Friday, February 15, 2013

facebook



So, as of just a few hours ago, I started a Facebook page for our blog.  It's for those who want more "real time" updates (like if Emma is in the hospital) or for those who prefer Facebook over blogs.  I'll still be updating this blog, just as I always have.  So, if you hate FB, just stay here like usual, and it'll remain the same.

I realized back a few weeks ago when Emma was in the hospital that some people (friends on FB) were getting better updates than others were, and I'd really like to offer a page where anyone who wants to can follow along, while allowing us to keep our privacy.  (I think it might work even if you aren't on FB at all.  You just won't be notified of it like you would if you were signed up with FB.)

I have a hard time keeping everyone updated - thinking mostly of during more critical times when I only get a few seconds here or there to update a FB status and not enough time to update a blog entry.  So, hopefully this will help!  This will be a little more focused on just Emma for right now, but it will also include our other children.

You can go HERE and click "like".  If you want it to notify you (like a text message instead of just in your newsfeed), you can click and hold on "like" and choose "get notifications".

Thanks everyone.  :)

Saturday, February 9, 2013

decisions

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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.

So, first...

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.

Monday, February 4, 2013

cardiology

Emma had a cardiology appointment on Thursday (or some day last week that I can't remember).  It'd been a little while since her last appointment.  On the way there, she kept telling me she wasn't scared like she used to be and that it was so weird to her to not be scared.

Tyler met me at the hospital entrance.  He had left work early so he could take the kids via the van (he had already parked his car in the parking lot for me to take when we left).  We did a fast trade off, and Emma and I ran into the hospital because we were a little bit late.  He took the kids home.

We finally found the right room, and I was signing in when I realized I had no keys.  Tyler had both sets.  So, I called him to let him know I had no way to get home. It had maybe been ten minutes.  He made his way back over, in somewhat busy traffic.

Meanwhile, Ella puked...because that's what babies should do when their dads take the van.

We had to run back down five stories (or more like, take the elevator) and meet him out front to snag the keys.  On our way back, we had to slow down considerably because Emma was out of breath and very fatigued.  She was looking more pale than usual and needed a breather.  She told me she was having a hard time walking and needed to rest.

Just in case I ever forget she is in severe bone marrow failure, I just need to go on a little walk with her, and the effects (affects?) of it are obvious.  It made my heart hurt.

Here she is getting her echocardiogram (basically an ultrasound of her heart).



The guy here was really nice.  The whole time I didn't know if he was actually the doctor or if he was a tech guy or what.  The last two heart checkup appointments she's had here, the doctors did this part.  And...each time we came in, it was a different doctor (though, they ALL have the same last name), so I knew I might not recognize him.  I kept looking at him...wondering...is he the doctor or is he someone else?  Should I ask him?

I didn't.  Instead I got out my camera (yes, I brought it with me...of course!), and I asked if I could take a picture.  He smiled & laughed...and then I think he felt very awkward.  Okay, maybe not, I wasn't positive.  But I like to think and hope that he did because I find it amusing.

Oh, and my camera was set to take jpg images somehow.  How?!  I didn't change it!  I never let the kids touch the camera, but maybe one of them did.  I always take in RAW because you can edit them so much better.  So, it was frustrating to realize the file was wrong once I got home.  Oh well.  I think I'll survive.

Photo:  Here she is sticking her tongue out at me...  She also kept rolling her eyes at me when I would tease her.  She cracked me up.  It was awesome seeing her actually having fun and not being afraid.


Then we were taken into a different room where they did the EKG.  I didn't take a picture there, though.  That's where they stick like 10 sticky things to her chest and then there's like a squiggly line machine that does a reading of her heart.  I'm pretty sure that's the official medical explanation of it all...

Emma needed to lay on the table in there so they could stick the stickies on her.  She refused to lay down because she was "being funny" and sat with her legs up and arms around her legs.  In a teasing/challenging voice, she said, "You can't make me do it.  You can't tell me what to do!"

I've mentioned the steroids before, right?  They are to boost her bone marrow production, but they have also increased her strength (even though they aren't those kinds of steroids, it's still a side effect).  She is crazy strong for her size.  If she doesn't want to lay down, and if she's sitting in the fetal position, it takes a lot more than just pulling her arms off her legs and straightening her legs to get her to lay down flat like you can do with a typical child her size.  I simply cannot muscle her without putting forth a ridiculous amount of effort to pry her hands off and then getting her to lay down.  I'm honestly not sure I'm capable of doing it without putting forth enough force (perhaps some headlocks) that it would hurt her.

So, for a few minutes, I was trying to untangle her from herself, and she kept laughing at me, saying, "You can't make me do it!" and it was like we were a flurry of arms.  We were both being goofy.  It was funny, and the cause of much laughter.

But then she wouldn't stop, and she would not lay down.  At that point, I knew I had the option to do a sneak attack - maybe poke her in the eyes a couple times for a distraction.  Then maybe body slam her a few times until she came out of the fetal position.  Which then would let me pin her down on her back while yelling, "Okay!  I've got her down!  Hurry and attach the stupid sticker thingies to her chest and turn on the squiggly line machine before she overpowers me!"  But I'm pretty sure they would have frowned at me for doing that, so I didn't.

It's times like this where I need Tyler's man strength to help me with a tiny ten year old who is the size of a 6-7 year old.  haha.

Instead I stepped back, realizing that muscling her into it wasn't going to work.  It was time to get serious and try to make her choose to comply.  I always try to keep her feeling like she has a choice and isn't being forced into something (like how I say this after I mention how I was about to body slam her?).  The nurse lady was tiny.  I don't even know if she was 5ft tall.  Well, she was stockier than me, maybe she could have muscled Emma down...

So, anyway, we approached it differently and tried to get her to choose to lay down.  We must have talked her into it because Emma ended up laying down and doing it.  But the funny thing was she started meowing "No.  No.  No."  Just like a cat would if it meowed the word "no".  It cracked me up.

A minute later the lady was done.  It took faaaaaaaar longer getting her to lay down than the test itself took.  (After the nurse left, Emma and I had a chat about what was appropriate teasing and what wasn't.  And she needs to not resist so long in the name of humor.  She seemed to get it because she did great later on!)

Then we were moved into another room to see the doctor.  This room had cold air blowing in it.  It was freezing.  I gave Emma her shirt to wear over her little gown.

This is how serious she was being:

I like this picture.

The doctor finally came in and saw we were freezing and moved to another room.  He took out the blood pressure thing and took her blood pressure.  No machine, just him doing it the old fashioned way.  Then he took off her boots and socks and checked out her toes.  He kept checking her extremities, and at that point, I started feeling a really big sense of concern over things.

Never in the past had the doctors (and he was actually the doctor we saw the time before) been checking into things that had already been done and stuff like that.  He excused himself and then almost a feeling of panic set in.  I'd never thought she would have something wrong with her heart again, but what if she did.  He didn't look terribly happy.

He finally came back maybe ten minutes later.  He had apparently spent his time drawing me a pretty little picture of a heart.  Emma's left ventricle is dilated.  It was enlarged last time.  That's not due to her heart defect.  It's due to her anemia.  So, I wasn't too concerned about that.

Then he said that her aorta is mildly narrowing and it's twisted, like a hose.  He said that her blood pressure is just fine and she's getting blood out like needed, so he felt like she's just fine.  It's more turbulent in that area, though, too.  He does want her back in a couple years to check her out.  If we choose to do a bone marrow transplant before then, he wants her back before then so he can take a peak at it again.  He said that it absolutely shouldn't cause any sort of issue during a transplant, though.

So, pretty much I almost freaked out for no reason.  Yay!

We stopped by the gift shop on our way out, for a little gift for her and some candy for the kids.

Then it was time to find the car.  Tyler had pointed in the general direction of where he had parked.  I had him send me a picture of it in relation to the hospital.  So, this is what I had to go by:


It took about 10-15 minutes to find the car.  I didn't have the key thingy that would let me honk his car horn, but that would have helped.  It was a little more challenging than I thought.  The parking lot was a ways away from where Emma's appointment was.  It was like playing an "I Spy" type of game.  Only we were looking for our car.

Anyway, so that was our adventure.  Emma was pretty fun & funny.  It was fun that she didn't get scared.  I really like that little pickle.

We've been having a lot more bone marrow transplant talk and considering some pretty heavy duty, life changing decisions.  It feels like we're nearing a crossroads in our life where things will likely change very drastically.  We're likely coming upon a time where we will only be able to walk by faith, trusting God, with everything else out of our control.  It's something we knew would come one day, and it feels like it is sooo very, very near.

But that's an entry for another time.

Also, some really exciting news...we have an adorably cute little niece that was born this past week at home (go momma!).  A little red head too.  How precious is that!  Congratulations to their family.  :)

I hear banana bread calling me...asking me to make some...so off I go.