Leaving for the hospital almost six weeks ago now, realistically I would not have believed you if you told me that we’d be here this long, with still a good chunk of time to go. But from a mother’s gut instinct, I knew it wasn’t going to be good. I’ve been terrified and have had this gut feeling for a long time, after seeing so many respiratory failures and arrests, being bagged and coded many times, that one day it would get the better of him. No matter how stubborn and strong he proves he is, whatever disease this adorable little boy has, isn’t something like cancer where he can try and fight it. He and we have no control over what his brain is going to do to his body, and when. As a parent, it terrifying and heartbreaking that you can’t help you kid, be able to just slap a band aid on it and make it all better.
We’ve been a little reserved lately and withholding information from a lot of people, besides our close family and friends. Things got caught up in a big tail spin and weren’t ready or had the energy to update and share the life threatening situation that Riley was in. He’s nowhere near 100% out of the woods yet, things can change on a dime with this kid, so still holding our breath. But things are certainly looking much better than they did a week ago.
So we’ve had two care conferences within the last week and a half in preparation to get Riley ready for extubation, and kind of start to address all the “what ifs”, to have plans set in place and not be making decisions in the moment. We first met with his care team last Tuesday to discuss specifics on his second extubation trial attempt, what bipap mask to use, settings, what to do about his comfort level, etc. We extubated the next morning, expecting we’d get him back on bipap like the last time, and if he had issues he’d probably show us throughout the day, like the last time. Nope. I don’t know if it was THE most terrifying moment for me, as I’ve sadly had a lot of them with Riley, but it’s certainly up there. Tube came out, and he tanked. And tanked fast, faster than even the doctors expected. Now thankfully, they’re always prepared, so they were able to knock him out really fast and re-intubate him within a matter of minutes. But I certainly have his terrified look of “Mommy help me, I’m scared, I CANNOT breathe”, burned into my brain.
Trying my hardest to not cry as I type all this, so I can at least see what I’m typing! Like I said, not even the doctors expected him to go downhill so fast. From the very unexpected outcome, things went pretty quickly in regards to trying to understand what he was and was not doing, and getting that tube back in him. From what the doctors gathered and explained to me, is that his entire upper airway (throat muscles) collapsed and not even the pressure of the bipap mask could get through them. On top of that, they had a lot of differing opionions on his drive to breath with his weaker diaphragm and lungs. X-ray afterwards to check the replacement of the breathing tube showed that just in that matter of minutes, his upper right lung had already collapsed. Thankfully being back on the vent, he recouped rather fast and was back to his original low pre-extubation settings, within a day. I waited for things to calm a bit after all this happened before making the hardest call to Chris that I’ve ever done. Thankfully his boss and company has been so overly understanding, compassionate and accommodating; there wasn’t a question when he updated them and they up and took over his route. And he’s been out since, and they’ve been dealing. Guys, can’t thank you enough for that.
As you can imagine, after that horrible and unexpected experience, since then we’ve had a lot of life and death questions, scenarios and things that we’ve started discussing even more than just a couple weeks ago. As shit seemed to get REAL, REAL FAST. The thought and clinical evaluation of him this time last week, was straight up basically his breathing system is starting to fail him. Whether it was strictly his upper airway muscles, or his lower breathing muscles, or all of the above. The fear was that extubating him again we’d either end up with the exact same scenario or possibly loose him in the process (small chance, but a chance). Our other two avenues at that point where to move forward with a trach, to permanently fix the upper airway problem, and then probably be going home permanently attached to a ventilator. Essentially keeping him on life support to get him home, for however long. And the other more dark road for us being to look at what his quality of life at home being permanently hooked to life support would be like. Is that something he will tolerate? Will he still be able to enjoy the same things and in the same way? Will he seem even more bored than the attention I feel like already can't constantly give him? Will he start to have other things fail beyond this soon that will keep him uncomfy or create more pain? Asking yourself these questions and racking your brain for answers sucks and is not easy. Finding a way to answer them somehow to decide if now is the time to accept that something life sustaining is something it's time to give in to? Staring at these petrifying roads and decisions to make was just so shell shocking and completely numbed us. There have been a lot of tears in the last week. Not having that specific diagnosis, or even an idea of his disease/disorder being progressive, stable, etc., makes those kinds of decisions is near impossible. I HATE how much we’ve had to talk about the possibility of losing our precious child.
Ultimately, we did decide, after another care conference this Tuesday, to try extubation one more time. We knew there was a risk involved in losing him, as the docs had told us that re-intubating him the last time was much harder than the times before. It was a small risk, but decided we needed to take it. We wanted to try again, with the expectation that everything would go the exact same. But this time, we’d all be ready for it and we’d have certain people in place like his pulmonologist and head ICU doc (that I chose) watching and interpreting more precisely what is and isn’t happening. That way we’d have more precise answers, eliminate as much doubt as possible, to allow us to even attempt to make an educated decision on his care; not that we thought either road we were facing at the time was the right one. And if he actually fell into that small risk we took of losing him in that moment, decided that was our answer right there, that God chose that this was really his specific time, and it’s just not going to be in our control.
Neither of us are religious, even though we’re both baptized Catholics. I don’t refer to God much, but I do pray from time to time; especially for my grandparents, my Uncle Den and Jerry to watch over my little boy if he ever does have to leave us. So I feel comfortable enough to say, BY THE GRACE OF GOD, he did the exact opposite as we all expected during this extubation. He KICKED ASS. He said “screw you guys, I got this, I’m stronger than you think”. He didn’t obstruct in his upper airway, he tried to get those big breaths, even though having tape peeled off your face and a tube pulled out of your throat does not by any means feel good, so he wasn’t a happy camper, but he fought with everything he had. I don’t think I’ve had happy tears in a while. He has successfully been on bipap for (by the time I get this published) about 33 hours, with no signs of tuckering out. Heart rates have remained normal and comfortable, labs look great, x-rays look the same, sounding well, etc. He’s not thrilled to have a mask over his nose and look like a Heffalump (Winnie the Pooh reference), but he’s tolerating anyway. He’s moving his arms and legs a lot more, more like his normal self. He’s alert and aware appropriately, have even started going down on his sedative. And he’s slowly starting to find his voice again. I’m sure his throat is very sore, but he’s not complaining about it, and is starting to get very quiet sounds out. Almost six weeks without hearing his voice, I’ll take even a little whine right now!!
We do have a small update in regards to his overall condition. It’s nothing that is utterly helpful in the day to day situation at the moment, but something for us to keep in mind and try and wrap our heads around it, and learn how to deal with and accept. Something new to make us numb now for a while. Vlcek is back from his conference in Amsterdam where he was actually able to sit down with the top dog over there, on white matter diseases. He was able to talk with her about Riley, go over his MRIs with her and get her thoughts and opinions. Well, even the top dog can’t say, “he has this”. Which means, whatever his disease, it’s THAT RARE. SOB right? Should we really be surprised? Of course that’s the case. Looking at the changes in his MRIs over the past (almost) two years, she is confident that he does have a progressive disease. She can’t say for certain that it’s a specific white matter or gray matter disease; too hard to tell in his MRIs as of now regarding one or the other, just that it’s progressing. Of course not having a clinical gene mutation/deletion/change come back in his whole exome sequencing doesn’t help either. So now our geneticist does have the raw data and is starting to go through it all looking for red flags, specifically anything related to the brain. She came in this morning, and got our permission to send off the data to another doc she knows in DC, that specializes in leukodystrophies and rare cases. So hopefully with reaching out for help from other docs, and our own, we might find some sort of information regarding another child that has the same thing going on. Then that could lead to a specific diagnosis, and prognosis, and becomes a clinical thing, rather being in the research level. But all that takes TIME. And now knowing “Riley disease” is progressive, we don’t know what kind of time we have. For all we know it’s 30 years (God hoping), or a few more, or another six months.
It's hard to explain, but I’ve always had this terrifying gut feeling that it would specifically be his breathing system that would fail him one day, and that’s how we lose him. Now maybe that’s just because that’s been a big player in all his hospital trips, and I’ve seen a lot of bad happen first hand with it all. But he’s also had significant and life threatening seizures, but it’s always been just this gut feeling. We’re still holding our breath a bit on how well he seems to be doing at the moment. I know he’s an incredible stubborn and strong little boy, but I always worry about the control he continues to have or starts to loose with that. The hope and goal is now that we can slowly make changes down on his bipap settings, eventually give him little breaks, etc. And ideally, be able to bring him home at some point, with a clean bare cute face as normal, during the days. Then at night, he sleeps hooked up to the bipap to help his lungs and breathing system recharge from the day’s weakness. I will be jumping up and down if we can do that. Because compared to trach with home vent or the quality of life questions with that and his course, it’s beyond what we thought was possible. Once again, things turn on a dime with this kid, so we will be holding our breath for a while, but at least we’ve taken small steps down a much better road with a brighter future.
Now with knowing that his course is progressive, knowing he won’t get tons better and tons stronger; knowing that the likelihood of him walking, being able to eat normally again, etc. is pretty low. Just means I’m going to become even more of that annoying mom to doctors, and guess what I don’t give a shit. I’m going to do everything in my power to keep my child alive and with us as long as possible. And in that mind set, keep reminding ourselves what that means for him, and make the best decisions FOR HIM. I don’t expect doctors to be able to hand us something on a silver platter with he has “this many of days”, or “this many of years” left. But as things happen for him, like this hospital trip, his baseline level will probably be chipped away at, and never be back up to where it has been. It’s been chipped downwards in his regression of movements and strength in his muscles and he got a new baseline. Then it got chipped downwards with seizures. Then further down with losing ability to eat normally. And further down with each respiratory infection and hospitalization. Now it’s being chipped even further down with this visit, to an even lower baseline, which means an even lower threshold of reserves to be able to help when things threaten him. So as we see the light at the end of the tunnel this time, just means that the next respiratory problem, could be a MUCH bigger problem than we’re use to or ready for; just like this one has been.
Can’t even begin to explain how much I want to swear, yell, cry, etc. right now. I think the numbness will stick around for some time, but seeing my kid smile and knowing I might get him home in a way he’ll enjoy, gives me that hope I need.
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