Managing Huntington's Disease in Senior Care

Recently, I have become very interested in the major neurodegenerative disorders affecting seniors in long term care. One of my concerns is the care that takes place as the disease advances and then at the end of life. I’ve known about Huntington’s disease for a long time, but wanted to see what’s new in research and literature about it and about the care that is required in the later stages. Here’s what I found.

Huntington’s Disease

Huntington’s disease is a slowly progressive, neurodegenerative disorder that involves degeneration of neurons in the brain that leads to numerous physical, behavioral, cognitive and emotional changes. It involves gradual changes in the person’s ability to control movements of the arms, legs, trunk, tongue and lips. Changes also take place in mental functioning, learning and memory. The individual will experience varying degrees of changes in emotional state or mood, and psychological and/or psychiatric disturbances.

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The disease is caused by a defect in a gene called “huntingtin”. Everyone has two copies of this gene but anyone who inherits the defects in one or both genes will develop Huntington’s disease. Dr. George Huntington was the first to describe the disease in 1872. At first, the disease was called Huntington’s Chorea and the main focus was on the movement problems. We now know this disease involves much more than problems with movement. Motor, cognitive, psychological and behavioral problems also are characteristic of the disease, which can last up to 15 to 20 years.

Although Huntington’s is a neurodegenerative disorder, just like Alzheimer’s and Parkinson’s, people are diagnosed much younger and have few medical problems, unlike the other neurocognitive disorders. As a matter of fact, the typical age at onset is between 30 to 40 years of age. And unlike the other neurodegeneratives, children are at high risk of developing Huntington’s through genetics, and are also vulnerable to depression and anxiety due to the possibility of carrying the genes. It is therefore important to treat the patient and entire family as one unit when it comes to this devastating disorder.

Symptoms of Huntington’s Disease

There are three sets of symptoms to monitor with Huntington’s disease. Those associated with movement disorder, cognitive disorder, and psychological and psychiatric effects. Because of these three sets of symptoms, Huntington’s tends to be a more challenging and complex disorder to treat. Movement disorder is the most obvious and involves loss of control of voluntary movements that lead to clumsiness, dropping objects, changes in handwriting slurred speech and gait abnormalities. Cognitive disorder begins as loss of executive functions and eventually ends in dementia. The psychological and psychiatric features of the disease can range from compulsiveness, irritability and depression to aggression, psychosis and bipolar symptoms. This is truly a multifactorial disease.

Special Care Issues in Late Stage Huntington’s Disease

One of the saddest aspects of the disease is placement into a skilled nursing facility at a younger age, generally between 30 to 70 years of age. For the most part, these people have nothing in common with elderly residents living in nursing homes and life can become frustrating and depressing. Most people will enter a care facility around 10 years after the symptoms begin. Care should be person-centered, and the family should receive attention too.

The individual can live for years in long term care and the staff must be able to effectively handle the changes that are going to take place with the advancing disease. Comfort, safety and autonomy should be at the top of the list. Pain will be an issue that requires attention as does anxiety and depression. Changes will continue to take place and the individual will deteriorate in motor and cognitive function as well as mood. The plan of care should address how well the individual tolerates activity, bowel function, pain, alterations in nutrition, urinary pattern, sensory perception, thought process, confinement to bed or chair, the use of restraints, death and dying issues and family dynamics, just to name a few.

Final Words on Managing Huntington’s Disease in Senior Care

As I travel and speak about many health care topics, this one really gets to me. It’s not that I personally know anyone with Huntington’s, but I know how devastating it can be to both the patient and family. It truly is one of the cruelest of the neurodegenerative disorders known to us. The individual will eventually require hospice services to address and deal with pain and symptom management, the dying process and death itself. Most people will die from a mix of aspiration pneumonia, malnutrition, falls or other trauma. What will become the most important aspects of care will be comfort, quality of life and dying a good death.