Hello
I was thinking the other day how interesting it is that our lives are full of stories. Have you noticed it, too? From casual conversations to books to movies to religion to everything. Stories are everywhere. I think it's because they're important, and we're meant to tell them. So, this is ours.
Our Story
If you've never met us, we are a homeschooling family of seven. Two parents, four girls, and one boy. We also have two boxers, and three geckos.
Our oldest daughter, Emma, was born with a rare, incurable disease called, Fanconi Anemia (FA). It causes bone marrow failure and different types of cancers. There are treatments to extend life, but so far there is no cure. Most live into young adulthood.
During her lifetime, she's gone through a lot. When she was born, the blood work started and has never stopped. It ranges from every couple months to multiple times a day, depending on the situation. Before her transplant, she had open-heart surgery, regular bone marrow biopsies/aspirations, years of bone marrow failure, blood & platelet transfusions, and a few years of androgen therapy (in an attempt to avoid transplant altogether). Then when we ran out of options, I took her from Texas to Cincinnati, almost 1000 miles away, in an attempt to find someplace that would be able to perform a successful transplant on someone with her illness (as it makes things much more difficult). We were there for several months, separated from the rest of the family.
Post transplant, she developed a virus-driven lymphoma that was caused by the EBV virus waking up. On paper, it's Burkett's Lymphoma, but because of what caused it, it's called PTLD. It's scary when you transplant because of things like this. Those viruses, that we get throughout our lives, stick around, and when our immune systems are weak enough, or destroyed, it allows them to wake up and run around unchecked and behave in ways that they normally never would. She had multiple other viruses wake up, too, and cause problems, but this one was the most serious.
We had to fly her from Cincinnati to NYC for an experimental treatment at Sloan Kettering to save her from it. Unfortunately, the PTLD caused more harm that we realized. It took her six years to do what most kids do in one year, when it comes to her immune system returning.
So, during most of the years after her transplant, her immune system was equivalent to someone with AIDS. She didn't have those helper t-cells like she needed to make her immune system function properly. Initially, we were told we would have to isolate for a year post-transplant, but because of this, that continued on, seemingly forever, for six years (to varying degrees). The restrictions that they had her under, never fully came off, until six months before the pandemic hit, when she was finally given the green light to return to life as normal. I can't describe how it felt to have freedom for so short a time.
Currently, she is dealing with GvHD in several areas. (GvHD is short for "graft-vs-host disease." It's where the new bone marrow attacks the body.) It can be mild to life-threatening. Emma has GvHD of the eyes, causing limbal stem cell deficiency, which can lead to blindness. She also has GvHD of the hair follicles, causing her hair to fall out multiple times. Then she also has GvHD of the nose, mouth, and possibly lungs and liver. So far, it's all mild (excluding eyes), or, at least, not life-threatening, and they've started her on Jakafi to try to calm things.
Recently, she's lost the ability to eat. Her stomach and intestines stopped working properly for no known reason. If she eats, food enters her stomach and stays there for days, causing her to starve. She has been given a GJ tube to give us a way to feed her formula, bypassing her stomach, and putting it directly into her intestines. Then we give medications to try to make her intestines work better. Due to how slow things move, this can, at times, still not be enough.
Alongside all of this, she has also been dealing with a throat that feels like it's collapsing at times, as well as Parkinson's/Tourette's type movements (see video here). As I write this (Jan 2022), in the last two months, she has been admitted three times for a total of a month, as they try to figure out what is going on.
During this last stay, she was diagnosed with mild Chiari Malformation, syringomyelia, tethered cord, scoliosis, and a couple fused vertebrae. We are currently waiting to meet with the neurosurgeon to see if he thinks she needs surgery.
Her siblings have had a unique journey in life because of all of this. They never got to experience what most would consider to be a normal childhood. When I think back to when I was a little girl, I lived in this false reality where it seemed like these types of things only happened to other people. And I've noticed my children never had that false reality. Instead, when they were little, they were watching blood transfusions, hospitalizations, experiencing their sister be too weak to play with them. It was normal for them to have to go get her toys, sit close to her, and help her play with them when she was too weak to play with them herself.
While it was normal to them, it wasn't easy. They got to experience all of those big feelings at such young ages. They saw her fear and pain, along with their fear, and saw that they could do things that she couldn't. They had to give her hugs goodbye, knowing they might not see her again when she had to leave for months for her transplant. They've had to give her hugs goodbye multiple times. They had to give up their mom as well, never quite knowing what was happening or how long it would last. It hurt them in a lot of different ways. Not just transplant but all of the things that have happened before and after.
I've also seen that they've learned empathy and the importance of a support network. The importance of a hug and of listening when someone is crying and hurting. I see how quickly they respond to the pain of others with such kindness and support. It's been difficult, as a mother, to see that a lot of their childhood has involved sacrifice and having to think of others (more than I would have ever wanted them to experience) and of trying to not bring illness to her. They have given so much, and, as of yet, they each have done it without hesitation. I have been in awe of them.
But, at the same time, we've been acutely aware of the fact that each of our children need our attention, love, and support as individuals. We have to make sure they develop healthy and strong, too, and that their own dreams and desires matter just as much to us and is a priority to us as well. They're just as loved and important to us, too. So, throughout the years, and while we see how much is being asked of them at times, we work really hard to focus on each of them. To make their needs our priorities, too, and to make sure to help them through their own struggles, connect with them one on one, and try to make sure they know how important and loved they are, too. Our story here is just as much about them as it is about Emma.
Sometimes our journey through all of this has felt like we're endlessly climbing a steep cliff face, being blown around and slammed against the rocks. Where some days we feel so exhausted that we don't know where to find the energy to take the next step forward to find that safe place. Sometimes we wonder if there really is a safe place ahead, or if this is simply our path of life, where we're always going to be crossing difficult terrain.
We've come to the realization that we can't wait for things to be better in order to enjoy life and be happy. Like, we can't have this perpetual feeling of, "once she's over this hurtle, then we can be happy," because we realize, if we wait for that, we'll run out of time. Instead, we've had to learn to dance in the rain. And, if there's any purpose I can give to anything that we have, or are, going through, I want to find it, and I want to share it. I want all of this to matter in some way.
