Josh arriving at Dayton Children’s Hospital
Josh is going to be having his surgery today. After waiting for over 3 years for this day, it feels completely surreal. We came into Dayton a bit early for Dr. Lober, our neurosurgeon to check on Benny. Instead of moving the family back and forth, we decided to stay. We’re here for Josh to have life-saving brain surgery/ gene therapy, yet we’re trying to keep our routines and have some sense of normal. We’ve seen the local Dragons baseball team, visited the air and space museum, and gone to the splash pad at the local park. Over the past 3 and a half years, I’ve gotten so good at “Carpe Diem” - “seize the day”, -living one day at a time, and trying to make the most of each moment we have, that it’s been hard to think about tomorrow’s.
For Canavan kids, degeneration is the norm. Slowly seeing their abilities fade away as they incur more and more brain damage over time. Longer periods where they don’t seem to be “there”, staring off, not engaged. The life expectancy for Canavan kids can be 2 years, or ten. Some, though very uncommon even making it to young adulthood. With this treatment, that can change. A new quality of life can be possible.
After Benny was treated this past April, the first thing I noticed was his focus. He had become noticeably more focused, aware and reactive. His eyes focused better, and he began making meaningful eye contact. Soon after, he began tracking us as we walked by… a milestone he never met, even as an infant.
At Benny’s 1 week follow-up, I asked Dr. Lober, if it was possible to see changes so quickly. “Well he’s the first human to have this treatment, so we really don’t know, it’s certainly possible," he answered. Dr. Paola Leone, the researcher, and developer of the treatment said, that we know from the animal studies that the new genes are active within 48 hours. It's incredible to think, that his brain is already working in a whole new way.
Benny has been fed with a G-tube since he was 2 and a half. We’d always give him a bit of food by mouth for pleasure, but now he’s begun to eat by mouth more, and more often. He tells us he wants to eat, by signing the word “eat” with his hand, or vocalizing incessantly during mealtimes. “Benny do you want to eat with your mouth?” we'll ask. A resounding big smile for “yes,” lets us know. He even began grabbing at his G-tube and feeling where it is. I tell him not to make trouble and pull on it, but I couldn’t be happier.
He used to cough all the time on his saliva… choking, coughing and pneumonia are a big concern with Canavan disease… seeing that he is swallowing better and hasn’t been coughing on his mucus is amazing, he’s so much more comfortable.
He’s playing with toys more, is more engaged and alert in everything he does. Just last night when we were changing his diaper, he lifted his bottom to help! We asked him to do lift his bottom again, about 6 times, we were so excited. When we put on his favorite songs he will vocalize, and “sing” along. He’s so engaged that he’s actually getting bored! He isn’t content to sit on the sideline and wait. I used to have to make sure that I pushed him to stay engaged. I’d try to include him in activities, like cooking, or whatever work I was doing… now he’s demanding it, and I couldn’t be happier.
He’s moving his body around more…. When I’ve been putting him to bed at night, he’s been spending time moving his arms and legs, looking around in wonder. Feeling his legs with his hands, touching his hands together… one night he even lifted his leg to touch and grab his toes a few times. I’ve never seen him do this before. It's like he’s discovering himself for the first time in a whole new way…
With these changes in Benny, I wonder what the treatment will mean for Josh. They have such different personalities. Benny is our chilled-out dude. Happy and content with whatever the day brings. Josh is more opinionated. He’s stubborn and has a determination about him. This past Tuesday Josh had his presurgical neuro visit tests done. A psychologist and physical therapist tested and scored his various abilities. We were told he scored higher than any other typical aged-matched Canavan kid. We’re lucky to have a great team of therapists at home and school to support him, but I believe it's his determination that has made him so strong. He pushes himself to do things, and he always wants to be part of the action. With very cautious optimism, I am so hopeful by what the treatment will mean for Josh. I believe he will take every bit of ability he gains and work even harder to do more. After what I’ve seen in Benny so far, and with Josh’s relentless drive, I have so much hope for how life-changing this treatment will be for him. I can’t wait to see what he will do and gain.
Of course, I say this with very cautious optimism. This is major surgery with serious risks. Four boreholes will be drilled into his skull and catheters will be inserted into the four ventricles in his brain, to deliver the genes directly where they need to be. The risks of the surgery itself are huge, and we need all of your help. When Benny had his surgery, I could truly feel the power of all the prayers from around the world coming together. I was so grateful to have that support when we so needed it. Today, we need your help again. We will be arriving at the hospital at 6:45 am, after anesthesia and MRI, surgery will begin at 10 am.
Please think of Josh, pray hard, send good thoughts, positive energy, and all the love, for healing that you can. We need you.
Jennie & Gary, Michael, Benny, Josh, Evan and baby Raqueli.
The boys and their Hebrew names as follows:
Shalom Binyamin & Yehoshua Natan, ben Shayna Toyba
שלום בנימין בן שיינע טייבע ויהושע נתן בן שיינע טייבע
Cure Canavan Fund is a registered non-profit 501C3. Donations to Cure Canavan Fund are tax-deductible. EIN# 83-4489648
Josh getting presurgical testing
Enjoying Memorial Day weekend together