Sunday, March 30, 2014

CT Scan Results

I can't believe I didn't know that her doctor called me on Friday and left a message!  I just listened to it and he said that they reviewed her CT scan, and it did NOT show any arteriovenous malformations.  So, no collaterals.  Which means her lung is just not working sufficiently anymore.  Crap.


He said he would call me again on Monday to discuss it.  So, I'll know more tomorrow.  

Crap.  Crap.  Crap.

I'm going to go cry now.  Thank you for all your thoughts and comments regarding my last post.  You are the best.

Much love,
Christy xo

Lung Details

I've been feeling blue these last few days.  And usually I don't want to write when I feel that way.  But, I'm hoping that writing about it will help me shake it.

Harlie had her CT scan on Friday.  The night before I told her that she had a doctor's appointment in the morning and that we would have to wake up early.  I don't normally tell her about just regular old appointments.  She must have noticed the difference and sensed that this was a bigger deal.  So, she started to cry - a real sad cry.  Ugh.  I told her that it wasn't that big of a deal and that I was going to be with her the whole time and that it would be over quick.  No over night, I said.  She cried for about ten minutes and signed "scared."  There are times I'm not that sympathetic, and tough love is the way to go.  But, this was not one of those times.  It kills me that she has to be scared so much.  And for darn good reason, too.  But, she eventually believed me, I suppose and went to sleep with no tears.

She's usually a BEAR to wake up.  I have often said that I have no idea what we're going to do when she is a teenager, because that girl will sleep past 10 am every day if we let her.  I got up at 4:15 am and got myself ready.  Then I woke her at 5:15.  I went in her room, put in her hearing aid and whispered, "It's time to wake up" in her ear.  And can you believe that girl sat right up?!  That is a FIRST for sure!  I guess telling her the night before was the right way to go!

Anyway, we left the house right on time (another amazing feat, if you know me) at 5:45 am and arrived a few minutes early for check in.  See?  I just haven't been myself, lately!

Then we hung out in the waiting room... for a long while.


She really cracks me up.  Despite the tears the night before, she was one happy little girl!

I suppose that the anesthesiologist looked at her chart before calling her back and ended up spending more time than originally planned going over her history.  I'd love to be a fly on the wall when a new doc clicks on her name.  Anyway, we talked and he asked the usual questions.  Thank God she has no issues with anesthesia.  Can you imagine?

They were so kind.  They wanted to let her fall asleep first before putting in the IV.  So thankful!  The reason she had to have anesthesia is because it was a CT scan with contrast (which requires an IV) and she has to lay really still for several minutes.  Considering there's contrast involved, it's not worth the risk of her moving.  I believe they can only give you so much contrast in a specific amount of time.

All went fine, of course.  I wasn't worried.  She woke up a little mad, though.  The nurse didn't apply pressure to her IV site long enough to stop the bleeding, so she bled through her dress.  That was very upsetting to her.  Blood got all over her other hand and she kind of freaked out.  Of course I had extra pants, but no top.


We left the hospital around 9:30 am.  As we were walking out, I saw Harlie's nutritionist from when she was a baby.  It's always good to see her.  We chatted a bit.  And then Dr. Williams came up to say hello.  He was one of her PICU docs from back in the fall when she had the infection in her jaw.  He didn't have to stop.  It wasn't like we passed each other and he saw that I saw him, so he felt like he had to stop.  I didn't see him and he could have walked right on past without me being the wiser.  But, he stopped.  Isn't that so nice?  He asked how she was doing and what was going on.  I gave him a very brief update.  It is very nice to see friendly faces there.  And I always find myself surprised that people remember us.  But, I'm told Harlie's pretty hard to forget.  It's so funny what I find to be normal.  And to others, she's so unusual.

Anyway, I took her straight to Target to get something for being so brave.  If she could eat (or liked to) I would take her for ice cream.  Oh well.

I don't know when I'll hear the results of the CT scan.  I hope this week.  I hope that it will tell us something.  I don't think I ever told you about her appointment with her pulmonologist a few weeks ago.

Here's the recap.  We saw him.  He had already spoken with her cardiologist (Dr. G) and the head of the radiology department about what tests she should have.  I love it when they come in prepared.  He said that the best test would be a cardiac MRI.  Of course she can't have an MRI because she has a pacemaker.  No magnets can be near her.  So, no MRI.  I knew that was going to bite her in the butt one day.  We chatted some more and I wanted some clarification on what I was thinking.  I've been asked questions by family and friends and I wanted accurate answers to give them.

1.  Are you happy that it's not her heart that's causing her oxygen requirement?  No.  You need both organs to live.  They are both important.  And it seems that there are more options when it comes to a heart.  Plus, one can get a heart transplant and go on to live a long, good life.  It is not the same with the lungs.  How many lung surgeries have you heard of?

2.  Can she have a lung transplant?  No.  A lung transplant is trading one disease for another.  It isn't a cure - ever.  And it only buys you time.  Like five years.  If we have to consider a lung transplant, we will be very sad people.  That is a terrible position to be in.  I really can't think about it.

3.  If her lungs just don't work right anymore, is there anything that can be done to "fix" them?  No.  Once all the parts in there stop working, they are done.  No fixes.  Refer back to question #1.

4.  Can she live with just one lung?  Yes.  You can live with one lung.  Clearly her heart makes it more complicated.  But, it is "believed" that her remaining good lung could handle the blood flow.  I don't think there is another person walking this earth like Harlie, so it's hard to say for sure.  The obvious negative with relying on just one lung, is that you only have that one lung.  So, when she gets a respiratory illness, it will affect her greatly.  And her physical activities have to be adjusted accordingly, meaning no contact sports or being on the top of the cheer leading pyramid.  Those are his words, not mine.   Because I always assumed those were out, even with both of her lungs.  She can't even walk around the block.  I love how both of her doctors went straight to, "Well, she'll never run a marathon."  And that's said like who the hell wants to do that anyway?  

Um, me?

So, no contact sports - fine.  No cheer leading - fine.  I don't want her to wear make up anyway.  I hate that they have little girls do that.  No running?  Ugh.  Honestly, I knew that all along.  That's no surprise.  Heck, she can't even walk around Target or the grocery store.  I don't know what we're going to do when I can't put her in the cart.  Anyway, it does make me sad that she will have all these "nos" and "can'ts" in her life.  As parents aren't we supposed to tell our kids they can do anything they set their mind to?

And the worst thing about having one lung?  If there's trauma (like in the activities mentioned earlier, or a car accident - which would be the most likely for her) and her lung collapses or is punctured, she's done.  Meaning death.

While discussing what tests we should run to confirm that her right lung has been damaged beyond repair, Jim came in to test her CO2 levels.  The two of them started talking about how a certain test would be done with a patient with a trach and Jim asked Dr. Schmidt, "So, what are you going to do?"  And Dr. Schmidt said, "Whatever Mom tells me to do."

I had to laugh!  I love him!  I immediately thought of this thing on Facebook that was going around.  It said something like "You know you're a parent of a medically fragile child when..."  And one was "when you tell the doctor what to do."  Too funny.  Another one was "when you take care of other things before you take your child to the ER."  I told Dr. Schmidt about it and said I may or may not have taken a TRX class at the gym before taking Harlie to the ER.  We had a good chuckle.  Oh, the things I find amusing....

Anyway, he said he would have to do some research and call me later.  Which he did.  He spent a lot of time going over her history, and called me the next day.  He said that they want to rule out a collateral vessel(s) before doing anything else.  I suppose that could be a cause for her poor sats coming out of that lung.  He also said that after talking with some other doctors, they are all in agreement that they want to try to save this lung.

The first test was this CT scan with contrast.  We'll see what information it provides and then go from there.  In discussing the kinds of tests she needs, Dr. Schmidt said, "Ideally she would need a _______ (I can't remember which test it was) and she would need to hold her breath like 20 times in a 45 minute time period (or something like that).  I looked at him and said, "Let me tell you what would be ideal."  He totally got my humor.  We had another good chuckle.  Then I told him that I don't think she knows how to hold her breath.  So, that test is out for now.

So, in summary, here is where we are with her lungs...

As she is right now, with a full, good left lung and one remaining lobe "limping" on the right:

  • She needs oxygen.  I can't believe it's been almost 16 months of oxygen 24/7.  I just can't believe it.  
  • She has a hard time trying to speak.  When you talk, you hold your breath.  She can only pronounce a few syllables before she has to take a breath.  She just doesn't have the lung capacity to hold enough air.
  • She can NOT tolerate any exercise.  And I don't mean exercise like you go to the gym to do.  I mean just normal, walking around kind of exercise.  A couple of weekends ago we had a friend over and his kids.  Harlie wanted to play in the backyard with them and so I let her come off the oxygen for a bit.  After some walking around I could hear how hard she was breathing and saw how blue her nails were.  I checked her sats, and they were 71.  That's just terrible.  Terrible.  She can't even play.  Kills me. 
  • Last night (really, this morning) she was awake.  Maybe she had a bad dream.  Tom said he had to get up to drain the water from her tubing (condensation from the humidification) and she signed "scared" to him.  A little while later, it had to be drained again, so I got up.  This was about 4 am this morning.  She was still awake.  She signed "potty" and disconnected herself from the oxygen and the pulse ox and went in the the bathroom.  After she was done she signed "Mommy's room" and went in our bed.  She has never been able to sleep in our bed.  Never.  After a minute of letting her lay there, I held her tubing up (showing her it needs to be connected) and she sat up.  I carried her to her bed.  And when I hooked her back up, her sats were 77.  In a matter of five minutes, her sats went from the low 90s (on oxygen) to 77 (no oxygen) and she barely walked at all.  I carried her back, even!  

I'm hoping that it's something easy and simple (like collaterals) causing her low sats.  But, clearly, I would have to say that's unlikely.  I am going to continue to hope.  But, I know in my heart that we might seriously be disappointed.

My thoughts have been heavy these last few months (or 16).  I don't want her to lose this lung.  But I can also see that as it is, it isn't doing her much good.  The whole thing just makes me sad.  She's seven.  How many lives does this girl have?  I can only surmise that if she has to have that lung removed, that living with her one lung must, in some way, reduce her life expectancy.  It has to increase the risks in every way - infection, illness, collapse from anesthesia, etc.

Obviously I can't let my thoughts go there.  But, sometimes I can't help it.  I try so hard not to worry.  But, what mother doesn't?  And these worries aren't crazy, out-of-the-realm-of-possibility worries.  They are very real.

And when I can't sleep, I start to think of living with this oxygen.  Will we know more about what to do before summer?  I can only assume that she'll still be on oxygen when the pool opens.  What then?  Can I let her go in the water with oxygen?  I think the answer is yes.  But what about the tubing?  That can't be safe for other kids.  What if they don't let me put her in the water with the tubing?  How can I possibly NOT let her go in the pool?  Seriously, I can't even think about it.

The kicker to my blues yesterday (it was a bad day) was that we couldn't take her to the movies.  Tom took the boys and I stayed home with her.  She was pretty junky and required a lot of suctioning yesterday.  There's no way I can take her to the movie theater, on a rainy Saturday, when I have to suction her a lot.  While she would be fine, the suction machine would be a major distraction (annoyance) to other movie goers.  The fact that she has to miss out on so many normal activities (and knows about it) just makes me mad.

Anyway, I have to stop now.  I hate complaining about this stuff.  It doesn't do anyone any good.  And I think I might feel a bit better now that I've gotten all that off my chest.  I'm sorry if you now feel worse!!

I will let you know what I find out about the CT scan as soon as I hear.  As always, thank you for reading and thinking about our sweet girl.  You get me through these tough times.

Much love,
Christy xo

Tuesday, March 25, 2014

Quick Update

I have so much to share with you.  Here's a snapshot of what's been going on.

We had Harlie's appointment with her pulmonologist on the 13th.  I will go into more detail very soon.

The Deep Run Marathon Dance was the 14th and 15th.  It was awesome.  What an amazing experience!  The whole thing was incredibly impressive.  I really want to tell you all about it (and show you pictures and videos) but all that stuff is on my other computer, so I will have to save that update for later.

I spent last week in bed, sick with some horrible flu-like virus.  It's pretty much gone now.  While I feel SO much better, I can tell that I am not fully back to 100%.  My lungs and exercise endurance is dragging a bit behind.  Which is perfect timing with the Ukrop's 10k coming up on Saturday.   I don't know why I signed up.  No more races after this one and my half at the end of April.  None.  At least for a long while.

Now that I am better, Tom is sick.  It's much worse when he is sick, since he is the one that provides dinner for the family.  I am going to try really hard to make dinner tomorrow night to give him a break.  I know that sentence makes me sound like a complete idiot.  But, as I've had to learn so much in certain areas (almost every major medical specialty) some basic brain function had to be sacrificed.  The cooking was the first to go.  Poor Tom.

Cooper started soccer again and is loving it.  Apparently the last few months of growth and maturity made a difference, because Tom said he could immediately see that Cooper was much more into it than in the fall.  I missed all the action because I was sick.  But, he scored two goals in his very first game!  What a turn around!  I also signed him up for basketball through his preschool.  I love that they offer programs like that.  He stays for two hours one day a week to play there.  Awesome.  He liked his first class earlier this week.  We'll see how it goes.  It is amazing how incredibly different each parenting experience is/has been for each of our kids.  Murphy has NO interest, Harlie is unable and Cooper is ALL in.  It's a good thing I'm so flexible...

I want to share a video I have of Harlie tubing herself her dinner.  But, it's on my other computer.  So, I will have to save that post for later, too.  If you're on Facebook, you might have already seen it.

My Mom had her lumpectomy and all went well, I think.  She has another appointment next week and we'll know more then about the next steps (radiation, etc.).  But she isn't in any pain and she is in good spirits.

My younger brother, Cabell and his fiance are expecting their first baby in August.  They just found out they are having a boy!  We are all excited about that.

Now that the Deep Run Marathon Dance is behind us, we are setting our sights on the We Heart Harlie and Friends event May 3rd.  It will be here before we know it!  Here is the flyer...

Registration for the 5k and Kid's Fun Run is open and new shirts are for sale.

We are going to have Women's shirts in Tahiti Blue, Purple, and Vintage Black, Men's in Vintage Black and Envy (green) and Kid's in Turquoise.  Pictures of the shirts will be up on the website soon.  If you have come in the past, please note the new location.  It is going to be at Deep Run High School this year (instead of Glen Allen Elementary).  We outgrew the space at Glen Allen, which is a great problem to have!

I can't remember what I've told you about We Heart Harlie, but it has grown into a foundation!  It is now We Heart Harlie and Friends and Lynda has been working with an attorney and CPA to get all the paperwork and applications done to make it be an official foundation with a 501(3)(c) status!  It is so exciting!  Here is the new website, which is in its beginning stages as Lynda is adding to it and adjusting it as necessary.

We are so excited to be able to help more families now.  Each year we will select different families to help.  We have a board, and it is almost full.  If you would be interested in being on the board, please contact us and let us know.  And let us know if you, or someone you know, would be interested in donating a product or service for the raffle.  We are so grateful for every donation!

We are hoping to do some more (and different) fundraisers for the foundation throughout the year.  We would like to do an adult only silent auction with food and drinks.  And last night Cheeburger Cheeburger had a fundraiser night where they donated a percentage of the proceeds to We Heart Harlie and Friends. How cool is that?! Tom and I ventured out and took the kids.  It was so much fun!  There were so many Glen Allen Elementary teachers there!  It is so sad that we don't get to see them since Harlie is still on home bound.  But it made seeming them out (and wearing their We Heart Harlie shirts) even better.

I have so much more to share!  But, I have to stop for now.  Harlie has a CT scan scheduled for Friday.  I want to share what I know about that so far.  So, I am hoping to update again tomorrow or Thursday.

Thanks for checking in!
Much love,
Christy xo

Wednesday, March 5, 2014

Heart Cath Report and cardiology appointment

Harlie's last heart cath was December 13, 2013.  I received the report a few weeks ago and met with her local cardiologist last week.  The number that we (okay, I) was most concerned about was the 24 with a line over it (to the left of the pink circle).  The 24 is the pressure of her Fontan.  I won't even try to describe her heart anatomy (or function) in this post (other than that her heart was formed in a mirror image - so what's normally on the left, is on the right, etc.).  I guess I could have color coded the picture before I uploaded it.  That would have made explaining it a lot easier.  Oh well.  All you really need to know is that her heart is nuts.  And, while the 24 isn't great, it isn't "the" problem.

The main problem is the circled 86, which is circled by a pink marker.


That is the percentage of oxygen in her blood as it leaves her right lung and enters her heart.  Her circulation works like this:  heart is single ventricle, so it pumps in one direction only - to her body.  Red blood (fully oxygenated) leaves her heart and goes to her body.  It returns from her body (blue, needing oxygen) and goes straight to her lungs to get oxygen.  After it gets oxygen from her lungs, it goes into her heart, to be pumped to her body again.

Since the blood comes from her lungs - fully oxygenated - her sats should be close to 100% (not 86%).  It hasn't entered her heart yet - so her heart defects should not play a part yet.  Plus, I gave her to the cath doc on oxygen.  She was on oxygen in recovery afterwards.  Does that mean that she was on oxygen during the cath?  Because if that's the case, 86 is ON oxygen, which means that it would probably be lower if she wasn't on oxygen.

So, the question is... Why are her sats so low leaving her lungs?

And that means it's not her heart.  It's her lungs.  UGH!!!  This was actually my fear before the heart cath.  I just felt like her heart was probably more "fixable" than her lungs.  If the lungs don't work, I don't think there's much you can do about it.  While her heart is jacked up, but it "works" because they did a bunch of stuff to it, you know?  Plus, all of a sudden, I felt very unprepared.  I was all ready to learn about pressures and crap, and then I had to change gears.  And wait two weeks to talk about it again.  So frustrating.

I can't remember if her cardiologist said that her pressures of 24 are an after-affect from the low sats or not.  Or maybe they are just 24, just because that's her.  I can't remember.  I suppose it doesn't matter for now.  He did say that she has early elevated Fontan pressures.  The pressures go up in time, that's normal.  He said that kids 16 years old and up have pressures in the 20s.  She's 7 and hers is 24.  But, one can live with high pressures.  So, that's not the main issue right now.

So, now we have to see her pulmonologist to see what tests we can do to find out what her lung function actually is.

For those that don't know, Harlie had a chest mass in or around her right lung.  The right lung is made up of three lobes and the left is made of two lobes.  During my pregnancy with her, they found the lung mass and it was preventing the normal growth of her right lung.  After her birth, it became a back burner item, until she was about 8 months old.  The mass was still growing and it had begun to squish her good lung, compromising its function.  So, the mass had to come out and in August of 2007 (she was 10 months old at the time) they removed two of her right lung lobes.  They were able to leave one lobe.  And after that, she did much better.  She was finally able to come off oxygen (after a full year on it) and was able to learn to sit up and crawl.  Life really took off for her after that.

But now, is that one lobe doing more harm than good?  Is it permanently damaged?  Should it be removed?

We see her pulmonologist next Thursday.  And we'll go from there.

The whole thing is frustrating.  I just wish she could get a break.   It seems we are always having to worry about a life-requiring issue (heart, lungs, airway).

Managing her care (from my perspective as her mother) has become very overwhelming.  I have moments when I am confident in my decisions and my observations.  And I have moments when I am NOT.  And in her case now, her symptoms are not black and white.  Everything is grey and open to interpretation.  What if I misinterpret something?

I try to remember the times where I feel confident in what I interpret... for example, we took Harlie to the bowling alley a few weeks ago.  It was a fundraiser for the Deep Run High School Marathon Dance.  Brandy was with us and was focusing on Harlie while I talked (as usual).  Brandy started to notice that Harlie would walk up to the ball return and stop and rest.  Then pick up the ball, and stop and rest.  Then walk - slowly - and bowl.  Then rest.  So, she made her sit down and she checked her sats.  ON oxygen, they were 76!!!  Obviously, that's a sat she just can't tolerate.

So, clearly she NEEDS the oxygen.

Did I ever tell you about her not being able to digest her food when I was experimenting with her oxygen needs?  Well, just in case I did, I'll make it short - I wanted to see if she could tolerate lower sats, without the oxygen.  Her sats seemed to hang out in the low 80s (which I didn't think was that bad) without the oxygen.  But, after a few days, her body wasn't able to process her formula and I couldn't get in all four cans in a day.  So, her body was sending oxygen to main organs (and not as much to her GI system).  So, that means she cannot tolerate low 80s sats.

You'd think I'd be confident by now.  But it's scary to have to be the one to have to notice everything and know whether that thing is important or not.

Anyway, I don't know how definitive the tests and/or any answers are going to be.  One confusing thing is that he said she needs a CT scan of her lungs.  She had one back in June of 2013.  Lucikly, here in Richmond.  So her doc was able to pull it up and read the report.  It doesn't say much other than that her lungs are in better shape than they were in 2007.  Maybe if they did another one, or had that one from June re-read, with the radiologist knowing that her sats are low leaving her lungs, that would make a difference?  At least now we know what to look for.

Her cardiologist said that we will have to let her symptoms guide us and maybe make some decisions on faith instead of hard data.  Which is how we made the decision to remove the bad lung tissue.  He was the one that said it had to come out - and from what I remember, not every doc was in agreement at the time.  Yet, that proved to be the right call.

So, that's heavy on my mind.  And again, I find myself wishing time away.  Wanting to hurry up and just get to that next appointment, that next surgery, that next... whatever.  It's really a terrible way to live.  And I wonder when we'll get to place of just... living.

Another thing that's heavy on my mind is my Mom.  A few weeks ago she found out she has a bit of breast cancer.  We're really focused on the "bit" part.  Of course I don't think that's a real, medical term.  But, I'm making it one in this case.  She is scheduled for a lumpectomy tomorrow.  So, that's a good sign.  And her surgeon said it's the kind that responds well to hormones, so that's good as far as after treatment goes.  And it did not get into any lymph nodes.  We all feel positive that this won't be that big of a deal, really.  Just a small amount of time that was uncomfortable (she had a bunch of biopsies and an MRI) and a bit scary.  As long as that time stays small, all is good!

And to end on a more positive note, I have two more funny Harlie stories for you.

The other day Harlie wanted to play on the iPad.  She signs and says "game" at the same time.  Tom told her no, and to go play with toys.  With electronics around all the time, the toys just lay around untouched more than we like.  So, Harlie was clearly disappointed and went and sat in the living room for a few minutes.  Then she said, "Daddy" and pointed to her Vest treatment machine.  He said, "Oh, you want your Vest treatment?"  This was surprising.  She never wants her treatment.  So, he was like hell yeah you can have your treatment and went and got her Vest on.  He said that the second that last buckle was buckled, she looked at him with a smirk and signed and said, "Game."

That little sneak!!!  She knows she can have her iPad while she's getting a vest treatment.  You should have seen Tom's face when he realized that he'd just been had by a seven year old!  We were so damn proud.  That girl is one smart cookie!  I realize that this behavior is something that most kids do and most parents aren't as thrilled.  But, you have a different appreciation for this kind of thinking when your kid doesn't learn to read before first grade. Or talk.  Or isn't pegged as talented and gifted at age five.

Then, the other night after I had tucked her in and went back downstairs (and JUST sat down, of course) her heated trach collar equipment started to alarm.  So, I had to go back up there to see what was the matter.  And the second I walked into her room, the alarm stopped and she sat up and said, "medicine."  So, she figured out how to get the machine to alarm, knowing that I would have to come up there.  She is something.

I have to tell you that for YEARS I have read about kids doing stuff like this (on the trach board and Facebook) and I have always wanted Harlie to be able to do that stuff, too.  Another proud moment.  Of course, as my dear friend Sarah said, cute at first, not so funny later on.  I suppose she's right about that.  But, my other kids can scream my name, or jump out of bed and come down and bug us.  This is just Harlie's way of doing that.  And she should have a way, too.

Well, that's it for now.  More later!

Much love,
Christy xo