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By Matthew Santamaria (msantamaria@hdsa.org)

In Watertown, South Dakota, Jody Niemann Priest has a brave and strong son. His name is Wyatt Niemann. He is only sixteen-years-old and has a smile that will turn your day around.

Wyatt does not let a disease take control of his life.

Wyatt was diagnosed with Juvenile Huntington’s disease (JHD). 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. Compared to adult onset Huntington’s disease (HD), JHD has a more rapid progression rate that adult onset HD. JHD affects teenagers and children.

At the age of five-years-old, his father passed away due to complications from 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.

Wyatt has developed symptoms of JHD. The symptoms of Huntington’s disease are described as having ALS, Parkinson’s and Alzheimer’s – simultaneously. Symptoms of JHD include stiffness of the legs, clumsiness of arms and legs, decline in cognitive function, changes in behavior, seizures, and changes in oral motor function, chorea in an adolescent, and behavioral disturbances.

“Since he was diagnosed, Wyatt has had a progressive loss of coordination,” Jody explains. “He frequently loses his balance and has had numerous falls. His ambulation now is mostly assisted with a gait belt and uses a wheelchair for longer distances. He needs to be fed due to inability to hold onto silverware and is no longer able to write or keyboard. He has a full time para-professional with him at school and attends regular high school.”

According to Jody, her son stands out amongst the crowd because nobody in the school system is diagnosed with JHD or HD which results in limited resources for him.

However, in school, Wyatt is one of the popular kids. According to Jody, she is amazed with how the kids go out of their way to help Wyatt. He was on the honor roll at his school.

“He is in the band and plays the tambourine,” said Jody. “Despite all Wyatt's physical limitations and his difficulty with speech (very slurred, difficulty with articulation), Wyatt has an active life and chooses to join many activities and participate to the best of his abilities. I like to say that Wyatt has JHD but he's so much more that!”

Wyatt is in the Boy Scouts. He is currently at Life Scout rank and is working his way towards Eagle Scout. From first to eighth grade, he has been a part of Destination Imagination, volunteer-led educational non-profit organization that teaches 21st century skills and STEM principles by collaborative problem solving challenges to kindergarten through university level students.

With Destination Imagination, he traveled last year to Tennessee for the Global Tournament. This is the culmination event of every Destination Imagination season that consists of more than 17,000 people attending the event.

“He participates in the Special Olympics in swimming and bowling where he has earned first place ribbons at Regional Tournaments and earned three gold medals and a silver medal at the recent State Special Olympics Equestrian Tournament,” said Jody.

Wyatt and his family are active in the HD Community. Each year, they attend the Sioux Falls, South Dakota Team Hope Walk. 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. Since their inception in 2007, it has occurred in more than 100 different cities. His family also attends support group.

“He has worked with the University of South Dakota first year medical students in learning about neurological disorders and JHD symptoms undergoing physical assessments from the students and acing the memory portion of the Mini-Mental State Examination,” said Jody.

Wyatt continues to enjoy the little things in life.

“He loves all animals, has visited numerous zoos and aquariums and gives love to our two dogs, cat, turtles and fish.”

Jody and Wyatt have a message for the HD Community:

“Despite the challenges of JHD, which we can't change, we choose to be positive and enjoy life and get the most out of each day. The participation has changed as Wyatt's condition changes but his willing attitude and contagious smile win over this disease.”

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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