When Bryan was younger, he used to love playing baseball with his dad. Every day after school, Bryan’s dad pitched to him and helped him become a very good hitter.
Almost every time Bryan’s friends came over to play, his dad would go outside and play with them. He would throw passes to them when they were playing football and he would shoot baskets with them when they were playing basketball. All of Bryan’s friends liked Bryan’s dad because he was so much fun to play with!
One summer, Bryan’s dad started to act a little strangely. When he walked, his hips moved around almost like he was dancing. When they played baseball, he began to throw pitches that missed the plate a lot (he used to throw perfect strikes). When Bryan’s friends came over, his dad could not throw passes or shoot baskets as well as he had before. Soon, his dad stopped playing with them.
Bryan was sad and confused about why his dad was having so much trouble moving around. He became even more concerned when he noticed that his dad was treating his mom differently. Bryan’s dad had always been very nice, but now he began to start arguments with Bryan’s mom and he sometimes said mean things.
Soon, Bryan returned to school. One day when his dad came to take Bryan home, Bryan’s friend Lisa said, “Your dad walks funny!” This comment made Bryan sad and angry because nobody had ever said anything bad about his dad before. Bryan was not just upset at Lisa, but also at his dad for becoming so different.
On the way home, Bryan’s dad noticed that his son was upset. He asked Bryan what was wrong and Bryan replied, “What’s going on with you, Dad? You don’t play sports as well as you used to, you walk really weird, and you haven’t been very nice to Mom lately. Why are you being like this?”
Bryan’s dad had noticed these changes in himself, too, and he told Bryan that he did not know why he was acting this way. “Actually,” he said, “your mom and I are going to see the doctor today to find out what’s going on. You are welcome to come along if you want.” Bryan never liked going to the doctor’s office, but he decided to go with his parents this time because he really wanted to know why his dad was acting so strangely.
When they got to the doctor’s office, the doctor asked, “So what seems to be the problem?”
Before his dad could speak, Bryan’s mother said to the doctor, “Well, my husband has been acting strangely the past few months. His body seems to shake when he walks and he has also been kind of grouchy.”
Bryan added, “We used to play sports together all the time, but lately he hasn’t been throwing as well as he used to and he even stopped playing with me and my friends.” Bryan’s dad looked sad when he heard his family say these things, but he knew there was truth in what they said.
The doctor replied, “There are a lot of different things that can cause these kinds of problems, so I will need to find out some more information.” She went on to ask Bryan’s dad about many different things, including how old he was and if any of his family members ever had a disease.
Bryan’s dad replied, “Well, I am 35 years old and my mom passed away when I was 27 from something called Huntington’s disease.”
Then Bryan heard his dad say this, he asked the doctor, “What is Huntington’s disease?”
The doctor replied, “Well Bryan, Huntington’s disease is a disease that can cause a lot of changes in the way someone moves, thinks, and feels emotions. These changes start to show up usually when the person is between 30 and 50 years old. The disease is generally passed down through families. Just like when you flip a coin and you have a 50% chance of the coin landing on ‘tails,’ a parent who has Huntington’s disease has a 50% chance of passing it on to his or her child. Both men and women can get the disease and it occurs all around the world, although it is generally very rare.”
Bryan’s dad asked, “So how do these changes come about?”
The doctor replied, “Well, the brain is made up of millions of small living cells called nerve cells and these basically allow us to do everything we do, from talking and reading to walking and even throwing a baseball. In people with Huntington’s disease, many nerve cells are damaged in certain parts of their brain called the basal ganglia. Healthy nerve cells in these parts of the brain help with making the smooth movements that we normally make when we do things like walking or playing catch. So when these nerve cells are damaged, this can lead to a lot of problems with movement.”
Bryan asked, “What is wrong with the nerve cells? Why are they damaged?”
The doctor replied, “The problem actually exists down within the nerve cells in tiny, microscopic structures called genes. The word sounds the same as the jeans you wear on your legs, but these genes inside the cells work in a completely different way. They contain a special kind of information in the form of DNA. Have you heard about DNA?”
“Only a little, in the news and at school.” said Bryan. “I don’t really understand it. What does it do?”
“Well,” said the doctor, “DNA is chemical stuff in the body’s cells that carries information for sustaining life. Each cell in the body has DNA that contains many thousands of genes, but the problem in Huntington’s disease has to do with just one of these genes, one small piece of the DNA that is called the Huntington gene.”
“Like every other gene, the Huntington gene contains many small sections called codons, each made of DNA. You can think of it like a chain: the entire Huntington gene is like a long chain and each codon is like a different link in the chain. In people who do not get Huntington’s disease, there are 35 or fewer identical links in a key part of the chain. But in people who do get Huntington’s disease, there are 40 or more of these identical links in the key part. Huntington’s disease results from having too many identical codons in the key part of the Huntington gene.”
“So the chain is too long?” asked Bryan. “Does it take up too much space or something?”
The doctor replied, “Well, not exactly. The system works like this: each gene makes another chemical called a protein and proteins are the things that do much of the work in cells. The Huntington gene makes a protein that we call huntingtin. In people who do not get Huntington’s disease, the huntingtin protein comes from a version of the Huntington gene with 35 or fewer identical codons in the key part. This huntingtin protein is able to do its normal work in cells. But in people who do get Huntington’s disease, the huntingtin protein comes from a version of the Huntington gene with 40 or more identical codons in the key part. This causes the huntingtin protein to have a different shape than normal and because of this altered shape, the protein cannot do its normal job.”
“After enough time, huntingtin proteins with the altered shape form into clumps or aggregations down inside cells, and we think these clumps clog up cells. We also think that the huntingtin proteins in these clumps may grab onto other proteins and keep them from doing their jobs in cells. So, these altered huntingtin proteins create a big problem, especially for nerve cells in the basal ganglia regions of the brain.”
“Eventually, these altered huntingtin proteins cause enough damage that the nerve cells containing the clumps begin to die. When enough nerve cells die in the basal ganglia regions of the brain, people start to have problems doing the movements that they always did before. This can be followed by changes in the person’s personality. For instance, the person might become more sad at times or more grouchy.”
After the doctor finished, Bryan’s dad asked, “So do I definitely have this disease?”
The doctor replied, “I don’t know for sure because your attitude changes and problems with moving could be caused by something other than Huntington’s disease. But since you said that your mother had Huntington’s disease, this means that there is a 50% chance that you might have it as well, just like a flip of a coin.”
In order to see for sure if Bryan’s dad had Huntington’s disease or not, the doctor suggested that Bryan’s dad go to a genetic testing center where he could give some of his blood to have it tested for the disease. Bryan’s dad went the next week.
At the dinner table one night, Bryan’s dad told him that the results of the test had come back from the genetic testing center. He said, “Bryan, I don’t know how to tell you this, but the test that I took showed that I do have Huntington’s disease. That is the reason I have been acting so strangely.”
Bryan could tell that his parents were upset, but they were trying hard to be brave about this news. As his dad finished talking, Bryan saw that his mom was beginning to cry. Bryan was not used to seeing his mom cry. He was also scared to hear that his dad had Huntington’s disease. Bryan felt overwhelmed and this made him cry too.
Later that night when Bryan was going to bed, his parents came into his bedroom to tuck him in. Bryan’s dad said, “You know Bryan, this disease may cause a lot of changes in my behavior from now on. The doctor even says that these changes will get worse over time.”
Bryan’s mom added, “But even though some things your dad does might start to change, he will never stop loving you. We both love you very, very much and nothing about this disease is going to change that. There may be a lot of challenges for us in the future, but the most important thing is that we keep a positive attitude and keep loving each other.”
Although Bryan still felt a little sad, these words from his parents made him feel much better. “Keep a positive attitude and keep loving each other,” Bryan repeated to himself.
The next week at school, Bryan’s dad came to pick him up and Bryan’s friend Joe said, “Your dad walks funny!” This time Bryan knew the reason why his dad walked differently. He was no longer angry at his dad. He wasn’t even angry at Joe. He realized that it was not his dad’s fault for changing; it was the Huntington’s disease that was making his dad act differently from before. Bryan knew that it would be hard for his friends to understand why his dad was acting differently, because they did not know the things that he had learned about Huntington’s disease. Maybe someday he could teach them about the huntingtin protein and nerve cells, so that they would understand too. But for now, his main focus was to keep a positive attitude and not allow these comments to hurt him. He knew that he would be much happier this way.
“Yeah,” said Bryan, “My dad has a medical problem. But he’s a real sport about it, and I’m learning to be a sport about it too. We walk and run less these days, but we still have fun together doing new things.”
On the ride home, Bryan’s dad said, “Hey Bryan, I know I don’t throw the baseball as well as I used to, but I can sure catch fish. I was thinking that this weekend we could go down to lake and go fishing.”
“That sounds great, Dad!” Bryan said. He loved to go fishing, but he was even more excited by the chance to hang out with his dad.
Bryan and his dad were wonderful fishermen! They both reeled in six fish! As they were getting ready to go, Bryan’s dad said, “Wow Bryan, this has been a blast! Thank you so much for coming out here with me.”
Bryan quickly replied, “No Dad, thank you!”
Bryan realized that although his dad was changing and could no longer do some of the things he had done before, they could still do plenty of other fun things together. He knew that his dad’s Huntington’s disease would present tough problems in the future, but he loved his dad and he knew that his dad loved him. No matter what the future had in store, nothing could ever change that.
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-M. Stenerson & S. Fu, 3-09-04