What Is Huntington’s Disease And How Is It Defined?
By Nadia Khan
Updated February 04, 2020
Reviewer Natalie Feinblatt
Some of the most terrifying diseases are those that affect the brain. The brain is what makes us, and if there is any damage or changes to the brain, the effects can be life-changing.
Today, we'll be looking at one such disease, Huntington's disease.
What Is Huntington's Disease?
The basic definition of Huntington's disease is when your nerve cells start to break down. They degenerate slowly, and this, as you may expect, vastly affects their life. It can affect the way you move, the way your brain functions, and it may cause mental illnesses.
Huntington's disease usually develops through age 30-40s, but with any disease, it may come later or earlier. Juvenile Huntington's Disease, where a person develops it before 20, is possible. It will progress faster, and it's terrifying for a young person to experience.
Huntington's disease is inherited. If one of your parents has Huntington's Disease, you may wonder if you're going to get it as well. A mutated gene causes the disease, and the person needs just one of those genes to have Huntington's. The chances of a child developing Huntington's disease are a flip of the coin in most cases. There is usually a 50% chance.
Look into your family history. Having prior knowledge about Huntington's disease in your family can help you if you have inherited it.
Huntington's disease is, luckily, rare. If you're reading this article out of sheer curiosity, and not because you or someone you know has the disease, we can safely say that you probably do not suffer from it. However, despite its rarity, the symptoms and the nature of the disease is enough for people to want to see a cure. Even if you don't know anyone who has it, the symptoms will be enough for you to want to reach out for a cure.
Since it's a disorder in the brain, it's going to affect your brain in different ways than it would another person. You may experience different symptoms in the beginning, and these symptoms can differ in their effects or their appearances. There are three ways Hunting's disease affects the brain: movement, cognition, and psychiatric.
We all experience involuntary movements from time to time, but those who have Huntington's disease may have chronic involuntary movements. These can include a sudden jerk. You may find yourself writhing. Your muscles may contract, or feel rigid. Your eyes may twitch or move slowly.
You can also have trouble balancing, swallowing, or speaking. Some people may have minor episodes, while others may be affected more greatly. You may find yourself having some difficulties with your job, being able to do activities around the house, talk to people, and just support yourself or your family.
You can experience impairments in your cognition if you have Huntington's disease. What do we mean by this? Here are a few examples.
- You may find it difficult to focus on certain tasks. Everyone has their days when they can't focus, but someone with Huntington's disease will have more difficulties as they degenerate.
- You may find yourself freezing mentally or being stuck in thought. You may space out.
- Impulse control is lacking in some who have Huntington's. They may have outbursts, be promiscuous, and do something before thinking.
- A person's self-awareness may decrease.
- You may find it harder to formulate new thoughts. You may also find it hard to retain new knowledge.
Huntington's disease may cause some psychiatric disorders as well. Depression is perhaps the highest one on the list. Some may think a person with Huntington's is depressed because they have the disease. However, the disease can also change the chemistry of the brain, causing depression. They may feel sad, feel withdrawn from the world, have fatigue, and they may have suicidal thoughts.
Other mental disorders that can arise include OCD, mania, and bipolar disorder. As you can see, feeling manic, or having an elevated mood to the extreme, is a common theme.
Another physical change is unexpected weight loss.
Juvenile Huntington's Disease
As mentioned before, Huntington's disease happens mostly during later adulthood, but it can happen before adulthood too. Those who have juvenile Huntington's disease may have behavioral problems, have trouble in school, and forget information they've learned.
This makes it difficult to diagnose. Some of these behaviors may be seen as common changes for someone in their teens.
Also, they may have muscle contractions, lose their motor skills, have tremors, or possibly have seizures as well.
If your child has any of these symptoms, it won't hurt to take them to see a doctor.
Huntington's disease is when your brain cells gradually deteriorate, so the disease is going to grow worse as time progresses. If one is diagnosed with Huntington's disease, they may wonder how long they have until death. This will depend. Sometimes, they may have ten years to live, and other times, they may have 30. The odds of a faster death increase if you have juvenile Huntington's disease.
Some may not die due to the deterioration itself, but instead by other factors. Suicide is one of the big reasons for a person with Huntington's disease may pass away. Some cannot fathom living in a deteriorated state, and they want to die before they are that way. The risk of suicide does spike when a person is in the middle stages, where their independence drops. Other times, a person may try to commit suicide before they are even diagnosed.
Sometimes, a person with Huntington's disease may die from infections such as pneumonia. Other times, their loss in balance causes them to fall, and they die from falling. Their difficulty swallowing may cause death by choking.
A person with Huntington's disease will need help living and performing their daily tasks. Later in life, those with Huntington's may not be able to speak and may be bedridden, but their ability to understand people may still be intact.
With most of these diseases, there is never a cure. Instead, there are ways to manage its symptoms. These include:
Medication exists to help treat many of the symptoms of Huntington's. For example, if you're depressed, antidepressants can help you. There is also medication that can help with movement. If you or someone you know has Huntington's disease, medication therapy can help those manage the symptoms. There have been some promising breakthroughs that may help slow the disease even more, but a pill that stops Huntington's completely is not on the horizon anytime soon.
Going through physical therapy can help those who are losing movement and motor skills. By having regular physical therapy sessions, it keeps your balance in check, controls your swallowing, and prevents abnormal movements. Of course, as the disease progresses, therapy can be more difficult. However, by having therapy constantly, it can slow down the physical symptoms of Huntington's and let the person live a longer, healthier life.
Huntington's Disease And Parenting
If you or someone you know has a history of Huntington's disease and wants to start a family, they may wonder if they should. Passing on a horrifying disease such as Huntington's to your child is not something you want. Some may look to adoption or look into genetic testing.
One option is genetic counseling. What is a genetic counselor? It's someone who will talk to the potential parent if they are at risk of passing Huntington's disease, and explain the chances of passing it to their child. Some parents may not want to spread Huntington's, and instead adopt, have in-vitro fertilization, or other alternative methods of having children.
If a parent wants a child that's genetically theirs but doesn't want Huntington disease, there is still hope for these individuals. It's known as preimplantation genetic diagnosis. In a lab, the parents' sperm and egg are fertilized, and as the embryos grow, they are tested for the Huntington gene. If the embryo does not have the gene, it will be put into the uterus of the mother.
It's an expensive and complicated way of preventing Huntington's disease, but you can see why many parents would rather do that than have a child who will have to go through Huntington's. For some, it also raises some ethical concerns, such as creating the 'perfect' baby who has no diseases or bad genes. However, it can be a lifesaver for parents who want to avoid passing it on to their children.
Huntington's disease affects those who suffer from it greatly. It also hurts those who have loved ones who will deteriorate as time goes on. While there is no cure, education, and a desire to have children who do not have the gene, are some effective ways to combat it. Perhaps someday, there will be no Huntington's disease.