ScheisskopfFTW2319 karma2018-11-26 21:31:40 UTC
Zellweger Syndrome is a spectrum disorder. All cases prove fatal it just varies with how long it takes before you die. Some live days some live years. As very long chain fatty acids accumulate due to the condition the central nervous system is slowly destroyed.
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ScheisskopfFTW2033 karma2018-11-26 20:57:33 UTC
It's been incredibly difficult, at least it has for me. I'm sure Taylor will answer as well. Before Lily's diagnosis I couldn't even imagine going through something like that. Now that I've experienced it, I don't know what to think.
Watching your baby struggle destroys something in you. The seizures, screaming, and apneas are all torturous to witness. At the time it seems like an insurmountable rolling hill. Each iteration becomes even more steep than the previous, but you keep pushing not because you want to, but because you have no other option. You can't let your child die because of your inaction.
Sometimes I debated the validity of my efforts. We knew she was going to die from day two of birth. She was struggling and sometimes it showed. Every action you take as a parent in this situation brings guilt. There's no correct answer. It's a moral gray area and there's no way out.
Once Lily died things changed wildly. Suddenly there wasn't a constantly beeping alarm to tend to. There was no feeding and changing schedule. There was no medication to give. Only memories to clean up and box away. Items to be categorized; those that are thrown out and those that we keep. What do you keep? What's important? What will I miss? Is it weird that I have an attachment to this item but not that one? Should we bury or cremate? What do I say at the funeral? The funeral is over what do I do now? It's been four months already? The world moved on. The cards stopped coming. The meal train is over. We are still here and it feels the same as day one.
Instead of wallowing Taylor and I decided to try to help others. Our nurses helped us with random item recommendations that we bought. The least we can do is pay it forward to others. Hopefully our boxes help parents spend less time cleaning and organizing so they can spend more time creating memories with their kiddos. Memories are all we have left.
ScheisskopfFTW1492 karma2018-11-26 22:43:35 UTC
Great question. No one knows what to say and depending on my mood sometimes there's nothing right to say at all. Tay and I try not to take anything personally. Most folks mean well and that's all that matters.
There are some things that can cross the line. For instance, a certain side of our family continuously disregarded our requests. We needed to constantly remind them to speak softly around Lily, wash their hands, etc. Due to her condition all of these things could easily trigger a seizure or get her fatally sick. Our requests we're met with a judgemental adherence. That got to be a bit frustrating. Another mistake folks often make is throwing the "if there's anything you need" line. Don't ask "what can I do?" This forces me to give you a task to feel important. The helpful people found what needed to be done and did it.
The most hurtful comment was made by my mother. After several visits Taylor and I decided to minimize contact with my family due to their behavior. We couldn't risk getting Lily sick. After her death my mother called me and casually said, "I would've been closer to my granddaughter if you would've let me see her more". That one hurt quite a bit.
ScheisskopfFTW899 karma2018-11-27 00:31:53 UTC
That would be lovely. We are fairly new and haven't applied for any grants. We are definitely interested.
ScheisskopfFTW890 karma2018-11-26 23:08:54 UTC
Good question and thank you so much!
There are quite a few complications due to the Syndrome. Usually patients will have multiple broken bones and destroyed livers from the heavy use of antiseizure medications. They also suffer from enlarged or missing portions of brain matter. Often times the child will pass from suffocation. The central nervous system reaches a state in which it can no longer tell the lungs to breath. Oxygen saturation slowly lowers until they stop breathing.
On July 5th Lily had 40 seizures in an hour. The following week on July 19, 2018 she began to lose oxygen saturation. She went from 100% to 85% oxygen. This wasn't uncommon as Zellweger kids have apneas frequently. I went through all the procedures. I used a suction machine to clear her airway, spoke to her, held her, sternum rubbed her, and turn up her oxygen. 70%. Nothing seemed to be working. My wife and I continued trying to save her for about ten minutes until the suction machine started pulling blood from Lily's throat. 60%.
At that point I completely panicked. I've been in some pretty rough situations, but realizing she was dying was cataclysmic to my world. I ran into the spare bedroom and turned her oxygen on full blast. 50%. Nothing i could do would help. My hands shook violently and I begged her to stay. Lily stared at Taylor and looked absolutely terrified. 40%. It was time.
Taylor and I told Lily it was okay to let go and see Jesus. We turned on her favorite jazz playlist (what kind of kid likes jazz?) and held her. 30%. Her breathing became sporadic and short. Lily finally relaxed. She had heard our message and was finally giving in. Our dog Piper came and rested her head on me as I laid with Lily.
Slowly over the course of the next fifteen minutes or so my light went out.
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