In 2012 When you googled Battens disease the same words appear on every site “ A extremely rare and fatal autosomal recessive neurodegenerative disorder” followed later in the articles with “No treatment or cure” and “Eventually children with Battens disease become blind, bedridden, demented and die”.

Probably not the best idea to google it when I was home alone. Brady’s father was in Red Deer at the farm show and I had the 2 boys at home. First person I called was my mom. I didn’t tell her much on the phone, I just said “can you come over? I Know what Brady has” and without a question she was on her way. And then I went to B. He was in his room playing with a fire truck on his bed. I just stared at him. He was still so able, he was walking around, smiling, playing. He was a perfect, innocent child. I couldn’t imagine him bedridden. I couldn’t picture him blind and demented. I was just told that my son was going to die, and there was nothing I could do to stop it. I tried to hug him, in tears wrapping him in my arms…only to be pushed away. He didn’t understand why I was upset; he didn’t care either. He just wanted to play with his truck, like any other 3-year-old.

My mom came, and I couldn’t tell her what B had been diagnosed with. I just told her to read what was on the computer. The rest of the evening was a blur. Letting immediate family know, spending time on the computer doing as much research as I could only to find I didn’t want to read any of it. Finding pictures of kids just like B. Kids who once were born completely healthy without a care in the world, and were now bound to wheelchairs with feeding tubes and oxygen tanks. Hands and arms contorted; eyes unfocused. Reading the stories from families that had endured the disease and those who were going through it at the time. I couldn’t read it anymore. I couldn’t stand to look at another picture. How could this be? How can this happen to kids, how could this happen to Brady? It couldn’t, its not possible. Its not fair. I was angry and scared, but mostly I was devastated. I had just been told that my son would die. Everything I had ever hoped for B was gone in a moment. There were no more dreams of anything for B. No NHL, No MLB, he could have been anything in this whole world, and even the less grand dreams of graduating high school, getting married and having children of his own. Even the smallest hopes and dreams for him, being able to talk to his friends, being included, getting to go to school at all. The only hope I could have for B now was that I could make the remainder of his days the best that they could be. That I could keep him as comfortable as I could for as long as I could. I would make B know how loved he was, everyday for the rest of his life.

It was such a surreal time. The grieving started instantly. I spent a lot of time with my family right after the diagnosis. I remember talking to my brother in law, and he said, “whatever gods will is, will be” and I said “what if his will is for B to die?” but no one had an answer for that. Word spread quickly, most people didn’t know what to say, and some shouldn’t have said what they decided to say. I was told that Brady could be healed, that I needed to pray harder, that I wasn’t asking for the right things. I was told that miracles happen every day, and I needed to believe. I know people were just trying to be positive and were hoping what they were saying would help, but it didn’t. I had just been told that my son was going to die. NO one had ever survived this disease, why and how would B be the first to somehow survive it. And I felt like all those words placed the blame directly on me. I didn’t need that. I had enough on my plate without adding mass amounts of guilt for being the reason B wasn’t being healed.

3 days after I got the call from the Dr we went to meet him and the geneticist to discuss the disease and what it meant for B. We sat down at a table, and the geneticist had asked if we had read anything about the disease. I told her what I had read on the internet, hoping that the information I had received was all wrong, and that there was more we could do. I was mistaken. She said everything we read was true. She went on to tell us how B inherited the disease. Turns out B’s father and I have the EXACT same defective Gene (CLN2). The CLN2 gene is responsible for the production of a lysosomal enzyme called TPP1. A deficiency of TPP1 results in abnormal storage of proteins and fats in the brain cells and impair cellular function. In short, B got both copies of our defective gene, which was causing his brain cells to die.

They referred us to palliative care, to the Rotary Flames house, Family Support for Children with Disabilities as well as filled out a Children’s Wish application on Brady’s behalf.

Numb is the best word to describe how I was feeling after that meeting. And numb would be how I felt from that moment on. I shut down the emotion side of my brain, I wouldn’t be able to take care of B the way I needed to if I was crying all the time. I had a job to do, and B needed me to do it to the best of my ability. I had to let Aspire know about the diagnosis. They were such a huge help in the months following B’s diagnosis. The occupational therapist set us up with Aids to daily living to get us diapers as B was starting to become incontinent. He also got us in to the seating clinic as soon as he could so we could get B’s wheelchair ordered, as he knew it could take a while.   We also equipped B’s room with a lift system, as well as the bathroom for when we needed it. The school was so amazing. They always helped as best as they could. Making sure B stuck to his keto diet, which he was very happy was over when he was diagnosed. They kept seizure records for me so I could have as much information as I could when I went to Neurology appointments. They took every change in stride, just as B did. B started the year sharing 1 CDF (child development facilitator) and by February had 2 full time CDF’s. I cannot thank Aspire enough for what they did for B, or for myself. I am so grateful for the wonderful staff, especially B’s first CDF Stephanie. He loved her very much.

B started IVIG treatments right after his diagnosis. IVIG (intravenous immunoglobulin) it a mixture of antibodies used to treat several health conditions. In B’s case we were using it to keep him as healthy as possible, because when B got sick his seizures would increase and there would be more cell death. As well as the IVIG had some brain protecting qualities. The treatment took about 4 hours and we went every 3 weeks to medical day treatment at the ACH to have it administered.

In January 2013 We got to go on a whirl wind wish trip. My parents joined us as well as Brady’s father’s family. We flew to California. We went to Disneyland for 2 days, Universal Studios, Lego Land, Sea World and San Diego Zoo. It was the best and most exhausting trip I have ever been on. B loved meeting all the characters at Disneyland, He LOVED going to Lego land and getting to ride the Lego horse. The ride attendant would let him stay on over and over. You couldn’t take the smile off of his face, although they probably thought I was crazy, pacing beside the ride because I couldn’t go on it with him and I was worried he would have a seizure and fall off, or worse be stuck on a Lego horse seizing. While at Sea World he got to feed the dolphins, which would have been a bit more magical if he didn’t have a tonic clonic seizure right before from the glare off the water. He enjoyed running through the hotels in his walker. We all had a great time, until we went to fly home. B was already starting to have a hard time swallowing, which was one of the many abilities he would lose, and while we were in California, he aspirated and developed Pneumonia. He was coughing so much, and the flight attendant had to call the airline doctor who told us we should wait until he stopped coughing and catch the next flight out. I knew I could handle what ever he threw at me, and that we just had to get back to Calgary. After signing many releases saying they would not be at fault for anything we finally got to take off. And sure enough, once we got home and I took him in to the hospital he was started on antibiotics, one of his many bouts with pneumonia.

In the start of February, the communities around where we lived had a benefit for B. It was called Bear Hugs for Brady. Over 1000 people showed up to show their love and support for B. It was an overwhelming experience. I could not thank everyone enough that night as I still cannot. That fundraiser made it possible for B to have the best life possible. That fundraiser helped us get the equipment that we desperately needed. B’s wheelchair weighed 60 lbs, and at that point B was in a wheelchair full time, so at school I was having to lift B into the van, Lift his wheelchair into the back, drive an hour to Red Deer, Lift it out of the van, lift B out of the van and into the chair to get into school, and then do it all over again when school was over. Because of the benefit we were able to get a wheelchair accessible van, and that made a WORLD of difference. B could sleep comfortably in the van as we drove as his chair could tip back. There was so much more room to give his feeds and his medications, and as sad as it was it gave me more room to deal with the seizures that would happen.

In June B received his feeding tube and had a port put in. He was still able to swallow, but we felt it was best to get the tube put in while he was still strong enough to undergo surgery. The surgery itself didn’t take long. B had some issues with his blood pressure when he came out of surgery, but they leveled out and we were sent up to the unit to recover. I remember B waking up in so much pain. I felt so guilty. That morning I had to wake him up to drive all the way to the city to get a surgery he didn’t ask for, and now he was in pain. I wish I could have known then how much easier the feeding tube would make B’s life, as well as mine. No more trying to get him to swallow meds or wondering how much he drooled out.  He never asked for food after his feeding tube was put in. He was perfectly content to receive his feeds. At first, he was on gravity feeds, and that was painfully slow, every time I looked away from the bag of formula it would quit dripping. So, I complained until B was given a pump. What a world of difference. Feeds went from taking over an hour where I had to watch it the entire time, to half an hour and a handy little alarm would go off to let me know when it was done. All I had to do was mix his formula in the morning and clean the supplies. Every night you could find me at the sink washing his feed bag and syringes. Washing everything with soap and then doing a vinegar rinse. The best was every three days when I got to throw the supplies away and start fresh the next day. Its small victories like throw away night that got me through