(Oops, I accidentally posted this before I was done with it...) Hello again! How is everyone? I know it’s been a while, but we’re still here.
I’ve actually tried to write many, many times, but writing has been really difficult this year. There's been too much to say. (Like 60 pages in, and I’m still writing, type of stuff to say! But writing is my therapy, so, clearly, it's been a lot to work through.) So, it's taken a lot of time to whittle all of that down into this.
Basically, last year was a hard one. The hardest we’ve had. And it’s been quite a road to get to where we are now, today. But we did get here, and I want to tell you about it.
FANCONI ANEMIA
In order to do that, though, I need you to know about Emma's overarching illness. And how, as we’ve gone through each of these things, throughout her life, and as we continue to go through them, it's always there, either causing the issues or looming over it all.
And this came into play pretty strongly last year. It's a huge part of what made it so hard.
So, if you don't know, Emma was diagnosed with a rare genetic disorder, called Fanconi anemia, and it more or less breaks down into four main phases:
Healthy Phase (often early childhood)
Life-threatening Blood Issues Phase. (Often mid-childhood)
Return to Health “Bucket list" Phase (often late-childhood/teens)
Cancer/life expectancy Phase. (20-30 years old)
Emma went through Phase 1 and 2. So, Phase 3 was our next phase. The one we starting calling the Bucket List one. And when a person enters this phase, it means they’re experiencing a return to health, a return to freedom, and a return to the ability to live life more fully, without such extreme risk, and without consuming health conditions, all while also knowing that phase 4 is coming.
And that combination of perspectives generates an energy. It's both a place of freedom (having health back) but also a place of feeling a lack of control (the looming future).
So, how do you satisfy all of that and find as much peace as possible with it?
I don't really know. But, for us, seeing it more as a "bucket list" phase, where you actively live life, consciously treasuring the time, consciously doing some of the things you want to do while you're here in this phase, seems a pretty good way to do that.
So this has been our plan:
- Make a bucket list.
- Go do all the things.
- Make memories with those you love.
This way, no matter what happens, there's at least the comfort of knowing that the living was lived. That this time was appreciated. And hopefully that there will be no regrets.
EMMA’S PHASES
So, you’ve seen Emma go through Phase 1 and 2. And once Phase 2 arrived, we wanted to get her to Phase 3 as quickly as possible.
But that didn't happen. We dealt with bone marrow failure, transfusions, three years of androgens, bone marrow transplant, years of infusions, a virus-driven lymphoma, a brain bleed, lungs filling with fluid, experimental t-cell therapy, virus-driven kidney disorder, menopause at age 12, an eye disease, an abdominal mass...
But the thing that took the longest was the six years of doctor-ordered restrictions, that varied as time went on (and that we followed to varying degrees, too, during those last years) due to basically trading one blood disorder, aplastic anemia, out for another, an AIDS-like immune system to varying degrees (same type of immune dysfunction, just different cause).
Restrictions aside, simply dealing with having a child whose immune system couldn't protect her properly, while also trying to make sure she got to live the life she has, and experience life, with that type of risk against her, was exhausting. Like, think of how exhausting a simple test at school can be (like anatomy & physiology). It's all the things you have to think about and watch out for and keep in mind, and the statistical high risk on her life, the emotional landscape that this creates, and for it to last for so long...
We spent nine years in Phase 2 in order to get to Phase 3. And when time already feels like your enemy, that's a punch to the gut to have so much taken away for that.
So, when we got to Phase 3, we took off like it was the start to a race. We didn't have time to waste. Phase 4 was way too close.
Only, that would only last for six months, because the pandemic started, and all the restrictions were suddenly back.
But pandemics, thankfully, don't last forever, so Phase 3 was still on the way, just delayed, yet again. But we felt very anxious because we were creeping ever closer to Phase 4, and we were scared we wouldn't have time. So, we got an RV and tried to bypass everything that way, going on trips and making memories.
PHASE 3
So, that's a huge, huge summary of where we were when this GI condition hit, seemingly out of the blue.
But, looking back, it was a few weeks after she picked up an illness, one that, months later, she would say she lost her sense of taste/smell from (she still hasn't regained it), which makes us wonder if it was the catalyst. Previous illnesses had caused severe bone marrow failure, forced her to transplant, turned into a lymphoma, attacked her kidneys...so it wouldn't be far-fetched to think it could have triggered this, too.
But it was a condition that took away her ability to eat (she got about 300-800 calories a day) and her days were consumed with trying to accomplish the basics of the basic. Despite getting a feeding tube, starting multiple medications, being hospitalized six times, nothing was fixing this.
That meant, when looking at her life as a whole, the last time Emma was healthy, she was 7 years old. And that other than a six month break, once, nine years in, that was it.
Phase 3, the Bucket List Phase, the thing she's been chasing all of this time?
It disappeared, and this, instead, took it's place.
MARATHON
Imagine if you decided to run a marathon, and, at the end of the race, you crossed the finish line, just to discover it was the starting line of a new race.
That’s not supposed to happen. You're supposed to get to rest…
Only you can’t. Because they released wild animals that are chasing you. And if you run, you're probably safe. If not, you won't be.
So, you get to the next finish line, thinking it’ll be over, just to discover that it’s the starting line of another new race.
And you have to keep running.
And it keeps going like this.
You see people around you, other runners. Some end up getting attacked, despite running. And some finally ran far enough that they can stop running. So, you believe you’ll get to stop, too.
Only, for some reason, every time you cross a finish line, it's the starting line to a new race, and it does it a ridiculous amount of times...
That is how this--her life--has felt.
We were already so exhausted from everything else.
But then this GI condition blew in like a tornado, and it changed everything.
So, when I say last year was the hardest, it’s because it came in at the end of so much more. And, together, it's all turned into one big experience.
We were already running what we knew had to be the last marathon. At least for a while. It was like all the signs were up saying, “This finish line is for realsies this time!" just to cross it and see that it was a new race again.
But all the signs were different with this race. They said there was no finish line, there never would be, and, there never was going to be.
And it turns out, it feels really different running when there is no final destination.
Just effort.
Just exhaustion.
And to know that it’ll be “just forever.”
At least, until Phase 4 arrives.
And this realization, while sitting in this place, looking at her life, seeing how it went so different than we ever imagined it would, was devastating.
It felt like a thief.
This can't be the life we give her...
DARK NIGHT OF THE SOUL
When this GI condition hit, a tsunami of grief hit alongside of it.
And, on top of it, she was physically starving, no matter how hard she tried to eat enough. And she tried so hard, refusing to give up the ability. Because she was a little foodie. Food was a happy place for her. So, to lose that, after everything else, was too much.
And when she looked back at her life and looked forward to the future, mentally and emotionally, she fell apart. It's not like this hit her when she was in her 30's, when she'd gotten to live at least a little bit of her life. The last time she'd experienced health, plus freedom, plus having closer to normal risks against her life, was back when she was 7. And all this time, between now and then, she didn't know that type of freedom wasn't coming, not until then, not until those moments, not until she suffered, and not until there was no relief from it.
And I don’t mean that she gave up. I mean that this came in and blew her to pieces.
It changed what the past meant. And it changed the future, too.
And the “dark night of the soul” came to her. The one that comes when everything we thought we were, everything we wanted to be, everything we thought life was, falls apart.
For a while, it’s like you sit in this hallway. You’re not who you were and you’re not yet who you’ll become. You just are. Only, it’s incredibly painful. It’s the ripping away and tearing apart of hopes and dreams and things so dear and close to you. It’s the losing of who you thought you were. And, for a while, it’s the experience, mainly, of things being taken away. But then, eventually, it settles. And it’s the slow and careful process of rebuilding. Often it takes years.
So, Emma was very fragile during this time for many reasons.
Her nights were full of nightmares, and she struggled to be alone.
She also spent a lot of time talking, every day, often for hours, often crying, about everything, working through it.
But how do you work through this?
COMFORT
We know that, at times, physically, people need time and help to heal from severe injuries, like shattered legs or burns. Their physical needs are so high that they need a caretaker with them, helping them.
I believe this can happen mentally, too. Where everything can fall apart to such a degree that having someone nearby and right there to help, to be a caretaker to the mind, just like to a broken body, for a while, can help tremendously in fully processing what’s going on and healing from it all.
Because if emotions could bleed, she was hemorrhaging.
If emotions were a physical injury, she had full body burns.
And, for a long time, it was like Emma was trapped at the bottom of a dark pit, and one of us always stayed with her, essentially filing the role of sitting at the top, calling down to her, shining a light into the darkness, reassuring her that she wasn’t alone.
All the hopes and dreams, things we’ve been holding onto, simple things, like going to Disney World, the promise we gave her during transplant—something she never got to do—all of it, and everything else, was gone.
While we had finally finished the one race--the transplant one--physically she was in such high need, and incapable of doing the basics, that it didn’t really make any difference. She could barely leave the house.
GETTING HELP
Imagine if your loved one fell off a cliff, and you called in rescuers to save her, but the rescuer that came, said nothing was wrong, refused to even lower food down to her (aka TPN), and told her that if she wanted it, she needed to climb up and get it. Then turned and walked away.
Because that was the type of help we dealt with once they called in the "big guns."
And while there are way more details to this, that type of medical neglect and medical abuse was a life-changing experience for all of us.
In a really bad way.
He became part of why she continued to suffer. And we saw how a person like him worked in an environment of power, like that, and we saw that nothing seemed to be able to stop him, despite others seeing it.
And when you are part of a society where you have seen families lose their children due to medical mistakes, and when we've been doing this for 20 years and have a lot to compare to when it comes to understanding what's normal, and seeing how wrong all of this was, it was a terrifying experience. Especially how this played out, where so much didn't make sense until the last hospital stay. But it was an experience that held on, even after we left. That said, "Hey, if this doctor can do this, another one can, too." And when you never want to experience something like that again, it makes it very difficult to risk stepping into a doctor's office again. It's been really difficult to find feelings of safety again. Luckily, it's not like she has a life-threatening illness that requires the help of doctors to keep her alive.
Oh, wait.
She does.
But to have to watch the look in her eyes change, as she understood what was going on, and as she began to accept the reality that she might die. And not because she should. But because they were going to let her die.
It was like it set a fire inside of me.
This isn't going to be how her life goes.
But I had no idea what to do.
It’s been one of the hardest things for me, personally, to ever go through. Specifically dealing with GI #2, the power he had over others, the havoc he wreaked in her care, watching her be hurt through it, and then how to move on from that and find help.
And that’s all I’m going to say about this part because it’s a huge story, and it’s one that I've never fully told, and I can’t even sort of summarize it.
NEW DIRECTION
But it felt like we left there with everything in shambles, and there was so much to rebuild.
And while I could tell you about the amazing new GI doctor, the extensive time she spent, wanting every detail about what was going on, wanting all the history (which GI #2 never did, he hadn't even bothered to ask us what was going on), and, ultimately, being convinced that Emma had something wrong with her intestines, and she wanted to start retesting things…there’s no reason for me to write about all of that anymore because Emma decided she was done with testing.
They already had her on all the medication for dysmotility, so there wouldn’t be anything different that they would do with a diagnosis. And she felt like the safest thing to do, in efforts to avoid anything like happened before, happening again, was for them to go solely by her symptoms.
And as much as I wanted evidence to show others, to prove GI #2 was wrong, when I looked in her exhausted, puffy eyes, as we were walking away from that appointment, where she'd spent about 20 minutes sobbing when they'd asked if they could call GI #2 for him to explain her health situation, and seeing that reaction, while feeling my own panic at the idea, as well as knowing the life she's lived, with all these health issues, I felt like she was making a very good choice for her mental health. And in a snap, we were heading a new way.
Without the testing, though, there was nothing new for them to do.
And for a while, it felt like we were at a standstill.
She was terrified of the hospital. Both of us were. So, she was doing everything she could think of to avoid ever going back because it felt dangerous to her. She switched to only formula, in hopes that her body could handle that better, which it did.
It was a tough place to be. To get up every morning, pour formula in her bag and just let things continue like this, knowing that a proper investigation was never done.
But, for several months, that's what we did, and she began to finally get enough nutrition in and began to gain weight. Going completely liquid, with a healthy formula, was tremendously helpful.
And we sat there for a while, time ticking by, as it gnawed at me.
This couldn’t be the life we give her.
She didn't go through all of that, just to have this be her reward for it.
ABDOMINAL THERAPIST
Somewhere in there, Emma’s physical therapist, Aaron (who is amazing!), had a random thought. He wondered if their abdominal therapist, Paula, might be able to help Emma with her gut issues…
And it turns out she could.
She had Emma lie down, and she began to slowly feel along her intestines.
She said that intestines are supposed to be soft and move a certain way under her fingers, but Emma's were firmer and more resistant. She said they had dysmotility.
She also ended up pinpointing two spots that were extra troublesome, and they happened to be the same spots that Emma had tried telling the doctors about, who told her nothing was wrong. But here Paula was, having no idea about that, and she stumbled upon it herself.
And I cannot tell you how validating that was!
Her belief is that Emma has scar tissue in there, and that overtime, scar tissue grows like a spider web. So, for the next five months, Paula massaged Emma’s belly regularly. Trying to break up the scar tissue.
And, one day, when we went in, she said that Emma intestines felt bad everywhere. She was on a temporary higher dose of lactulose right then, which Paula said can mess with the intestines ability to move properly. So, we stopped using it (now using Calm/magnesium instead), and it helped.
SILVER FERN
A couple months into this, we began looking into products a microbiologist put together. Emma had been on antibiotics prophylactically for six years. When a 10-day course can do as much damage as it does to some people, what can six years do?
It seemed functional health-ish, but I’m not positive that it is. Palliative care approved the approach, so we began Emma on a whole new set of “medication.”
Their company was amazing. I reached out to them, explained the issues Emma was having and the medical history she has, and they told us what to.
We spent about 110 days in the first phase of treatment. This reduced inflammation, which she seemed to have a lot of because her system began to move more easily in response.
About two weeks in, that feeling of fullness she’s had, for about 18 months, began to noticeably lessen. She began to be able to handle her formula at much more normal speeds (versus running 24 hours a day).
We ended up, one by one, over time, being able to get her off of EES and mestinon, her motility medications. Leaving only Linzess (which she's still on). And considering the side effects they had, it was a huge boost in feeling better, as well.
Close to four months in, we switched to the second phase, which is using immunoglobulins, in pill form. It works like IVIG, only for the gut. And previous testing showed that she had a lot of bad bacteria in there. So, this goes in, attaches to the bad bacteria, and pulls it out. Then we try to help replenish with good bacteria with their probiotic. (We're still doing this, as well as doing the first phase, once a day, instead of three times a day.)
SOLID FOOD?
Seeing such drastic improvement, somewhere in there, we began to wonder if maybe she could eat solid food again.
We had tried this, repeatedly, previously, and it went poorly each time, so it made us very anxious.
We also took into account the fact that she had not eaten solid food for over a year, and her gut might be weaker now.
So, we started off with simple things, like, pho, no chicken, just noodles & broth, and she handled it fine. But it's usually a few days in, once her system is full, that the problems arise. Only, this time it never did.
Her system was moving!
For a while, we did just one meal a day, doing it with gentle food.
Then, it was a process to not only transition to more solid food, but to also do it for every meal. So, over time we did both of those things. And now she's eating three meals a day by mouth.
I think altogether, this was an 8-9 month process in actively trying to help her gut heal. We're still in the process of doing it.
It’s not back to working perfectly. She still struggles with certain foods (high fats, spices, and probably fiber), and feelings of fullness from them, but it’s not anywhere near hospitalization level type of struggles.
And she’s still on her feeding pump, but it's only to give her the water that she needs.
The progress, and change, has been tremendous. It's night and day. Her current issues, while definitely there, feel so minor compared to what they were before.
Emma eating out with us for the first time, as a family, once she could eat again. It was so fun! (Even if I did catch them in weird expressions, haha, you're welcome, Violet.)
RELIEF
When the monster of FA has chased her for so long, and when you see it take so many people you love over the years, seeing her regain this ability has put more of a buffer in place. It makes it feel like she's safer from it.
And I don’t know that I’ve ever felt so relieved.
Especially since we knew how important food was to her and how she's already had to give so much else up over the years. So, seeing her eat again has allowed that part of my brain to finally settle. It was that feeling of being able to pull her up over the cliff, and just hold her, so relieved she’s safe again.
And, often, I think about each of the unexpected people involved in accomplishing what we did. The people who came around and listened, who thought and came up with unique solutions. To take her from that to this? Something that we didn't think would be possible. They are our heroes.
Both of her palliative care doctors, now, (previous one and this one) have asked for all the information, because they want to use it to help their other GI patients.
RACES & PHASES
You know that race she's been running?
Well, it's like we finally crossed a finish line. One that didn’t turn into another race this time.
That means that Phase 3 reappeared, and we're in it now.
Only, we’re also in the range of Phase 4 starting. She’s 21 now.
So, it’s time to go enjoy and experience and treasure this time. Because we have no idea how long it’ll last. We have a bucket list to fulfill. Experiences we’ve been waiting for. Memories we want to have.
HELP?
Which leads me into this...
While we’re late to the holiday season, we’ve started a couple Etsy shops (that we’re still adding to), with the hope that one day they might grow into something big enough to help fund some of our adventures. And, if not, we'll figure it out other ways.
So, if you're looking for any stocking stuffers or gifts, please check both stores out! We'd love the help. Even just initially to increase sales and reviews. But, also, no pressure.
Store #1--Digital & Paper Products (use coupon code EMMA20)
Store #2--Home Decor (coupon code EMMA20)
And that is our update, my friends. Life feels so much more stable and safe than it did a year ago, and we hope that in another year, it'll be even better. But we're going to assume that this is the best that we get. And we're grateful for it. Very grateful! We hope you're all having a beautiful holiday season. And for those of you who are hurting after loss, we're thinking about you, too, and sending all the love we can.
