Meet our sweet and spunky girl, Tessa.
But behind that infectious and beautiful smile is a heartbreaking story.
Just two months before her fourth birthday, on July 17th 2019, our lives changed forever. After more than a year of misdiagnosis and unanswered questions, a geneticist diagnosed three year old Tessa with CLN2/Batten Disease.
CLN2 is known as neuronal ceroid lipofuscinoses. It's a fatal disease that affects the nervous system. Batten disease is incredibly rare - 2 to 4 babies out of 100,000 in the U.S. are affected.
The heartbreaking reality is that within the next few years, Tessa will be robbed of her speech, mobility and eye sight. Batten disease has no mercy and those who are diagnosed are unlikely to survive.
Born weighing 7 lbs and 1 ounce, on September 5th 2015, Tessa was a healthy baby. Her early childhood seemed normal. She was an active, happy little girl, strong willed from the start - taking her first steps just days before her 1st birthday. She hit the anticipated infant and toddler milestones, all with the exception of speech.
Tessa's parents, Sean and Trista Bruner noticed speech delay around 18 months. They took her for a hearing test and also tested Tessa for autism. By the age of two, Tessa was in speech therapy for Childhood Apraxia of Speech, but her therapist started noticing additional issues with motor abilities and muscle weakness.
At three years old, Tessa went in for overnight sleep studies that showed she was having seizures in her sleep. After she experienced her first seizure, it wasn't long after that, she had a second seizure, which was longer and more intense. After Tessa had a 7-minute grand mal seizure, the neurologist decided genetic testing was the next step, which led to the diagnosis of Batten disease.
Tessa's future is grim, but this family is resilient and most importantly, Tessa is brave.
The top doctor for researching this disease and treating it to slow the progression has agreed to take on Tessa's case. There are only 7 hospitals in the country that perform this type of treatment.
Tessa will see Dr. Emily de Los Reyes at Nationwide Children's Hospital in Columbus, Ohio. Dr. Reyes ran the experimental stage of Brineura and it's now FDA approved.
Tessa will have a port implanted in the base of her skull so they can infuse enzymes directly into her brain to slow the progression of the disease. It is not a cure, but it is hope.
Tessa is currently on two medications for seizures: Keppra and Sympazan. Insurance only covers half the cost of Keppra. As for the Sympazan, they are using a medical card, but when it expires, the medication will cost nearly $30,000 a year.
The money raised will be used to assist the Bruner family while traveling to and from Nationwide Children’s Hospital in Ohio, medical equipment and prescription costs, and home modifications. Funding for research is limited because this disease is so rare. Any additional funds that are not used for medical or travel expenses will be donated to Batten disease research.
Tessa doesn't deserve this disease, but she deserves us.
Each and every dollar counts, a donation of any size would be greatly appreciated. Sean and Trista, Tessa’s family and all of our friends thank you for your time and consideration.
We are determined to make every second count.
We refuse to be overcome with fear, anxiety and sadness.
We will do everything we can to find a cure so Tessa can live a life well-lived, regardless of length.
That is our promise to Tessa.
This is Tessa's tale.