Fifth annual ‘Emmy’s Rustic Run’ to be held Oct. 2

By Kim McDarison 

The fifth annual “Emmy’s Rustic Run,” a 5k trail run and day of family fun, will be held Saturday, Oct. 2, at the Fort Atkinson family farm of Russ and Angie Nathan, N580 Poeppel Road. 

Maternal grandparents of Emmalynn “Emmy” Liufau, the couple has been hosting the annual event in support of finding a cure for a rare neurodegenerative disease called ataxia-telangiectasia or A-T. Emmy is one of between 400 and 500 children nationwide living with the condition, according to her mom, Ashley Liufau. 

Ashley, a native of Fort Atkinson, and her husband, Dave are Fort Atkinson high school sweethearts who today live in Florida. The couple has been married for 12 years and has two children: Emmy, 8, and her sister, Kate, 6. The family returns each year to their hometown community to celebrate their family and work toward a cure for A-T. 

According to the National Institute of Neurological Disorders and Stroke (NINDS) website, A-T is a rare, childhood, neurological disorder that causes degeneration in the part of the brain that controls motor movements and speech.  

“NINDS-supported researchers discovered the gene responsible for A-T, known as ATM (ataxia-telangiectasia mutated) in 1995. This gene makes a protein that activates many (probably more than 700) other proteins that control cell cycle, DNA repair, and cell death. Without it, cells are unable to activate the cellular checkpoints that protect against the damage of ionizing radiation and other agents that can harm DNA,” the website states. 

The disease causes a progressive loss of muscle control, immune system complications, and a high rate of cancer, which presents mainly as blood cancers such as leukemia and lymphoma, Ashley said.  

Children with A-T appear normal at birth, with early signs of the disease usually appearing during the second year of life. A degenerative disease, children with A-T typically find themselves dependent on wheelchairs by the age of 10, and find it difficult to read, speak and eat, Ashley added.  

“Emmy struggles with basic tasks like walking, getting dressed, using silverware and writing utensils, getting up and down stairs, and even sitting upright for long periods of time. Because A-T attacks the entire body, everything requiring the use of muscle is affected. From eye tracking to swallowing, standing up to sitting still; Emmy has to consciously put forth effort to control her body in ways that the average person rarely thinks twice about,” Ashley continued. 

Through it all, Ashley said, Emmy is “a kind and gentle spirit.” Even as she faces challenges, Emmy remains a “source of joy to everyone she meets.

“Her smile and laugh light up a room and she has helped us learn to appreciate the quieter, slower moments in life.”  

Like any third-grader, Emmy said high on her list of things she enjoys are watching “My Little Pony,” drawing, swimming, playing with her toys and visiting Disney World. 

At school, she said, reading is her favorite subject and she’s not a fan of subtraction. 

When people notice she is different, she said, “I tell them I am a little wobbly and I have A-T.” 

When visiting Fort Atkinson and participating in Emmy’s Rustic Run, she said, she looks forward to playing in the bounce houses and painting pumpkins. 

Ashley said the family was made aware of Emmy’s condition even before she was symptomatic. 

“Her newborn screening came back with a red flag on her white blood cell count. The doctors couldn’t figure out what was causing her immunodeficiency, but we saw a geneticist when she was 6 months old and they ran a whole genome sequencing on her and it come back with her diagnosis of A-T.  

“We began really noticing her differences when she was about 3 or 4 years old,” Ashley recalled, noting that kids with A-T usually begin walking at a normal age, but often never leave the “toddler” stage. They sway and show signs of issues with coordination, she said.  

In Emmy’s case, Ashley said, she was able to walk on her own most of her life, but the family has seen a large decline in her abilities in the last 18 months. 

In addition to issues with muscle control, 70% of all A-T children are immunodeficient. Emmy receives a monthly IV infusion to help give her immune system a boost, Ashley said. 

Emmy is aware her body is changing, Ashley noted, describing her daughter as “a trooper.” 

“She has always done her best in everything and rarely complains about needing assistance or not being able to do things. This past year has been the hardest, though, as she is beginning to realize how different her body truly is compared to others her age. She relies heavily on the assistance of those around her to eat, walk, get dressed, (and) complete school assignments,” Ashley said. 

“Since Emmy’s condition is progressive,” Ashley continued, “we take things one day at a time. Her progression has been fairly slow over the past 8 years and it hasn’t been until the last year or so that we’ve really had to make some changes in our lives. It takes Emmy a little longer to do things and we generally need to help her. Now that Kate is getting a little older, she is able to assist Emmy in some ways, like running to grab a toy or book or helping her get a snack from the pantry.”  

Looking ahead towards a cure, Ashley said, the “antisense oligonucleotide” or ASO treatment — a gene therapy approach, being developed to produce the functional ATM protein that is missing within the cells of children with A-T — has completed a first-phase of trials with no side affects or complications. 

A single child is currently receiving the developing treatment, Ashley said. 

The child will next enter into a second phase, called the “loading phase,” Ashley stated, at which time drug doses will be made high enough to correct the gene mutation. The hope is that the child’s body will begin to produce the ATM protein it currently lacks. 

According to Ashley, “the drug is tailor-made for each patient and is therefore very expensive to produce. While it will not be able to reverse the effects Emmy is already experiencing, it could stop the progression of the disease in its tracks.” 

While Emmy is not aware of the developing treatment, she is aware of the fundraising component of Emmy’s Rustic Run. She understands that money earned through the event will fund research to help arrest the disease, her mother said.  

As a family, Ashley said: “We have faith in God’s plan for her and our family and have decided that we will live each day to its fullest. We also realize that the only way for a cure or life-improving treatments to be found is through fundraising efforts like ours. It is such a small percentage of the world’s population that is affected by A-T. The research that is happening is almost entirely thanks to the grassroots efforts of families like ours — these small-town fundraisers are making a big difference.”  

Describing the importance of Emmy’s Rustic Run, Ashley said: “Our event is incredibly important to those who are affected by A-T. Because of its rarity, there is little to no federal funding for research on ataxia-telangiectasia and so it’s up to us and other A-T families to do what we can to assist the A-T Children’s Project in funding these projects. The money raised also supports the A-T Clinical Center at Johns Hopkins in Baltimore. 

“We also love hosting our event because it’s something our kids look forward to every year. ‘Emmy’s Run,’ as my girls call it, is a break from the normal routine and it’s a fun-filled day where Emmy gets to ‘be famous’ as she likes to say.  We have been touched by the outpouring of love from the community — both family and friends, and complete strangers that have stepped up to help us spread the word, find donations and raffle prizes, and get involved.” 

Said Ashley: “It’s nearly impossible to describe how we feel about the Fort Atkinson community.” Although Emmy’s Rustic Run is in its fifth year, Ashley noted, “this is our seventh year hosting a fundraiser, and each and every year, we continue to be blown away by the support we receive. It is incredible to see what one small community can do when it rallies together. Time after time we are caught off-guard when we receive phone calls and emails from people who don’t know us, but want to help. They see our posts on Facebook or hear about what we’re doing from a friend and they want to get involved however they can.  It’s touching and often overwhelming for our family to feel that sort of support and we are humbled by it over and over again.”

Ashley said the event features “delicious food spreads” and free kids activities, like bounce houses and a scavenger hunt in the woods. There is also a silent auction and “dozens of door prize giveaways.” 

“We strive every year to bring something new and exciting for all of our participants. We love hosting this event and hope that it will continue to be something the Fort Atkinson community can look forward to each fall,” she said. 

As the family works toward a cure, Ashley said, “we just want people to understand that kids like Emmy are fighting a really big battle in life. A-T is a devastating and relentless disease that, at the moment, does not have a cure, but we’re so close we can almost taste it. The hardest part is finding the funds necessary to continue research and to support these trials so that we can find answers and figure out what will give Emmy and these other kids a better shot at a long and healthy future. 

“The best part about our event is that our participants get a lot out of it, too. The trail is handcrafted by my dad and it is truly a labor of love. The 5k run and one-mile walking courses have beautiful scenery changes and lots of surprises along the way. The food is always hot and delicious, the raffle baskets and silent auction items are fantastic, and if your kids don’t leave completely content and exhausted, you just didn’t stay long enough.”  

This year’s plans for Emmy’s Rustic Run include: 

• Event registration/check-in, 8 to 8:45 a.m.

• Pancake breakfast, 9 to 11 a.m.

• 5k trail run kickoff, 9 a.m.

• 5k awards, 9:50 a.m.

• Kids races, 10 a.m.

• Approximately 1-mile walk in the woods and scavenger hunt, 10:30 a.m.

• Raffle and silent auction winners announced at noon. 

• Face painting, bounce houses, kids crafts, concessions, 9 a.m. to 1 p.m. 

Those interested in learning more about the event can visit Facebook @EmmysRusticRun.  Those looking to register for the run or walk can visit: www.ATCP.org/RusticRun. For further information, contact the organizers: EmmysRusticRun@gmail.com.