Family over everything

 I don’t even know how to explain the tidal waves of emotions that our family has gone through in the last week and a half. Nothing can prepare you for discussing ‘end of life’ plans. Although we know where Bridget’s syndrome will take us, having clear signs that you are nearing the end is absolutely soul crushing.

Here we are. We have had conversations for years about “what if’s” and “when’s”. Having a child with complex medical needs requires you to constantly be questioning and examining every moment of your child’s care. We noticed not too long ago that Bridget was becoming agitated toward the end of her day. We attributed it to another one of Bridget’s quirks. As I paid closer attention, we noticed that Bridget’s body seemed to be struggling to digest the formula we were putting into her. In turn, her g-tube started to leak. Like not leak a tiny bit, but the formula and medicine put into her belly was fully pouring out. 

Being relatively new to the g-tube world, I reached out to Bridget’s hospice nurse to see what she thought. Long story short, it was good that we paid such close attention. All of the odd behaviors that we noticed tied together…leading to a feeding intolerance. This is very common for Sanfilippo kids, I just couldn’t fathom that we had already reached this stage. We had a couple of options to try: go back for surgery to place a j-tube (a different placement in her tummy for her feeds) or give her stomach a period of rest and try to ease her tummy back into being able to accept food. We were also told that doing these “tests” to attempt to get food into her body might not be successful...and that would mean that there would be very heavy decisions that we would have to make.

I am glad that her hospice team has been with us every step of the way to try EVERY POSSIBLE strategy to help stabilize Bridget. We tried giving her tummy a break before digging in to see what was causing these used. We then tried to go back to doing bolus feeds through her gravity bags (which is what had previously been working). We quickly found out that was causing Bridget pain. Food (food) was literally the problem. Her stomach is no longer able to take on as many calories or formula anymore, causing her to be in pain. And when I say pain, I mean throwing her body all over the place and bawling, which she rarely does. 

If you have known Bridget since before her diagnosis-you know that food was her LIFE! We swear that she had some of the QUICKEST HANDS OUT THERE. We have several memories of her swiping food off of random strangers plates while attempting to take her out to eat. So it’s really just a punch in the gut that something she really enjoyed (one of the very few things), is the thing that is causing her the most pain. 

We were told the gravity bags (or quicker feeds) just wouldn’t work for her any longer. The last 10 months have been so consistent in her feeds, but we had to consider other options moving forward. The next option was to move to a feeding pump that she is hooked up to. When we tried a “typical rate” with the pump, Bridget was writhing in pain yet again. We have been going back and forth adjusting her doses and rates with Pedialyte. When we finally figured out a low rate that worked, we tried that for a couple of days. 

Just yesterday we were finally able to introduce formula back into her system. She tolerates it pretty well, and now today we had her back to trying formula at an incredibly slow and long rate. If she can handle this new method of feeding today and tomorrow then she will hopefully be able to go back to school. 

The biggest shock to the system is realizing that her stomach is really beginning to completely shut down. The process is much further along than we were previously aware of. I do realize that this new strategy that we are working with is not going to solve or fix anything. I have to keep reminding myself that there is still no cure. We never in a million years would want to inflict any pain on Bridget. We are at the point with her journey, though, that we really have to listen to what her body is telling us. As awful as it is to actually come out and say this….Bridget is at the lowest possible rate/dose that someone of her weight and size can be and continue to carry on living. I’ve been told several times about how this is just a “bandaid”. It’s difficult to be in a scenario where you want to be so happy about finding a quick fix, yet not knowing how long that quick fix will even last. 

When we were at the lowest point this last week, the hospice team was at our house and we had Bridget’s doctor on the phone. He politely, respectfully, and gently told me that if this doesn’t work (moving to the pump and her being able to keep in a specific volume), that we were at the end with no other options moving forward. I know that I have made myself physically ill with this news. It’s devastating. I would be lying if I said I was ok right now. I am trying to really just take in every snuggle that I can with Bridget as we continue to monitor her. 

Many have asked me how we will know if her stomach isn’t tolerating her formula moving forward. Essentially, we have to watch for discomfort, a distended belly, or for Bridget choking on her formula. Aspiration is a huge concern, and to be honest has kept me awake with nightmares several nights in a row this week. 

Along with all of these tragic events occurring, I’m also struggling with the fact that I have two ‘typical’ children that also need love, attention, and affection. I feel like I’m pouring from an empty cup, it isn’t like there is a parenting manual for parents dealing with anticipatory grief and juggling ‘typical’ parenting at the same time. 

I know many people were asking for an update…and there it is. Unfortunately, that isn’t the news I was hoping to share. But Bridget’s team and I have been discussing trying to live only in the current moment, and taking any tiny wins and designating them as BIG wins for her instead. We know our little girl is loved by many, and we are so grateful to everyone for thinking of her during this time.