Our Son Ollie – A Mother’s Story

by SUSIE EGR

This is our handsome son, Oliver James Egr. He is a big piece of our “why” and our superhero. He turned the big thirteen a few months back. When he was born I remember saying “Thank you God for all the abundance in our lives and for this sweet baby boy. He is perfect in every way and has now completed our family ”. He was the typical little boy- ornery but so dang cute. When he started kindergarten we noticed he kept sitting close to the TV and he wasn’t always paying attention. We soon learned he had visual issues. That led to months of additional neuro and genetic testing. I vividly recall that horrific day we received his official diagnosis.- Batten Disease. The genetic company called me on the phone while we were driving to Iowa City for an eye specialist appt for Ollie. To say it took my breath away is an understatement. Darin pulled the car over while I continued to listen on the phone. Ollman was singing “the ants go marching one by one” in the backseat (Not a care in the world.. while ours was being turned upside down) tears instantly flowed from my face and Darin just knew the results weren’t good. I remained quiet as I didn’t want to worry Ollie. That was the longest drive of our lives. After I got the news Dare and I just sat there holding hands with tears streaming. I felt helpless. “No cure….Fatal” is all I heard and it just kept playing over and over in my head. 

Ollie lost his complete vision 6 months after that appointment. Over the years we have seen this disease not only rear it’s ugly head in our son but take the lives of so many innocent, sweet kids. These affected families have became our extended family now. THIS HAS BECAME PERSONAL. 

My faith is strong and I remind myself frequently that our loving Father loves this child more then I will ever comprehend and he walks beside us daily as we fight the good fight. We feel his presence and see him through the kindness of others. That gives us hope. As parents we will never stop advocating for Ollie and the Batten community because they need us! What also gives us hope is putting our efforts towards funding a cure. We are so close!

BBDF 101 – Next Steps to Clinical Trials

In July, BBDF sponsored the Batten Disease Support and Research Association Family Conference where we had an opportunity to update affected families on the progress of our drug development program for the combination therapy BBDF 101.
Visiting one on one with parents at the conference allowed us to share information on the next steps required by the FDA to initiate clinical trials. We were also able to acknowledge the generosity and support of the BBDF community that created the opportunity for these children to receive the first ever treatment for juvenile Batten disease. On behalf of all the families we talked with, thank you for making this treatment a reality for their kids!
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Go Tyler! Beat Batten! – A Sister’s Story

by JAMIE KIM

My brother, Tyler, has always been the most energetic happy little boy I have ever been around.  He never runs out of things to do or things to say.

A few years ago, he started to have seizures and began to lose his vision.  Around a year ago, our family found out that Tyler has juvenile Batten disease. Batten disease is a rare and awful neurodegenerative disease that is terminal. There is currently no treatment or cure but there are researchers who have discovered a treatment that slows the progression of the disease and others are working on a possible cure for the disease.

When I first found out he had this disease, I was a little in disbelief.  I have seen many commercials of people diagnosed with terrible diseases but it never occured to me that it could happen to Tyler.  He seemed like such a happy and healthy boy that it was hard to understand that such a devastating disease was hurting him.

In the first few months of his diagnosis, I did not want to talk about it with anyone outside our family.  I did not know how to tell people what he was going through because it is not a common disease and I knew no one would really understand what was happening.  Our family began first with telling close friends and extended family. More recently, we joined other affected families and set up a fund at Beyond Batten Disease Foundation called Tyler’s Mission.  All donations to Tyler’s Mission go to the research for a treatment and cure for juvenile Batten disease. Seeing all the people who have donated to this cause has showed me how many people care and support our family and it really means so much to us.  It did not cross my mind that so many people who do not know us were also donating and leaving comments of support. Looking at these comments made me realize that it is possible to raise the 6 million dollars needed by next year to start a clinical trial for the treatment for kids living with Batten Disease. So far, Tyler’s Mission has raised over $45,0000! 

This summer, I want to make a difference.  I want to help raise money and awareness of Batten Disease.  It is urgent to raise the funds as quickly as possible because time is not on our side. With donations and support, there is hope for a treatment and ultimately a cure.

Every day I am reminded there is hope for my little brother and others with Batten Disease.  I love Tyler more than words can explain and he brings me happiness everyday from his little kisses to his big squeeze hugs to his goofy bad jokes.  Although he has hard days and struggles sometimes, he always hold his head up high and helps me see how much there is to appreciate in our lives.

Please help us raise funds and defeat Batten Disease by donating to Tyler’s Mission at beyondbatten.org/tylers-mission or visit the Understanding Batten page to learn more about Batten disease. To stay updated with his journey, follow his Instagram, Twitter, and Facebook @tylersmission.

GO TYLER! BEAT BATTEN!

Pieced Together – A Mother’s Story

by AN ANONYMOUS BATTEN MOTHER

I grew up in a family of story tellers. Every get together involved reminiscing and sharing the same old stories. Most happened before my time, yet I’ve heard them so much it’s as if I was actually there.

Many stories involve my Dad, his two brothers and his sister.   Many include their childhood shenanigans. They would be playing in the house and something would get broken. They would do chores and something would get broken. They would move furniture to turn the living room into a football field and something would get broken. The result would be my grandmother grabbing the glue and putting it back together.

I know these broken things on her shelves. They weren’t matching sets or family heirlooms. They weren’t pieces that were priceless or irreplaceable. But they were special to her. So as things broke, she could have easily thrown them out, but she chose to piece them back together. Displaying them once again in hopes that no one would see the damage that had been done to them.

I often think about these broken knick- knacks and feel we have a lot in common. All it took was one visit to the doctor’s office to knock me down and shatter me to pieces. As the doctor revealed our diagnosis, I wasn’t even sure what he was saying. But when he said it will affect other parts of his body and his longevity, I literally fell to the ground. I was completely broken. Not the broken that leaves you in two pieces. But the broken that shatters you. Picture a glass dropped from the highest cabinet in the kitchen and exploding into tiny slivers everywhere. I was that kind of broken. How would I ever get up off the floor?

Then I thought of the words written by my sweet little boy just 4 days earlier on his Star of the Week poster…I am a star because “I never give up.” That was the glue that started putting me back together.

I walked out praying, you know the kind of prayer that sounds like a jumbled ramble of words that even if God was yelling from the top of his lungs to guide me, I wouldn’t have even heard him. It was that kind of prayer. But, He glued me together enough to put one foot in front of the other to walk out of that doctor’s office.

We came home and I looked at our daughter. How is this ever going to be ok I thought and the glue started letting loose. She hugged me and asked if we could cuddle on the couch together. She held me tight. Gluing those pieces right back together.

That night on one of the first of many sleepless, tearful nights I said to my husband, “What are we going to do?” He said we are going to get up and go to work and school, just like we do every other day. Our children deserve us to support each other and not fall apart. His love for our family was the glue that I needed once again.

The days to follow, 902 to be exact, I have fallen apart and been glued back together more than I can count. If someone looked closely, they would see that I am not who I once was. Just like the knick -knacks on my grandma’s shelves, I am together, but a little different than I used to be. But as pieces break off, God finds a way to glue me back together. He provides glue from my family, from the courage of Craig and Charlotte, from the kindness of Mary Beth, from the encouragement of Danielle, from the strength of Wayne and Missy, from the knowledge of the scientific researchers and from the promise that He has a plan the far outweighs my understanding.

Defying Gravity by Joe Sikorra

Long time BBDF supporter, Joe Sikorra has published a book and dedicated a portion of the proceeds to BBDF! You can snag a copy via Amazon, but remember to shop via Amazon Smiles (www.smile.amazon.com) so that .05% of your purchase will be donated to your favorite charity. You can purchase the book directly HERE.

This moving story shows how a family found joy after their hopes and dreams were shattered by the rare, fatal neurological illness of their two sons. Told by the family’s father, a popular Catholic radio host and marriage counselor, it recounts the way he and his wife received the courage and the support they needed to give their children abundant, fulfilling lives in spite of their gradual decline.

What initially seemed to be a tragedy for Joe Sikorra’s family became a story of human triumph, an outcome made possible by the compassion and mercy of God. Their example can help those facing unexpected losses and challenges to believe that by placing their trust in Providence they too can overcome hardships with the most powerful force on earth—God’s love.

FDA Meeting Update


In May BBDF had its first meeting with the FDA to get input and direction on our plan for clinical trials. This meeting was a huge milestone and an important first step in the process to develop BBDF’s drug combination for the treatment of juvenile Batten disease. (Pictured above: key opinion leaders and BBDF team at the FDA)

We are happy to report that the FDA gave very positive feedback and it was clear they want to help us reach our goal. To put it into context, our regulatory consultants reported that they had never attended a meeting where the FDA was so interactive, motivated and engaged. The FDA guidelines provide a clear path forward for our program and do include some action items for us to address which will help set us up for success when we submit the final package. BBDF, our consultants and advisors are rolling up our sleeves and getting to work in order to get these Batten kids treated as quickly as possible!

June Update – Only 6 months to go!


Only 6 months until we wrap up our first ever capital campaign to fund the treatment for juvenile Batten disease. Time is of the essence as we still need almost $2 million to reach our $6 million dollar goal but with fundraisers on the calendar and help from our generous supporters we know we can do it! Take a look at our June highlights from last month’s newsletter! READ MORE

BBDF and the Louise H. Batz Foundation bring you Family Night 2018!

In it’s 4th year, the annual fall fundraiser, Family Night, is set for Sunday, September 23, 2018 and BBDF is excited to announce that this year’s event will be co-hosted by the Louise H. Batz Patient Safety Foundation based in San Antonio, TX. If you haven’t attended Family Night in the past, we have made quite a few changes and cannot wait for you to join us in “Splashin’ for a Passion!” For more information, including sponsorship packages and ticket information please visit beyondbatten.org/events/family-night.

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