The past couple weeks, we've been thinking about a lot of different things. Since Emma is getting to be a big kid (all the way to ten years old!), and since this is her life, we feel like it makes sense to include her in the decision making process as to what we choose to do for her medically. While we are still the ones making the decisions, we are pretty much following her desires. We are offering her as much information & support that we can, and trying to ease the burden of all the new information for her. It's a strange combination of goals because they contradict each other. How can we ease her burdens by telling her she's going to die without a bone marrow transplant?
Part of me wonders if it would be better for us to just make the decisions without asking her, since it would be easier. Or would it really? It would let her remain in her little kid bliss of not knowing the terrible reality of the disease she was born with. But, if we did that, and if we chose to transplant her, we know she would be very resistant to the idea. I think she would fight the whole process unless she understood why it was needed so badly. She associates bone marrow transplants with death, and it's a terrifying idea to her. She's made it very clear, many times, how against she is of ever having one. But, if we explain the situation, let her understand the choices & the consequences of those choices, then encourage her to tell us what she feels is best (with us giving her guidance and opinions along the way), and what she is willing to do or not, then we feel like it'll actually give us the best chance of success...if she is fully on board with whatever decision is made.
But, in doing this, it means we've had to explain the reality to her, which I knew would come one day. In my mind, I always imagined she would ask little questions here and there, and we would answer, and she would eventually learn about FA without us having to "spill the beans" to her without her asking. But, she hasn't asked as many questions as we thought she would. So, I've had a few different talks with her where I explain things that she never knew or thought to ask (which I'll explain in a bit).
Previous to her getting sick a few weeks ago, we had actually decided several years ago that we weren't going to transplant Emma, unless we had a sibling donor. It wasn't a simple decision to make, and it wasn't one we made in a way of giving up. It wasn't meant to shorten her life at all. (Transplant does not heal kids of FA. They still have FA and it's still fatal, even after a transplant. If successful, it does prolong their lives, though.) We made this decision because we prayed a lot about it and felt very clearly to not transplant her. We felt like by not transplanting, we were actually giving her the chance of a longer life because we felt convinced she wouldn't survive a transplant. So, instead, by trying androgens, then eventually blood transfusions/gcsf injections, we felt that could give her several more years than dying from a transplant would.
We also kept in mind that maybe it was just wrong timing. Maybe it was wrong at the time, but with medical advances, later on, it could be the right decision.
This is where we get a lot of different responses from people that we tell this to, and it's part of the reason we don't really publicize this a lot. Most think that if you have faith, there are no options. You transplant and trust God to heal her through it - that's where you apply your faith. You never consider not transplanting.
But, what if you pray, using faith, wanting transplant to be the right answer, only to feel incredibly strongly that transplant is the wrong choice. What do you do, then, when you don't feel like transplant is the one-size-fits-all answer that it seems to be for most everyone else? Do you still transplant because everyone else says it's the right thing to do, or do you listen to the answer you know deep down, and follow it? We chose to follow it and still feel confident in that choice. It was actually really hard to accept that we were feeling the way we did about it. I actually tried for a couple years to make it feel like transplant was right, but I couldn't do it. We didn't want to accept that we shouldn't transplant.
Despite some opposition, luckily, we received support from the people we needed to feel it from the most (thank you, if you've known this, and supported us!). We felt confident enough in our decision to not transplant that we didn't worry too much over what other people thought for the most part. I think God answers prayers in different ways. What is right for one is not always right for someone else. Choosing to go against all the current recommendations and popular choices took a great deal of faith and trust in God to follow what we knew in our hearts to be the right choice, at the time, for her.
Had we not made the decision to not transplant, we would have transplanted her about three years ago when she first went into severe bone marrow failure and required blood transfusions due to such critically low blood counts. Transplants were done differently back then, just a few years ago. Even though there is no way to prove this, we feel confident that it would have been a horrible choice for us to transplant Emma then. I can't really explain it beyond that, except to say that it was something Tyler and I felt so strongly inside...we simply knew. (Similar to how we knew we should have more children, but I'll share that story another time.)
Medical science has made advances since then. There are FA transplant centers that are no longer using radiation at all. These changes could have a huge impact on the success of transplant for Emma. Because of that, we have recently started looking into transplant again to see if maybe now it's time to prepare.
We're still in the middle of making our decision, but I will say that this time, I'm feeling a very different answer than I was before. I'll expound more once our decision is officially made, and we move forward with whatever is decided (because there's still the chance that things can change between now and then).
We've talked to enough FA adults to see how when parents avoided telling the truth (you want to save them from the knowledge so they can be happy easier, so I completely understand when parents don't tell), the children felt very angry later on when they discovered it themselves & felt lied to. We feel like honesty, when it's time, is the best decision. It's just a really sucky part of FA too! While it's a hard truth, she'll then know why we are considering a risky transplant, and she'll also know she can trust us to tell her the truth about things.
I asked her what she thought about having a bone marrow transplant. She yelled "no" at me and said she wasn't going to do it. She wanted to stay home. She didn't want to die like her friend Dianna did from it (we visited Dianna in the hospital and she died later on. It's something Emma still talks a lot about.). She told me she would rather just stay home and for things to be the way they are now.
Knowing it's a really touchy, and scary, subject for her, I tried reassuring her, with something along the lines of, "Emma, we will not make you have a bone marrow transplant if you don't want one. I promise. This is your body and it's your life. We will listen to you, and you don't have to worry that we'll make you do something you don't want to do. But, before you decide against it, I feel like you need to know a few more things."
She looked at me, slightly confused that there was more she didn't understand.
There was the moment of knowing I can never take back what I was about to say. This will change Emma's understanding of her life forever. It was really hard to make the words come out. I said something like, "Emma, if you don't transplant, you will get more and more sick. The androgens aren't working very well anymore. You will have to have more blood transfusions and more shots to make your body make white blood cells Those will work for a while. Eventually they'll stop working, and you won't have enough blood anymore. You have to have enough blood in order to live."
I had a hard time outright saying she would die from it, but she ended up asking what would happen when she ran out of blood, so I explained it - that she would eventually die from it (as she doesn't appear to be part of the lucky few who are able to avoid transplants like we were hoping), and then followed it up with hope by telling her how a bone marrow transplant would save her from that. She already knew she could die if she did have a bone marrow transplant, and she was shocked to realize she would likely eventually die if she didn't transplant. Both options are really sucky.
I didn't explain to her the full reality of FA...that there is no cure and that she will develop cancer later on. There's no need to share that right now, unless she asks. We need her focus to be on the current situation. We'll save her from the burden of that knowledge until later, until there's a reason to tell her.
She was silent for a few minutes, then her little tears started rolling down her cheeks. I gave her a hug and held her for a while, both of us crying, while Violet sat there saying, "Why are you crying? Why is she sad? Why is she crying? Why are you guys crying? Why are you sad?" haha.
I realized we'd never talked a whole lot about transplant (there was no reason since we weren't doing it), and her main experience with it was her friend, Dianna. I told her about all the kids that I knew who did survive transplant. I explained the statistics to her and that they've gotten better since Dianna died. We then started talking a lot about our faith and trusting God, the power of prayer, and listening to those answers. I told her that it's hard to decide, but I knew that God would answer those prayers for us. I told her that God is with us and will be with her. I can't tell you how deeply I believe that.
One thing little Miss Emma has is deep faith. She never seems to question anything about God. She knows He'll answer her prayers. She seemed to feel very comforted by that. Seeing how Emma reacted to it all, and seeing the strength of mind and faith that she showed, was really humbling...and amazed me.
She was upset for a little while, and has had bouts of it again, but overall she is still very happy like she was before. She sees the seriousness of the situation and is very interested in learning everything she can about it to help us make the right decision. She does look to us for our opinions and wants our guidance, of course. I told her that if she decides she doesn't want to make any of the decisions, we'll do it for her, and we can just explain our reasoning. But if she ever doesn't want something, she just needs to tell us. She's become very understanding of why we're talking about the things we are and she seems very trusting. She's actually seeming considerably more mature than she was prior to this.
Oddly enough, while it was a difficult conversation, it felt like a very spiritual experience, where God was guiding us and helping us talk about it. She really thought a lot about it. We looked up the hospital online and found some videos that she could watch to help her see what it would look like.
We have the benefit of some adults with FA who were able to share what it was like for them when their parents never cried in front of them (as a parent you don't want to burden your child). But it made them feel less willing to share those feelings with their parents. They didn't know how their parents felt, and they felt very alone with it.
So, I decided to share with her the story of her diagnosis. I cried through almost the whole thing and told her that we've known since she was just 11 days old that she was going to get sick. She used to have bone marrow like the rest of us, and we've watched her getting sick over time. I told her that sometimes I would go into my closet and cry by myself so that she wouldn't see me and wouldn't worry. She was very interested in all of it.
I think it was good for her to see that Tyler and I can emotionally relate to her fears. That if she is sad, we'll be there to hold her through it. That if she needs to cry, we'll cry with her. If she wants to ignore it sometimes, and just have fun and be happy, we'll do that too! We, as her parents, walk a fine line of making sure our emotions are not a burden to her...but that we still share them enough with her that we can share a deeper bond & understanding from it.
I believe it's possible to be happy, even when we're sad. Emotions are like onions, don't you think? There's so many different layers, and you can feel so many at once sometimes. Just sometimes some are a little stronger than others...ones closer to the surface.
I'll save the rest for another time, once we have a decision. There is a lot to consider.
I do feel a lot of change on the way. I feel a sense of hope, while simultaneously feeling like my memory is working a little harder at remembering all the little things...just in case things don't go as we hope. It's completely possible that in a year or two, Emma won't be here anymore. BUT, it's also completely possible that in 2-3 years she'll be much, much healthier than she is now. I like to hope and believe that that will be the reality. And, I know that if we keep close to the Lord, we will take the right path, and He will guide us through this.