This week I wanted to take time and write about a rare disease that has very recently claimed the life of my grandfather. Most likely you've never heard of Huntington's Disease unless you know someone who has it or you're a doctor. Huntington's disease is an inherited disease that causes the progressive breakdown (degeneration) of nerve cells in the brain. Huntington's disease has a broad impact on a person's functional abilities and usually results in movement, thinking (cognitive) and psychiatric disorders.
Most people with Huntington's disease develop signs and symptoms in their 30s or 40s, but the onset of disease may be earlier or later in life. When this disease's onset begins before age 20, the condition is called juvenile Huntington's disease. Earlier onset often results in a somewhat different presentation of symptoms and faster disease progression.
Medications are available to help manage the symptoms of Huntington's disease, but treatment can't prevent the physical, mental and behavioral decline associated with the condition. This disease is not curable, and trust and believe me when I say it's not prejudice either.
There are multiple symptoms that aren't just limited to the three main issues that are Psychiatric, Cognitive and Movement.
Movement Disorders that is associated with Huntington's disease can include both involuntary movements and impairments in voluntary movements
- involuntary jerking or writhing movements
- muscle problems, rigidity or muscle contracture
- slow or abnormal eye movements
- impaired gait, posture and balance
- difficulty with the physical production of speech or swallowing
Cognitive impairments often associated with Huntington's disease are
- Difficulty organizing, prioritizing or focusing on tasks
- Lack of flexibility or the tendency to get stuck on a thought, behavior or action
- Lack of impulse control that can result in outbursts, acting without thinking and sexual promiscuity
- Lack of awareness of one's own behaviors and abilities
- Slowness in processing thoughts or ''finding'' words
- Difficulty in learning new information
Psychiatric disorder the most common one associated with Huntington's disease is depression. This isn't simply a reaction to receiving a diagnosis of Huntington's disease. Instead, depression appears to occur because of injury to the brain and subsequent changes in brain function. Signs and symptoms are
- Feelings of irritability, sadness or apathy
- Social withdrawal
- Insomnia
- Fatigue and loss of energy
- Frequent thoughts of death, dying or suicide
Other common psychiatric disorders include:
Obsessive-compulsive disorder, a condition marked by recurrent, intrusive thoughts and repetitive behaviors
Mania, which can cause elevated mood, overactivity, impulsive behavior and inflated self-esteem
Bipolar disorder, or alternating episodes of depression and mania
Weight loss is common in people with Huntington's disease, especially as the disease progresses.
My papaw has had this disease for as long as I can remember. It got so bad it got to the point where he couldn't take care of himself. He was in a nursing home for a few years. The late stages caused him to have a hard time swallowing because of this he started eating less due to his fear of severely choking. The only alternative to this was to have a feeding tube placed and he demanded to not have one. After years of fighting this disease, it just took a toll on his body and it slowly started shutting down.
Wednesday night I got a phone call from my dad which was odd because we don't talk much and he informed me that my papaw didn't have much time left. That night I planned to go see him right after I got off work. My sister had told me he was on morphine and was doing worse than we had thought and that I should probably leave work. As I was getting my things ready to leave at 11:46 am on January 19th, 2017 I received a text message from my sister telling me he had passed away. Seeing him the way I saw him that day I do not wish on my worst enemy to see a loved one that way.
Even though I will miss him a great deal, I know in my heart that his suffering is now over. It overwhelms me with sadness just thinking I will no longer see him here in the physical world, yet I also get a sense of peace when I think of him. Even though this horrific and cruel disease took over his and so many others lives I can't help but feel at ease and a sense of calmness just knowing his battle is over.