Workout of the Day

Guest Post: Battling Batten Disease - Team 4 Titus and Ely

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Hello CFNB family,

I am writing this to you all to share about the amazing family behind my reason for doing this event. If you don’t already know, I had the immense pleasure of being a home health nurse for over a year to a very special little boy. He had a very rare disease that was neurodegenerative called Batten Disease, and more specifically, CLN2. It is an extremely rare and fatal disease that doesn’t show symptoms until kids are between 2-4 years of age, even though it was genetic and in their bodies from birth. In layman’s terms, he was missing an enzyme whose purpose was to help clean up all his cells. As waste built up and his cells were not cleaned by the enzyme, cell damage began to occur and eventually led to cell death. On the outside, what we can see from our eyes is a child who once was developing normally begins to lose the skills they once had. When I met him on August 12, 2015, he had lost his ability to walk, talk, eat, see, and move his body with purpose, his only sense left was hearing. Batten is an autosomal recessive genetic disease which means both Danny and Bekah, Titus’s parents, carried one good copy of the gene and one mutated copy of the gene. Each child they have has a 25% chance in actually having the disease (meaning they each pass on the mutated copy) and 50% chance that they will just be carriers (meaning only one passed on a mutated copy and the other passed on the good copy). In the Bowman’s case both their children have the disease. It didn’t start to show itself until Titus was 3 ½ years old, so Ely was already born. They received the actual diagnosis after searching for answers to Titus’s struggles just before Titus turned 5 and just before Ely turned 2.

When I started this job, it was just a job. I fed him through his gastrointestinal tube, gave him meds through the same tube, and changed him when he needed it. After only a few short weeks, it was no longer “just a job”. While hanging around Titus and his family for 40 hours a week, it became apparent to me there was something special going on. I couldn’t imagine being a parent of a healthy child and then at 3 years old seizures begin along with regression in speech and motor skills. Then finally after a year of fighting for answers for your child, knowing in your gut something is wrong, being told that not one, but both your kids have this fatal disease. The Bowman’s are the strongest people I know and I am a better person for taking this job and meeting them.

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Titus was not just my patient, but like a nephew to me. He was the same age as my own nephew and the videos I saw of him when he was running and talking were exactly how my own nephew acted. I no longer considered my “job” a job, but merely a day spent with this amazing little boy. Most days together were spent at school, playing with playdough, singing songs (really me singing in his ear and he would make faces like “stop singing please”), and snuggling while watching his favorite movies over and over and over again. His 3-year-old brother was also there every day when we were home, so of course if Titus was sleeping, I would play with Ely and we became very close. When Ely woke up from his naps he would come out to the family room where Titus and I were and he would run and jump into my arms and say, “tic tic tic” which was his way of asking me to play tickle monster with him. Needless to say, I became very attached to every person in the Bowman family.

On September 13, 2016, I was at “work” and Titus and I were cuddling on his recliner chair when I realized it had been about 30 seconds and he hadn’t taken a breath. I immediately laid him down and began counting breaths and he was at 2-3 breaths a min. His mother called her husband home from work and the family over right away. I just stayed close to him to continue monitoring his breaths. We knew he didn’t have a lot of time since his hospice had already told us he was in the end of life stage. Titus’s oxygen was at 78-82% on 12L of oxygen (normal is 95-100%). The whole family came over and just laid with him and kissed him and made him calm and feel loved. I took pictures of the family with him and just stayed to keep watch, and give meds when needed. I left at the end of my shift and kissed him goodbye, giving him one last hug. I knew in my heart that was the last time I would see him. Titus passed away 4 days later in his mother’s arms. His memorial service was joyful and sad, but he was no longer in pain. Ely, Titus’s brother, got accepted into the clinical trial 10 days after his brother passed away from the disease. He has now gotten 17 infusions of the missing enzyme and is doing great so far.

I am telling you this sad, yet hopeful story because we all have the opportunity to be a part of history and help raise money and awareness to find a cure and fund the ongoing trials at CHOC Orange for Batten. On the weekend of June 3rd till June 5th, there is a virtual 5K for Batten disease. I am challenging each of you to register under the name “Team 4 Titus & Ely” and we can walk, run, skip, or stroll together for this amazing family and others just like them. Together we can make a difference in the lives of so many children who have this debilitating disease who could possibly get better with this trial. You can register HERE to join our team.

As a nurse, we are taught to be prepared for all things. I believe that because of this family and this little boy, I am now prepared for anything. Because of Titus, his little brother was diagnosed early and is able to have a shot at halting this disease in his body. Ely will be forever grateful because his big brother paved the way for him.

Thanks for reading and I hope you all will join me next weekend!

- Mara Levy