Chase “Jasper” Battle With Battens

Chase The Cure

February 28, 2018

In 2015, after a recommendation from Chase’s 1st grade teacher we had Chase seen by a pediatric ophthalmologist. During the examine the doctor found signs of a dystrophy, which prompted him to recommend Chase to a specialist at the Moran Eye Center at the University of Utah. After additional, evasive testing we were informed that our son appeared to have ConeRod Dystrophy, was considered low vision with the possibility that his vision could deteriorate to the point of blindness.

Upon the recommendation of the specialist, in 2017 we submitted a DNA test for screening. The result came back that Chase possessed the reported 1 kb deletion in the CLN3 gene, now diagnosing the very rare disease Classic Juvenile NCL, Batten’s Disease. Loss of vision being the first symptom, seizures, and progressing to physical and mental incapacitation requiring 24-hour care until premature death in teens to early 20’s. Our family was caught off guard and absolutely devastated. The doctor delivering the news was very cold and non empathetic. He informed us that at this time there is no treatment and no cure available.

Let me introduce you Chase. At the age of three, he renamed himself to Jasper the Champion Rider Shooter. At age four we were allowed to shorten it to Jasper the Champion. He is now nine and is known as Jasper, with most people unaware his real name is Chase. He loves telling stories, the holidays, playing cowboy, teaching others and the animal cows among many, other activities. With his vision impairment he amazes me everyday. He is considered legally blind and not once has he ever complained or felt bad for himself knowing with his vision he is not the able to see as well as his brother, family and friends.

We began researching Batten Disease, at that time neither of us had ever heard of it. We were in a state of shock and devastation. We cried so many tears – our hearts were broken. I was now to advocate for Chase and I couldn’t even have a discussion about it without having a breakdown. I don’t know how to explain what it is like to watch your active, social boy with an abundant personality each day, knowing in the back of your mind the diagnosis. Questioning will there be a progression today? Will he be ok if I send him to school? The thought that in the future our son could be unable to dance his funny dance, unable to sing his favorite songs, unable to communicate with us. No!

We became tired of the specialists response of “there is nothing we can do, call us if you need us”. This was unacceptable. I started researching any information I could find, organizations that were created by other families who have lost their child, support groups, pending treatments and cures. We came across the Beyond Batten Disease Foundation, a nonprofit organization and Abeona Therapeutics, each with treatments in sight. One a drug that slows the progression of the disease and the other a gene therapy that delivers a normal copy of the defective CLN3 gene to cells of the central nervous system. The Beyond Batten Disease Foundation has been working diligently to advance their treatment to clinical trials, as has Abeona Therapeutics who is spearheading the gene therapy.

Being a rare disease, there is limited government funding and big drug companies refuse because they feel they will not recoup the costs of finding a treatment or cure due to the small numbers of affected children. Because of this the work organizations are able to do with patient advocacy groups and families working together is so important to addressing the disease for Chase, the other children and their families. Your financial support can help us get to a clinical trial in 2018. This treatment can also help others affected by other lysosomal storage disorders, and possibilities for other neurodegenerative diseases like Alzheimer’s and Parkinson’s.

I sincerely appreciate your time and consideration in seeking to understand Batten’s disease, how it affects families and how your support can help bring some peace of mind to families in 2018 with the hopeful release of a clinical trial for the first ever treatment and hopefully soon to follow a cure.
Sincerely,

Peterson Family

Contact: 801-668-7060

@ChaseTheCureFund