By Karen DeVenney
Mommy to Izekiel (“Bacon”)
Batten Disease (CLN2)
Born November 2018
Our journey started out pretty normal. Our son, Izekiel—who we call Bacon—could walk, talk, play, eat, and was very strong and intelligent. He was just a typical, happy little boy.
Yes—he got his nickname honestly. When he was younger, he absolutely loved bacon. One morning at my in-laws’ house, he kept going back for more, and my mother-in-law kept saying, “Bacon, bacon, bacon.” The name stuck.
One Monday, I took the kids to Burger King to play. When we went to the bathroom, the floor had been mopped with a red grease mop from the kitchen. Bacon slipped, fell, and hit his head. The next day, he was outside running around when we noticed him staggering. He collapsed in my husband’s arms, went limp, and turned blue.
We rushed him to the hospital, where they diagnosed him with epilepsy, ran a bunch of tests, and sent us home. Less than a week later, it happened again. At that point, they were calling them seizures. This time, they did more extensive blood work and discovered that Bacon had Batten Disease—CLN2—a very rare brain disease that happens when my husband’s and my DNA mix.
In the beginning, I wanted to do everything I could to keep my son here on Earth. We started brain infusion treatments, believing they might give us an extra year with him. Unfortunately, the infusions didn’t work for our son. In fact, they caused his disease to worsen and progress faster. That was incredibly hard, especially because another family started the same Brineura brain treatments at the same time, and it worked for their child—but not for our sweet boy.
After a lot of prayer, and realizing I didn’t want to put my son through any more suffering than necessary, we made the heartbreaking decision to take him off the brain infusions.
Shortly after stopping the treatments, Bacon got COVID and strep throat at the same time. From there, he began losing his abilities very quickly. He lost the ability to walk, talk, and eat on his own. He is now wheelchair-bound and has a G-button for feeding.
We now live day to day, never knowing when God may call our son home. Watching him slowly decline has been devastating for our entire family—mom, dad, and all three of his brothers. We can’t do the things we used to enjoy, like hiking, because his wheelchair can’t access many places. He can’t be in extreme heat or cold for long periods, because either can trigger seizures. His seizures are severe, to the point that doctors worry one day we may not be able to bring him out of one. And unfortunately, there is no cure for this disease.
Hospital stays have been especially hard on Bacon. The last time we took him to the hospital it was terrifying. The doctors were unfamiliar with his disease and wanted to intubate him. Some didn’t even understand the difference between dystonia and seizures, both of which are part of Batten Disease. In February of 2024, we were told Bacon had two weeks to live. We brought him home, but God had different plans. He is still here, still fighting every day. He is on hospice now, and we continue to trust God.
My three other children are really struggling, as are me and my husband. It’s heartbreaking to watch. The doctors can’t believe Bacon is still alive, and we thank God for every single day we get with him.
Bacon has a huge personality. He loves tractors and music, and his laugh is so contagious. He continues to make me the mom I am today and teaches me something new every day. He has his own way of communicating and showing love.
A friend told us about On Angels’ Wings. I had wanted family photos so badly, but every time I saved enough money, something else would happen and we’d have to use it. Our OAW photographer has been incredible. She made sure Bacon was the center of attention while also including all of our other children. She always made each of us feel special and truly went above and beyond. You could feel the love and compassion throughout the entire session.
Being part of the OAW community has meant so much to us. I love how everyone comes together like family—like when we went to a Cardinals game together. It’s amazing to see how many people they’ve helped and how many lives they’ve touched. With the financial burden of traveling back and forth to St. Louis for Bacon’s care, our savings depleted quickly, and something as simple as family photos felt impossible.
I didn’t want to admit that I couldn’t afford photos, but they never once made me feel like less of a person. In fact, they made our entire family feel incredibly special—especially Bacon. That blessing meant more to us than words can express.
My hope is that God heals our boy here on Earth, but I also know that true healing only comes when we are home in Heaven. I pray that God uses our son’s journey to restore faith in others and remind them of His presence.
