Connect with our region:
By Matthew Santamaria (msantamaria@hdsa.org)

When Minnesota resident Amanda Fiege was younger, her family would visit her grandmother and grandfather in Shorewood, Illinois. She remembers her grandfather always sitting in his chair or a wheelchair. The adults did not talk about what was wrong with her grandfather with the children around.

“He often would fall if he tried to walk unaccompanied,” Amanda explains. “My grandma would help him to the bathroom and help feed him at meal time. He could never have a conversation with me or anyone else. I just thought my grandpa was old and needed help.”

However, her grandfather’s deteriorating health was not because of old age. He was diagnosed with the gene that causes Huntington’s disease (HD). This is a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person’s physical and mental abilities usually during their prime working years and has no cure.

Her grandfather had four children and all of them were at risk of inheriting HD. Each child of a parent with HD has a 50/50 chance of inheriting the faulty gene that causes Huntington’s disease. Today, there are approximately 30,000 symptomatic Americans and more than 200,000 at-risk of inheriting the disease.

“I remember my dad explaining to me that my mother was sick and could not do the normal things a mother would do,” said Amanda. “I knew something was wrong with my mom. My mother did try to do as much as she could and fight the disease taking over her body. She did laundry, cook and clean. She would go on walks to keep her body moving. I didn’t like going out in public with her. I was that teenage girl who thought I would be made fun of for having a handicap mother.”

Amanda would see the symptoms take control of her mother’s life. The symptoms of Huntington’s disease are described as having ALS, Parkinson’s and Alzheimer’s – simultaneously. This includes personality changes, mood swings, depression, impaired judgement, involuntary movements, slurred speech, difficulty in swallowing, and significant weight loss.

“I was embarrassed when we would go to the mall or grocery shop because she would have the jerk movements and cause attention to us,” Amanda explains. “Being a teenager without a mother growing up was difficult. I only had my dad to guide me to grow up. We fought, we cried, and we smiled through the good and bad times.”

Once her father described her mother’s illness as HD for the first time. She relied on her grandmother as she has been through this with her grandfather. Her grandmother made Amanda realize that life is too short to worry about what could happen. Just live your life.

Though she had HD in her family, she realized that she was not alone.

“In 2001, my dad had a conversation with a friend’s mother during school conferences,” Amanda explains. “It was then I found out my best friend Britt’s family also had a history of HD. Once my dad told me, I was in shock! Britt and I had some good conversations and we both were there for each other. She was there for me even more since she knew I was having a hard time that my mother had gotten worse.”

That next year, her father told Amanda and her brother that their mother needed to be put in a group home and be taken care of twenty-four hours a day.

“I could tell my dad was worn out and depressed,” said Amanda. “He didn’t do much any longer, besides play on the computer, as he needs to be home for my mother all the time.”

In 2004, her mother was determined to watch her daughter graduate high school. However, she was physically unable to attend the graduation but attended the graduation party. That next year, on June 17th, she passed away due to complications from HD.

Amanda remembers that day like it was yesterday.

“I had been out with my friends and came home to my dad and brother sitting on the couch watching TV,” Amanda explains. “I sat down with them and we were just having that nice family moment when all of a sudden, my dad got a phone call. It was late in the night sometime after 9pm. He got up from his chair and walked into his bedroom. My brother and I sat there wondering if that was ‘the call.’ Well turns out it was ‘the call.’ My dad walked back in the living room and told us mom had passed away. We had a big family hug and cry session and got in the car to head where she was living at.”

She continues:

“My grandma had been on her way to come see us since my dad called her a few days before, recognizing my mom had took a turn for the worst,” said Amanda. “Grandma didn’t make it in time to see her daughter one last time. After many phone calls to family that evening, and seeing my mom once last time, we got home and I just couldn’t accept she was gone. I laid awake thinking of all the times with her, and how she will not be here anymore. I don’t think I slept much that night.”

That next day, as her family was arriving, Amanda was hiding in her room because it was too much to handle. This is when her grandmother showed up.

“I let her in my room, and just sat with her on my bed, crying, and telling her it wasn’t fair," said Amanda. "Having my grandma around for a little over a week was helpful. She made sure my family was keeping our chins up. I miss and think of my mother daily.”

Since 2013, Amanda has been involved with HDSA’s Team Hope Walks. The Team Hope Walk Program is HDSA’s signature grassroots fundraising campaign designed to provide hope and support for those touched by this devastating disease.

“Each year the walk keeps growing in size,” said Amanda. “It is amazing for the size of Duluth the amount of people who are affected somehow with Huntington’s Disease. I thought growing up it was just my family who had this horrible disease, in the area I grew up in.”

In 2017, Amanda got engaged and knew that she wanted to have a family one day with the man of her dreams. That next year, she decided to get tested and her father was with her the entire way.

The decision to get genetically tested is difficult for at-risk patients. Each year, 5-10% of at-risk patients are tested. It is never the right or wrong decision to be tested. There are people that see no benefit in knowing that they will develop the disease while others want to know in order to make informed choices about their future. It can take up to several weeks to receive your results from the genetic testing center.

“It was an intense discussion and that day I had my blood taken,” said Amanda. “Surprisingly I did not think much of it, and kept telling people who asked, that it is out of my hands now.”

On October 3rd, 2018, she received a call from her therapist.

“I was negative of the gene,” said Amanda. “I dropped to the floor and thanked her for calling me and giving me the greatest news! I was crying tears of joy! I called my dad, and the reaction he had was one of overwhelming joy! His prayers had been answered. I no longer have to have in the back of my mind the thoughts of having HD.”

###

Huntington’s disease is a fatal genetic disorder that causes the progressive breakdown of nerve cells in the brain. It deteriorates a person’s physical and mental abilities usually during their prime working years and has no cure. Each child of a parent with HD has a 50/50 chance of inheriting the faulty gene that causes Huntington’s disease. Today, there are approximately 30,000 symptomatic Americans and more than 200,000 at-risk of inheriting the disease. The symptoms of Huntington’s disease are described as having ALS, Parkinson’s and Alzheimer’s – simultaneously.

The Huntington’s Disease Society of America is the premier nonprofit organization dedicated to improving the lives of everyone affected by HD. From community services and education to advocacy and research, HDSA is the world’s leader in providing help for today and hope for tomorrow for people with HD and their families.

To learn more about Huntington’s disease and the work of the Huntington’s Disease Society of America, visit www.HDSA.org or call 1(800)345-HDSA.

This is a story featuring a personal experience with Huntington’s disease. If you would like to have your story told please contact Matthew Santamaria at msantamaria@hdsa.org