoms. Studies show that how a person responds to medication is so individualized that they should only be taken after careful consideration with doctors.
Psychotherapy, or talk therapy, can help someone cope with the slow progression of Huntington’s disease. A psychotherapist works to help patients manage their expectations and communicate with colleagues, friends and family about the effects of the disease.
Exercise has been demonstrated as beneficial for maintaining physical and mental strength as long as possible. Of course, exercise with diminished bodily control can be difficult, but a physical therapist can help someone with Huntington’s remain mobile for as long as possible and minimize the risk of falls that can result in injury or even death.
A speech therapist can help someone keep control of the muscles they need to talk, chew, and swallow. Even after the ability to talk is diminished, a speech therapist can teach someone with advanced Huntington’s how to communicate using signs and signals.
Mobility helpers like canes, walkers, rollators and wheelchairs can be useful for moving from room to room and are also important to prevent injury as the physical symptoms of Huntington’s disease become overwhelming. Remember that a doctor’s consultation is crucial to determine exactly which devices are best for your loved one.
Aids to help around the house for people with Huntington’s include special utensils for eating, key turners, no-spill cups, gripping lid openers, and other devices that are similar to those designed for people with Parkinson’s disease.
Because people with a family history of Huntington’s have a high probability of passing the defective gene onto their children, parents or potential parents can be understandably worried.
Genetic testing can determine if someone is at risk of passing down the disease. Genetic counselors consult with couples deciding whether to have children about options like in vitro fertilization, where eggs are fertilized outside the body. The resulting embryos are tested for the gene defect, and if the tests are clear the embryo is implanted into the mother’s uterus.
Taking care of a loved one with Huntington’s disease is different in important ways from caregiving for someone with Alzheimer’s disease. For instance, a person with Huntington’s is aware until almost the very end that they’re afflicted with this illness and that it will only get worse. This is heartbreaking; pride and self-esteem are greatly impacted when a person knows what words to use but can’t make their mouths work, or tries to walk and falls down. Compassion and empathy, therefore, are essential. Someone with this disease will remain at the same intellectual level for much longer than someone with Alzheimer’s or another dementia, even as movement and psychological and behavioral problems worsen. Therefore, caregivers must learn to see past moodiness and depression to interpret their loved one’s actions as a request to fill some need. Compulsions, paranoia, and other psychological problems can overwhelm someone with Huntington’s who needs help with several activities throughout the day because their bodies are breaking down. As caregiver, your first rule is to remain compassionate. Remember it’s the disease that makes someone act angry or aggressive, not a choice. Stay positive.
Also remember to take care of yourself. Caregiver stress is a significant problem because the person being cared for will pick up on your mood. Be sure to get enough sleep and practice principles of self-care so that you’re at your best when dealing with dementia-related behavior.
Practical tips for caregivers include:
– Look into aids similar to options for people with Parkinson’s, including special pens, weighted utensils, key turners, no-spill cups, canes, and more that will help maintain some independence as symptoms worsen.
– Any physical exercise, even something as simple as standing and sitting regularly, is a good idea. People in better shape have less severe symptoms.
– Serve softer food, like blended nutritious smoothies, as swallowing becomes difficult. Avoid food with crumbs that might get caught in the throat.
– Diet should include plenty of proteins and carbohydrates.
– Inspect the home for safety issues, and install grab bars and handrails in the bathroom and wherever else they might be necessary. People with Huntington’s become prone to falls, so minimize furniture and remove rugs that might be tripping hazards.
– Your loved one’s paranoia or aggression could cause them to act out, so be prepared. Locks with keyless entry will allow you to re-enter your home if your loved one locks you out.
It may become necessary at some point for a person with Huntington’s disease to move out of the house and into a long-term care residence. This is a process you, as caregiver, should begin as early as possible.
If your loved one’s needs become too much for one caregiver to handle, assisted living or memory care may be an option. Memory care staff is specially trained to assist people with dementia who need help with activities of daily living like eating and dressing. Assisted living, on the other hand, encourages more independence, and staff are not necessarily educated in the specifics of communication and activities for people with physical and cognitive difficulties. For this reason, memory care is probably the better option when Huntington’s symptoms become too much for a person to stay at home. Memory care also offers additional security, with more supervision and special locks on doors.
Finding one of these communities for a person with Huntington’s is harder than for someone who has Alzheimer’s disease. Huntington’s is rarer and symptoms are different. No assisted living or memory care communities may take in someone whose needs they cannot adequately fulfill, so more work is probably necessary to find a home with staff specifically trained to help people with Huntington’s.
There are resources available to find an assisted living or memory care home that can accommodate someone with Huntington’s, including a service provided by a partner of our organization. Get free assistance here.
If your loved one has lived at home until the advanced stage of Huntington’s, when safety is more of an issue, a nursing home (also called a “skilled nursing facility”) is probably the best option. Assisted living and memory care are generally not considered as appropriate for people with advanced Huntington’s because by the time they need to move out of their houses, their needs are so great that 24-hour nursing care is necessary. Someone in the late stages needs help swallowing, for instance, and might not be able to go to the bathroom without assistance. Assisted living is for people who are more independent than someone with late-stage Huntington’s, and memory care is usually for people with dementia that more closely resembles Alzheimer’s. Memory care staff is specially trained to help mostly elderly people with communication and activities that aren’t as applicable for someone with Huntington’s who understands but whose mood and body are breaking down.
If you think a nursing home will become necessary, it’s important to start looking well before the move is necessary. Finding a home for someone with Huntington’s is harder than with other dementias. People with Huntington’s are usually younger, and symptoms are somewhat different than Alzheimer’s or other more prevalent dementias. You need to find a community with staffers whose skills match the needs of your loved one.