I am always very honest when it comes to telling you all how Alex is doing. To continue with that, I have to explain a few things today. Please forgive me for not telling each of you individually, but this is the best I can do right now.
The last week has been sort of awful. On Monday I talked to Dr. LePichon and we increased one medication first thing each morning. Tuesday morning she woke up at 4:30am with congestion and I immediately gave her first meds and the rest of the day went well. That must have been a fluke, because Wednesday and Thursday mornings were awful again. By awful I mean that her dystonia (stiff muscles and spasms) was so severe that she was in pain and sort of panicking. She was wet with sweat and there was nothing I could do to comfort or help. I spoke with Dr. LePichon again yesterday and we had to make the decision to increase more medications in order to give Alex comfort.
We have now well passed any level of medications that are “okay”. By this I mean that she is on very large doses of lots of things. With the most current increases, we are toeing the line of enough versus too much. The risk of increasing these medications is making her too sleepy or that her breathing could be slowed and she may be unable to clear her own airway at all (which is even worse given her current congestion). We are aware of this line, but we must give Alex some level of comfort. We have always known this line existed and we went through this with Emma. The risks are known, but they are necessary.
Drew and I had been talking about some serious things over the last few days, feeling like God was preparing our hearts for whatever is ahead. When talking to Dr. LePichon yesterday, he asked me if we had considered where we want to be when it happens… home or hospital. Thankfully, Drew and I had already been discussing this and I felt comfortable saying that we prefer to be at home as long as we can keep Alex comfortable throughout. Hospice was discussed and it is an option, but I’m not ready to make that call just yet. Please understand that I am not giving any sort of time line here. Weeks? Months? Years? Your guess is as good as mine or any one else’s. We just don’t know. What we do know is that Alex’s PKAN has progressed to the point that everything is a delicate balance. We must be aware and prepared for whatever could happen.
Drew & I decided years ago, and still stand behind the decision, to not take any extraordinary measures. There will be no trach, no ventilator and no other machines to prolong life. When God is ready for her, we will not resuscitate.
I am sharing all of this with you all so that you may better understand what is happening. Thank you for your continued prayers and support as we do what is most important for our family and for Alex. We haven’t been out with Alex in more than a month anyway, but now that is a definite. We can’t get her in the car seat anymore and the only reason I can think that we would risk transporting her (and sacrificing her comfort) is to get her to a hospital or doctor in an emergency. Our absolute priority now is Alex, her comfort and her quality of life. At this point, quality of life means snuggles with Mommy & Daddy, enjoying our time together at home and visits from friends & family. Let me be clear… she is awake and she is even smiling. She is not sedated or out of the loop. Our plans from here on out include the proper medications to keep her from being in pain, lots of snuggles, licks from the dogs, our favorite television shows, reading books, and being together. Drew is still going to work each day, I continue to work in trips to the gym and life is as ‘normal’ as it has ever been. The difference is that we are acutely aware of the fragility of life.
Alex slept until 10 this morning and I immediately gave her first medications. While I can’t say that she is relaxed, she is not nearly as uncomfortable as she has been. She isn’t sweating or panicking and that is good. She is as wiggly as ever, but I keep joking her about having ants in her pants and she thinks that is funny.
Maybe I should share one more thing. We have not specifically had a conversation with Alex about death, but that is not to say that she is unaware. She very clearly understands that Dr. LePichon helps us make her feel better when she is hurting and that it’s okay to get frustrated when things are hard. She knows that Emma and other loved ones who have died are in heaven. I know she understands this because when I mentioned it yesterday she used her computer to tell me, “Jesus”. We often read Just In Case You Ever Wonder by Max Lucado, which is a children’s book about heaven. It’s a lovely book and it isn’t at all restricted to a child like Alex. If you have little ones who might need help understanding then I highly recommend it. I really don’t believe she is afraid. I don’t know how she does it, but sometimes it feels like she is the one comforting us. I truly and honestly believe that she has, and has always had, an understanding that surpasses reason.
So, as we always have, we are simply adjusting our definition of “normal” and living one day at a time. Are we scared? Absolutely. Do we sit around crying and wallowing in sadness? Nope. Is there anything that you all can do? Yes. Pray. Pray for Alex’s comfort and for God’s peace to be with us and all who love Alex. Be understanding of our limitations and priorities, please. We are at home and that is just a fact. Alex is here and so will we be. You are welcome to visit. Thank you all for your prayers and understanding and know how much we appreciate and love you all.