For so many in the rare disease community, courage isn’t a just a virtue – it’s a necessity.
Many have to overcome odds and obstacles that most others don’t have to; from the seemingly mundane to the extraordinary, disease-specific challenges, those who fight diseases and disorders have an extra bit of grit and grace in their spine.
Jo Klein and her husband Mike are proof of this extra bit of determination. Not only have they had to lay to rest their child Maximillian (Max for short), their son Charles was diagnosed with Batten disease.
Batten disease (also known as neuronal ceroid lipofuscinosis) is a fatal, inherited nervous system disorder that typically develops in childhood. It is linked to a buildup of substances called lipopigments in body tissues. Lipopigments are made up of fats and proteins. As these lipopigments accumulate, they kill neurons in the brain, retina and central nervous system. To learn more about Batten disease, click here.
Common symptoms include seizures, visual impairment/blindness, personality and behavior changes, and more.
Charles (full name Charleston – which is an objectively awesome name!) was first suspected having issues during preschool, when he had trouble seeing.
“He started squinting in class, and he would have issues seeing moving targets,” recalled Jo.
About a year later, Charles’s doctor noticed a spot in his eye, and requested digital retinal imaging of the back of his eye. In 2016, he had a working diagnosis of cone rod dystrophy, and in 2017 was finally diagnosed with Batten.
Jo and Mike recall the multitude of emotions from that diagnosis –
“Shock, disbelief, fear and anger – but at the same time we felt some small flicker of Hope. We let all of those feelings settle and then made a choice to put Hope in the forefront to fight for Charles and all the other children with Batten disease.”
Part of that hope was the determination to let Charles live the life of any 8 year-old, warts and all!
“Charles has a thankful heart. He is taking the loss of vision well. He has a positive attitude and is extremely motivated. He also can be sensitive at times and wants his voice to be heard; when we are having any conversation he gets upset when he is interrupted.”
“Charles also tends to have more behavioral problems when he is hungry. We will notice that when we give him a substantial snack that includes protein his mood improves.”
Charles has even taken to skiing; he is taking lessons at the Mt. Brighton Adaptive Sports in Brighton, MI. Jo and Mike can’t say enough about the ski school’s adaptive lessons! And this is just one of many examples of how Jo and Mike let Charles be a kid.
Despite the positivity and good adjustments, Jo and Mike don’t take a back seat to their son’s disease. They have turned an otherwise difficult reality into a rallying mission.
Jo has been working with the Beyond Batten Disease Foundation toward a 6 million dollar fundraising goal, in part to help fund a clinical trial for the first-ever treatment for Charles’s type of Batten.
“I reached out to Beyond Batten the night we received Charles’s diagnosis. I was frantically searching for information and found them. Within the next day I received a phone call from the president Mary Beth Kiser and within a week I was able to speak to Craig Benson who is the cofounder of the foundation and a parent of a CLN3 child. I was grateful for the connection and immediately felt the desire to join in their efforts.”
As of today, Beyond Batten’s has raised 3.7 million out of the 6 million – and both Jo and Mike have kept busy helping reach that milestone.
“The money that people donate will go directly toward funding a first ever treatment for a rare and deadly disease. It is not often in your life that you have that type of opportunity. You literally are helping to eliminate this disease.”
For Jo and Mike, life hasn’t always been so easy. But instead of letting themselves be defined by the choices that have been taken away from them – Jo has decided to define life by the choices she can make.
“It’s a choice. After Max died, we chose to continue to be a family and we chose to grow our family. Sometimes, it’s a choice you make in the long term, and other times, it’s moment to moment. I choose to get up in the morning. I chose to make a meal for my child or to go to work – and all those things add up to become a functional person.”
And sometimes, Jo says, that choice is to grieve and slow down.
“Honor the grief. Allow yourself to cry. But after, I choose to love Charles in that moment, even if that moment is frustration or him being wild – I choose to give him the most love I can – and I choose to be thankful for him and for all of our children.”
In addition to Charles, the Kleins has a whole boyband/family of younger sons, including Augustus (Gus) who is 6, Leuthar (Leu) who is 3, and Henryk (Henry), who is 19th months. Coolest names ever, I might add!
“Life’s a mess – but a good one! I always have someone asking me a question or needing help with something. I spend a lot of time in the kitchen preparing food and cleaning messes. We frequently have jokes about farts etcetera at the kitchen table. Legos are literally everywhere! Laundry is nonstop. But I can always get a kiss or a hug at any point in my day.”
“Love your children. Hug them. Be thankful for all the little things – because you can’t take love away. Love can’t be stolen.”
And through the pain and grief that Jo, Mike, and their family have suffered, they have spurred into action that can save the lives of many with Batten disease, including their Charles.
“We are hopeful that soon, there will be treatment available and that it’ll work, so we won’t have to tell Charles exactly what he has.”
And that’s exactly why Patient Worthy exists.
