Saturday, March 30, 2013

when to transplant & "if I die" request

The decision of when to transplant seems to be making itself for us.  Based off of how Emma's counts are doing, that she's in transfusion ranges, and that things keep going downhill, Emma needs a transplant, and she needs it soon.

We'll be moving forward with things when we get it all lined up with the donor (because there will be one, right?!), as well as insurance, and getting everyone organized who will help with the other kids.

We'll update more once we get more information and some idea as to when we can start doing this.

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Since telling Emma about the reality of her disease, death has become a somewhat common discussion in our house. It's done pretty low-key, kept pretty casual, with her being the one to bring it up. She doesn't seem terribly concerned by it.

Yesterday I got a little bit of a surprise. I got a "if I die" request from Emma. Want to know what she wants? hehe. I'll just share the conversation:

"Mom, if I die, will you make a sad video of me and put it on youtube?"

I paused what a was doing for a second and thought for a quick bit to figure out how I felt about that exactly. Part of me was feeling that emotional crushing feeling of hearing my baby girl tell me her "if I die" request, while the other part of me was finding her request to be absolutely hilarious.

I think I restrained myself from letting out a giggle (can't remember for sure)...and from crying...and I said, "You want a sad video made?"

"Yes, with music. Really sad music."

"And you want it on youtube?"

"Yes. I want it to be sad" (as though it would be possible to make it NOT sad!)

"Okay, sure I'll do that. I'll make it as sad as I possibly can."

"Thanks." She smiled big. Then she added, "But I don't think I'm going to die. I think I'll be just fine."

I agreed, as usual.

haha! It still makes me giggle.

(Emma has always been interested in youtube videos like that. She emotionally connects deeply with the families and their loss. So, her request came from that and was a very innocent, childlike request, in case it sounded differently in written word vs hearing it in person.)

So, guys, you better hope she pulls through her bone marrow transplant or else be prepared for the saddest video EVER. I might need some music recommendations. Maybe "Baby Mine" from "Dumbo". That's the saddest song in existence and why I will never watch Dumbo again.

I'm not really planning this in advance...just teasing and wanted to share! The things I never thought about having a conversation over.


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I heard Emma saying her personal prayers before bed, and she said, "And please bless me to surviiiiiiiive my transplant."  She said it in a sing song type of voice that didn't at all match the seriousness of what she was asking for.  It's hard to explain without being able to have you hear me imitate it.  But it made me giggle.

The things I never thought to experience with all of this!

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Emma has a penpal in England (that I'm a total loser at helping her write to her).  She's such a sweet little girl, named Grace.  Grace emailed and asked Emma how she was feeling about her transplant.  Emma said:

"I am feeling happy about the transplant. I think I will get better from the transplant and can't wait to do it. I am not scared. I feel like I should do a bone marrow transplant. It's what I think Jesus said to me. It's how I feel inside. I know I might die from a bone marrow transplant, but I'm not scared because of how I feel about Jesus."


I want to have faith like her.

Wednesday, March 27, 2013

Ultrasounds & CBC

Today Emma had a couple appointments.  She had ultrasounds on her liver and kidney(s) to start off the morning.  Then we ended with an appt with the hematologist.

Here she is eagerly posing for a photo while we wait to be called back to have the ultrasounds done:

She laid like this the WHOLE time he did the ultrasounds.  Not because I was taking pics either.  She did this well before I pulled out my phone.  She just wasn't in the mood at all to have doctor appointments.


We have no idea what the actual results are!  He said he'd get the results to the doctor by tomorrow.  They are looking to make sure her liver is okay from the androgens.  They were also checking out her kidney(s) to make sure it/they were okay - just a routine checkup.  No reason to think anything is wrong with it/them.

Speaking of which, when she was born, they told us she had only one kidney.  When she was four months old, they said she had two kidneys that were conjoined on her right side.  Then when she was a few years old, they said she only had one kidney.  This time, I asked the guy, and he said she might have one or two kidneys.  It's hard to say.  haha!  Either way, she for sure has one kidney.  If she has two, it's completely stuck to the other one, making it appear as one large one.

Once we were done with the ultrasound, we stopped by the restroom, and there was this door in there, as seen in the photo below.  It says, "Caution: Radiation Area".  That would be the first time I've ever seen a door like that in the bathroom!  Makes me (a) feel really safe, and (b) wonder if I am going to develop SUPER POWERS!!!


Ultrasound appointment finished way earlier than expected, so we went out to the van to grab Emma's drink so we could stop somewhere for her to eat.  She had to fast for the ultrasounds.  She was running out of juice (energy) on the way back, but I thought she looked so cute.


Here we are in the whatever-the-name-of-this-area-is-called, and Emma is eating.  Actually she was hiding behind her sandwich baggy - her see-through sandwich baggy.  Usually she doesn't mind having her photo taken, but a couple times today, she kept hiding.


We made it up to the hematologists about 30 minutes early.  Here we are hanging out, having a party.


All the pics of both of us making normal expressions, the lighting made Emma look a little odd - so just pretend it's not doing that to her.


And, now her being silly.  We're not really as shiny as the photos might lead you to believe we are.

So, now to her hematology appt:

She weighs 50 lbs!!!!  Those androgens really do their thing.  They give her an appetite, she eats like a little pig, and she's gaining weight.  It's crazy how much she has grown since we started her on them.  I used to think that means she'll end up taller than she would otherwise, but the doctors have said that it closes her growth plates quicker, so in the end, she won't end up any taller.

Katrinka, the awesome phlebotomist came in to do her blood work.  I really didn't want to force Emma.  I wanted her to feel like she could choose to do it...too bad there is no "or not", in the sense of choosing to do it or not.  As I mentioned, Emma was really extra anti-doctor appointments today.  She absolutely did not want to get a poke.  She did not want to hold her arm out.  It used to be that I could gently pull her arm out and hold it for her, while trying to verbally comfort her.  But now she is so strong, that I seriously can't do it! I'd have to put my foot on her shoulder and pull with a whole lot of might to get her arm pulled out.  And, putting that much effort into getting her arm out just doesn't seem right.

I tried to gently pull her arm, to see if she'd willingly give it, but she refused.  Katrinka tried too.  I think she thought Emma was a little bit stronger, like I'd mentioned, but she was probably thinking I'm just a wimp (I can look pretty wimpy) and that it wasn't too bad.  So, she tried to pull Emma's arm out, and she responded, "Oh my gosh!  She is really strong!" which made me start laughing.  She wasn't having any better luck than I was.

Anyway, Emma finally grudgingly got her arm out.  She was very upset, and then, of course, the poke didn't go so well.  She got the vein right away, but then it stopped for some reason.  So, there was digging and then another poke.  Emma was not happy.  None of us were happy!  But at least it wasn't 5 pokes and lots of digging with each, right?

After this time, I seriously began wondering about getting her a port, and when an appropriate time would be to do that.  I'm guessing closer to transplant?  She's getting poked at least every four weeks, unless she gets sick, and then it's more often than that.  It just seems like it would save her vein and save her from so much emotional distress.

Now on to the results...

WBC: 2.0 (was 2.0 last time too)
RBC 1.88 (was 2.06)
HGB 7.3 (was 7.8)
HCT 21.4 (was 23)
Platelets 19 (was 26)
ANC 700 (was 550)
MCV 113.8 (was 111.30)

Quick summary of what that means is that she's low on her red blood cells.  They are in transfusion ranges.  But since she is going to have a bone marrow transplant, they really don't want to transfuse because the body can start developing antibodies, which would causes issues with transplant.

It also means that her red blood cells are quite large, her bone marrow is stressed (MCV is kind of like a gauge for how stressed the bone marrow is...the more stressed, the higher the number.  I think normal is under 90).

Her platelets are super duper low.  They are also in transfusion ranges, but platelets only last for about two days and then are gone.  Meanwhile, her body would be building up a resistance to them via antibodies that I mentioned before.  So, we won't transfuse with platelets unless she starts bleeding.

Speaking of bleeding, she's no longer allowed to jump on the trampoline because it's too much of a risk of causing her to hemorrhage.  She has to be careful with anything she does that she takes precautions to prevent herself from getting hurt.

He gave us some medication that I can't remember the name of.  It's typically given to hemophilia patients to help their blood clot.  If she starts to bleed (like a nose bleed or something), we should give it to her to help it stop.

He also said to keep an eye out from intense headaches, which could be a sign of her bleeding inside her head.  Also, if there is any blood in her stools, it's likely she is internally bleeding.  In those cases, we would need to get her transfused to stop the bleeding.

We don't have any new news on bone marrow matches!  I am eager to know.  Our local hematologist asked how many potential matches we had.  I'm pretty sure they told us we had 30 potential matches (meaning, the testing was more basic, and now they need to do more detailed testing).  He says he's almost positive, from his experience, that out of 30 potential that she'll for sure have a match.

I also asked him if we were to transplant Emma this summer, if it would be likely that we'd be able to get everything in order fast enough to get that done (like getting the donor to donate, getting insurance squared away, getting everything organized that needs to get organized) and he said that no problem we should be able to.  He highly suggested we get going to visit the transplant center and have them check Emma out, so we'll probably be doing that soonish.  I'm thinking we'd need insurance approval first.  So, however long that takes.

We aren't sure when we'll transplant.  We do have the option to increase the androgen dose.  I explained the side effects to Emma, and since she is going to transplant, she'd prefer to just transplant instead and avoid any further side effects (which have been very minimal so far).  So, if we don't increase her androgens, I'm guessing her counts will continue to fall.  I don't think there's much choice in putting it off too much longer. We're still working on making a firm decision on when to do it.  Plus, we need to talk to the transplant doctor about it all.

Anywho, so that was that.  If there is anything interesting to share about her ultrasound results, I'll be sure to do that!

I hope everyone reading this is doing well!  :)

Monday, March 18, 2013

Bone Marrow Match Update

We got an email today from the transplant center that is helping us find a match.  They said they received Emma's updated HLA typing (bone marrow type) and compared it to the bone marrow registry database.  They found potential matches, but they will have to do more testing to determine whether or not someone is a full match.  The reason is, when the potential donors signed up on the bone marrow registry, it was a while back, and the testing they did isn't as detailed as the testing is now.  So, they'll call in people and do further testing, and then they'll be able to narrow it down from there.

Emotionally, it's an interesting place to be.  I feel such gratitude for everyone who has ever joined the registry, and I feel so thankful to whoever takes the time to go in to be tested - even if they don't match - as well as whoever does match...because there will be at least one!  Or so I believe.

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I totally forgot to post these!  I cut Rhys' hair about a month ago.  Still working on growing it out from when she chopped it off.  It's been 8 months (?) since then.  Whew.  Who knew it could take so long to get back to a normal hairstyle?!

Rhys is the one on the table.  (Don't mind Violet's expression...hehe.)  Her hair had grown in enough that it needed another trim.

So, I gave crazy-won't-hold-still-at-all Rhys a bob.  Then I styled it.  I think it's adorable.



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And, here are some pics of Rhys from church.  We don't actually go into the church building since a lot of people attend church when they are sick (a LOT of people!).  So, we just go and have a little lesson outside or in the van, and it still teaches that on Sunday we go to church!  hehe.

We love this picture of her! While she looks adorable with a little bob, it'll be fun to be able to grow it out again.





Her pink shoes were a nice touch.  :)  If only my lens was a bit more wide angle to have captured it in the first photo!

Here's a picture (taken with my phone) of three of the kids.  Violet would change her pose with each click of the camera.  It was really funny.



Saturday, March 16, 2013

a couple more things


In an unusual series of events, I met a wonderful lady, who not only does so much to help families and children, but she also reaches out to whoever she feels has a need.  If you haven't already seen this, she posted this about Emma on her blog:

http://kidlutions.blogspot.com/2013/03/emmas-fight-how-you-can-help.html


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And to the conversation that made me cry the most afterwards...


Last night Emma was talking to me about her transplant. She was telling me how badly she wants one and how she can't wait. She wants to be able to live a normal life again & she started listing off all of the things she wants to be able to do. She was giddy with excitement. It was really sweet.


But she knows the reality of a transplant, knows that it's a very difficult process, and knows that she can die from it. She stopped talking for a second and then said, "I might die from it." I told her that was true. She said, "I really don't want to die from it, but if I do, I know I'll get to see Jesus and be with Dianna." (Dianna is a little girl with FA that she met during her transplant, who ended up dying.) I told her that was true and then I listed off some more people she'll be able to see, like my grandpa and my sister. She thought that sounded very exciting.

She said, "You'll miss me a lot if I die, won't you. I bet you'll all cry a lot." Then she smiled. I told her I would miss her more than anything, and I told her that we would all cry for years over her. She said she would miss us too.

Then she went back to how excited she is to have her transplant. She said that she's going to believe that she won't die...it doesn't feel like to her that she will. I told her that I believe that too.

I just think of when I was ten years old and the things that I had to worry about...and here she is facing the reality of death, and her faith amazes me. I think we have the bravest little girl ever...and we are so blessed to be her parents.


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(okay, now I'm almost caught up on our blog!)


Oh, I did find out we won't find out about the bone marrow match until next week likely.

Friday, March 15, 2013

Swabbing

She's been swabbed!  Emma had her HLA typing (bone marrow typing) done when she was a baby.  But when the transplant doctor pulled up the registry to find matches, she emailed to let us know that Emma's HLA typing is "low resolution" (only matches 6/6).  We need to do it again and get a "high resolution" typing for her (matches 10/10).

We had some misunderstanding about how our insurance works, and so it ended up taking up longer to get the new testing done on Emma, but we did finally get it!  We gave the transplant doctor the go ahead, and she overnighted the swabbing kit to us.  Our local hematologist was willing to pull labs on Emma, or do the swabbing himself, but we didn't want to wait 4 weeks until our next appt.  So...that is why you see that I am doing her swabbing.  :)  They gave us the instructions (24 swabs!!), called and told me exactly how and what to do, and then included a prepaid mailing envelope to have it mailed directly to a lab and the testing done immediately...results then sent to them...and bone marrow match searched for.  The swabbing was sent in this Monday.

Where we are right now, I hope, is that they have received the results today (maybe?!!) and will be able to see if Emma has any bone marrow matches out there.  They thought we'd be able to hear back this week as to whether or not she has a match, and it's the last day of the week.  I definitely feel very anxious about it.  We'll update once we know the results.

(You should be able to click to see it larger)



Thursday, March 14, 2013

our decision

We have officially made our decision to do a bone marrow transplant.  It was an unanimous decision, Emma included.  I've had an overwhelmingly strong answer to prayers about it.  I feel very confident that our decision is the right one.  I might share more about all that at a later time...but I have about 10 minutes before dinner is ready and can't do it right now!

We've had a lot of difficult, tender, memorable, life changing discussions with Emma that I wanted to share as well.  I'm not sure there's much else that she doesn't know about her illness anymore.  She has completely amazed us with her views and her strength and her faith.  (copy/pasted from our FB pages)

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BABIES?
Today our topic was on babies, and if she'd ever be able to have any. She asked me about it. Not a topic I was excited to have to tell her the reality about. 

The truth is...

If she doesn't have a transplant, she won't live long enough to have a baby. 

Even if she did live long enough to have a baby, w/out a transplant, many girls with FA enter menopause very, very young and can't have children. (Then the few who can, most have very high risk pregnancies.)

But Emma's not going to be one of those that will be able to make it to adulthood without a transplant. And, the awful truth with a bone marrow transplant is that it will sterilize her.

So, almost no matter how you look at it, the odds aren't in her favor that she'll ever be able to have a baby. 

I explained that to her, in more simple, age appropriate terms (are there age appropriate terms for that?). Then I talked about how special adoption is...since it really is an amazing, beautiful thing. 

Her response to me was, "Mom, look. I think my ears are wiggling."

Not the response I was expecting. Haha! So, for a while, she randomly practiced her new found skill of wiggling her ears via her mind powers. I can't do it, so I don't know how she does it. She can also jiggle her eyes back and forth really fast. 

But I wondered if she understood what I'd said or not. I thought I explained it well. She had asked a few questions & watched me intently (maybe she was just concentrating on wiggling her ears, though). Due to her hearing impairment, her comprehension level is closer to about 7-8 yrs old in many ways, combined with the emotions of a ten year old. 

Then tonight, right before bed, she said, "I wonder if I'll ever be able to have a baby." So, we talked about it again. Turns out she had listened earlier, but she understands things the best when she hears them several times. I imagine we'll be talking about it again. 

It breaks my heart, though. So many hard things for her to learn lately. I don't want her to ask anything else! Not for a while. I don't want her to be sad. It's harder emotionally, than I realized it would be, to explain all these things to her (and I thought it would be hard). But, I do try to remain open and easy to talk to...even if I wish we never had to have some of these talks. 

Tonight, she asked Tyler how babies are made. Haha. These other conversations, about FA, give a perspective that makes that near-future conversation a little easier than it might have been before.


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WHY DO FA ADULTS DIE?
Today she randomly asked me why all the FA grown-ups die. I think she knew the answer, but she wanted to know if it was really true. 

One of the things we decided from the beginning was to be honest with her as she asks questions...but I didn't realize how hard that would be...and I was really hoping she would take longer to ask this one question. 

Anyway, so I asked her if she really wanted to know the answer, while I was thinking to myself whether or not I really should tell her. She said that she did want to know, and so I felt like I should honor that. I'm sure people will judge it for being right or wrong, but I feel like it's right.

So, I explained to her that they always end up developing cancer and dying at some point from that. Her eyes got really wide, and she gave her little smile that I'm starting to recognize as her I'm-smiling-but-not-really-smiling smile. Her eyes got all teary eyed. She didn't understand what cancer was, but she knew it was something people died from. 

I felt like I needed to explain why a transplant was still important - that it could give her a lot more time here with us before that happens than it would if she didn't do it. I explained that there are doctors and scientists working to try to figure out how to stop the cancer, so there's some hope they'll figure something out by the time she's older. I told her that there are some that have lived to be as old as daddy and even older (and you know that is REALLY old...at least by her expression, you'd think so!).

I'm sure we'll talk about it again, but I have to say how amazed I am at our baby girl for handling news like this like she does. I only know what it's like as a mother to find out this about my baby. I don't know what it is like for her emotionally to find out the information about herself. Now I feel like crying all night. It's hard knowing that she knows that now, but I feel convinced it's the right thing to do - to be honest when she asks or else she'll learn she can't trust us. We can't keep it from her forever...and it was obvious she had put it together and needed to understand it better. But still, I hate that she ever has to know!

Anyway, so that was the highlight of our day.


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Okay, dinner is finished, so I'm going to run.  There's a bit more to update on, which I will do soon.

We are waiting to hear if Emma has a bone marrow match...and I think I'm going to die from anxiety.