The Kasner family has two young boys diagnosed with Duchenne Muscular Dystrophy. Friend and neighbor Adam Klotz is hosting the KKD Gravel Ride which is intended to raise awareness and money for research to fight this terrible disease for the Kasner boys and others who battle Duchenne, a rare genetic and eventually fatal disease.
The inaugural KKD Gravel event takes place on Sat June 11, 2022 at the Dakota County Fairgrounds. The event has 3 distances: the Gravel Half 79er, The Gravel 79 Challenge, and the Gravel 158 Ultra. The first 100 registered will be entered into a drawing for a brand new Wahoo Kickr (V5). To learn more, visit the KKD Gravel Facebook event, the Registration Page, or the Kasners family page here.
Interview with friend and neighbor to the Kasner family, Adam Klotz:
Who are the Kasners and what is Duchenne Muscular Dystrophy?
The Kasner Family is made up of 4 crazy boys, AJ, Ben, Caleb and Dunky (ABCD) and their parents, Sarah and Dan. They reside in South Minneapolis where they love their neighborhood and thoroughly enjoy the summers where they walk, run or bike wherever they go. AJ and Ben are college-aged and living their best life.
Caleb and Dunky are 8 and 6 and are living a life different than any of us ever dreamed of.
Nearly 4 years ago, these boys were diagnosed with Duchenne Muscular Dystrophy (Duchenne). Duchenne is a progressive, neuromuscular disease that affects primarily boys and is terminal as there is no treatment or cure.
The disease happens because these boys are missing dystrophin, which is a necessary protein found in the muscles. With each muscle movement, muscles breakdown and build back up, but Caleb and Dunky's never build back up.
So essentially they are losing the ability to move their bodies with each movement they make. Kids with Duchenne are usually wheelchair bound by 8 - 12 and have a life expectancy around early 20's.
In addition to their physically failing bodies, there are also behavioral issues that come with Duchenne, which are exacerbated due to the daily steroids they have to take.
You're a friend of theirs. Why go through all this work to raise awareness and funds when it's not even your own family?
Fortunately, I don’t consider it “work” because I very much enjoy all things bikes, riding in organized bike rides, and being in charge.
Adding to that is the pure joy I get out of doing something for my neighbors the Kasners.
Dan and Sarah have been neighbors and friends of ours for something like ten years. My wife and I have two children who are roughly the same ages of Caleb and Dunky, and our kids have grown up with one another.
Seeing the impact of Duchenne on them made me want to do something to show my support and maybe even help.
Biking and the biking community has been very helpful for me in my life, and so an annual biking event to raise awareness and funds made sense.
Tell me about Dakota County. What are the gravel roads and scenery like?
KKD Gravel is the gravel roads that everyone grew up with.
KKD Gravel starts and finishes in Dakota County, and it travels into Rice, Le Sueur, and Scott
Counties as well, depending on the distance ride you are doing.
I chose that location because I grew up in Dakota County (minutes from the starting line), and so growing up I knew the surrounding gravel roads well. And when I now ride these gravel roads with my wife, who is also an avid biker, she describes them as the type of gravel roads that she also knew growing up, despite her being from the Duluth area.
So we’ve concluded the best way to describe the gravel roads we ride for KKD Gravel, is to describe them as the familiar gravel roads that everyone grew up with—relatively flat, uninterrupted skylines, corn and soy fields, spattering of cows, small townships here and there with their clusters of small surrounding homes, farmhouses, scents of manure and fertilizer, large irrigation systems, suburban outskirts, and various paved connecting thoroughfares.
KKD Gravel is not a gravel ride in the harrowing driftless region, nor one that takes place in a densely wooded logging region, like in Cable, WI.
While I love riding events in those regions, KKD Gravel is different, and I’m particularly fond of it for that reason.
A couple last things: The gravel is fast, which is great because in my opinion it makes rides like
these more accessible to those who are new or relatively new to riding gravel.
And for those veterans of the discipline, it allows for you to perhaps complete your first Ultra, or ride 79 or 158 miles and then weed and mow your lawn later that day. For the 158 Ultra riders, you’ll also get lakes for your scenery, and a surprise or two for the course medium.
What's the significance of 79?
The distances offered for KKD Gravel are 39.5 miles (or the Half 79er), a 79 mile Challenge, and a 158 mile Ultra. I’m also considering adding a 17.9 miler. The distances are based on the number 79, which is significant in the Duchenne community.
79 represents the 79 exons in the dystrophin gene.
The dystrophin gene is the largest gene in the entire human body, which makes any treatments extremely difficult.
Every child with Duchenne has a mutation in the dystrophin gene.
There can be duplication of exons, deletion of exons or nonsense mutations where the gene just stops. Caleb and Dunky are missing exons 6 - 17, which is considered a rare mutation of the disease.
Believe it or not, there are hot spots of this rare disease and Dunky and Caleb do not fall in the hot spot, making their mutation even more rare. We target 79 because our goal is someday they can have a treatment that would help them produce full length dystrophin, the 79 exons that they need.
It's hard to believe how lucky most people are to just be able to jump on their bikes and go for a ride. Just one exon missing from one of our many genes we have can result in such a terrible disease.