The Latest on her Lungs

I have so much I want to share!  Here's what I want to blog about:

1.  Deep Run Marathon Dance
2.  Spring Break
3.  We Heart Harlie and Friends Event
4.  Harlie's lung update

The first three items are all good stuff.  So, I'm thinking I should get the lung update out of the way first.  I've actually been dreading writing about it.  So, here goes...

The last I updated about her lungs was when I said that the doctor had left me a message on Friday, March 28th that there was nothing easy to fix in her lungs (no abnormal vessels) that was causing her oxygen requirement.  He called me again on Monday night.  He said that the CT scan of her lungs showed that she doesn't have enough alveoli in her right lung to produce enough oxygen to support her.  

And, in case you are wondering, there's no way to give her more alveoli.  What she has is what she has.  And in all likelihood, it will not get better.  In fact, it will mostly likely get worse.

What caused this, I don't know.  Her right lung was never good, even from the start.  That was the first thing we learned at 16 weeks pregnant.  At 11 months old, they took out two lobes and left one.  Her doctors at that time told me that lungs grow, and that the single lobe could get bigger over time.  Which, it did.  But, it never grew more alveoli.  Or maybe it did and over time and many, many surgeries, anesthesia, pneumonia, and/or collapsed lungs later, damage was caused to what was there.  I don't know.  It doesn't matter anyway.  She doesn't have enough and that's that.

What does this mean?

Well, it means that she NEEDS oxygen.  And will likely need it until something changes.  I say that because I really don't want to say "forever."  Which I think is probably the case.  No one will say that anyway.  The fact is that no one knows what will happen.  Here's how I think about it...

Her lungs gave her six years on room air.  Okay, that's not totally true.  Her first full year she needed oxygen, too, but that was for a different reason, sort of.  That was because the mass in her chest was pressing on her good lung.  She was able to come off oxygen after she fully recovered from her lobectomy (removal of her two lobes).

Anyway, the day after Christmas 2012, she got sick.  We've always had oxygen because whenever she had a respiratory bug, she needed the support.  With only three lobes (instead of five) she had no reserve.  Any hit to her lungs was noticed.

She's been on oxygen ever since.

So, to go back to my thought process - these lungs carried her six years with intermittent support.

Just SIX years!  That is not very long.  What does that mean for her future?  How much longer will they carry her?

She's 46 pounds now.  What happens when she's bigger?  Will they be able to support her then?
And if she had "no reserve" before, what does she have now???

Back a few months ago, her cardiologist and I had a few conversations about possibly removing the right lung entirely.  After her docs had the CT scan results, that idea was scratched.  Basically, if her left lung is doing 85% of the work and her right lung is doing 15%, she still needs the 15%.  She can't live on just the 85%.

And I agree with that logic.  Back in the summer when she had her jaw distraction surgery in Boston, her right lung either collapsed or had pneumonia (looks the same on an x-ray) and she needed breathing support from a ventilator to get her through that.  I know what she looked like before they put her on the ventilator.  She slept for several days straight (and that is not how a normal recovery goes) and when we put her flat on her back, she could barely breathe at all.  It was scary.  And that was her on her one, good lung.  No way could she live like that.  Here is the post about that experience.

So, will her right lung stay at 15%?  For how long?  Was it at 40% a few years ago, then at 30%, and it's now at 15%?  Because if that's the case, the trajectory isn't good.  We can't afford for that lung to decrease in output.

And in this post, I said,

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. 

So, will we have to consider a lung transplant?  It seems that it's that or... what?

I think it's safe to say that it's not likely these lungs will last her for as long as we would like.  All risks are increased now.  A sickness could go the wrong way.  Another bout of pneumonia, or atelectasis (collapsed lung) from a prolonged time on anesthesia, could do more damage.  That damage could change that 15% output to 10%.  Or maybe just her growth and time could put a toll on her lungs.  I don't know.

Honestly, putting it bluntly, I think the best case scenario (other than some miraculous change or staying status quo forever) is one in which a transplant is an option.

Worst case is that her lung continues to decline, and we are told that she is not a candidate at all, there's nothing we can do and we watch her deteriorate.  This is my worst fear.

So, I suppose I need to revise the above sentence, "If we have to consider a lung transplant, we will be very sad people."  And change it to read, "I pray to God that if/when her lungs can no longer sustain her and her quality of life deteriorates, we will be lucky enough to have the option of considering a transplant."

As I told her cardiologist on the phone, "Well, this sucks."  To which he replied, "Yes.  Yes, it does."

And then I said, "This is going to be very hard for me because I've worked so hard to make things better for her."  Then he said, "And you have."  But I can't make this better.

And then I started crying.  Which I totally HATE to do.  But, I couldn't help it.  I was heartbroken, still am actually.  I don't want this to be true.  I quickly got off the phone and I couldn't leave my room.  It was awful.  I don't think I've felt that devastated since I was pregnant and they sat us down in that conference room at Children's National Medical Center and told us about her heart defects.  Hard to believe that next month marks eight years since that day.

I would never have guessed then, based on what all the doctors were telling us, that we would be here, with her, loving her still, eight years later.

So, I know to have hope.  I know that no one knows what will happen.  But, I also know that this new reality... isn't great.

After she was born and she was here, we just tackled each problem as it arose.  While we certainly had some scary moments, nothing seemed insurmountable.  And I really believed, with all my heart, that one day we would get to a place of rest.  A place where all major obstacles would be behind us.  The trach would be out.  And she would be able to go get frozen yogurt with her friends, and eat it, by mouth.  (Even in my daydream I knew eating pizza might be reaching too far.)

I guess I was in denial.  I suppose I had to be, to get through all the crap we've had to get through.  I had to hope that there was something good for her at the end.

So, now what?  What am I supposed to do now?  For the first time in a very long time, I feel like we've hit a wall.

Which reminds me of something from the Nike Women's Half Marathon I ran on April 28th.  One supporter was holding a sign that read, "It's not a wall, it's a door.  Push through it."

As soon as I read it, I had to hold back the tears.  If only my inner strength could push us through this wall.  If only my inner strength could make things be different...

So, this is where we are now.  I've been trying really hard not to think about it.  Which is why I waited so long to write this post.  I just didn't feel like facing it.  I still don't, really.  But you can't hide forever.

A couple of other things that this new lung diagnosis (if you will) means for her and for us:

Trach.  Since she will remain on oxygen (forever?) this drastically reduces the chances of her being decannulated (getting the trach out).  This has been a dream of ours since literally Day 1 of her life when we were told she needed one to live.  I never thought keeping the trach forever would be a possibility.  And after ALL we've done to give her a good airway...  it's indescribable.  Can't even put into words how I feel about this.

School.  Will she ever go back to school?  Nothing has changed since I put her on home bound services.  She still needs oxygen.  And what if she gets sick?  Is it worth the risk?

So, where do we go from here?

I don't know.  I definitely feel like we are in a completely different place.  I have to accept some new challenges and I suppose it's time I accept some that have always been there.  I have a dear friend who suggested that I look into a palliative care program for Harlie.  Palliative care isn't just for the dying.  It's also for those living with life limiting conditions.  So, I did.
I spoke with someone last week.  She said, "First, we need to see if you qualify."  Then she asked me a bunch of questions.  Then she said, "Okay, so you qualify."  She understood that this changes everything - our direction, our decisions, our outlook on everything.

The bottom line is that I want her to have the best life possible for as long as possible.

And she gets that, of course.  She said she was going to do some research for me.  And she was going to help me figure out the whole transplant thing.  Where would we take her - Boston or Philadelphia?  When do you start that process - sooner or later?  I know we aren't there yet.  But does it help at all if a team meets her sooner?  Would we be looking at a heart/lung transplant or just lung?  I don't know.  I think part of me just wants something to do - to be working towards a bigger goal.  I still want to make things better for her.  I don't know how to do nothing, to just sit and wait.

I'm having a hard time, in general, with all of this.  There are a lot of things to be sad about.  After talking with her docs back in the beginning of April, I had a hard time just getting through the day without crying.  Every time I looked at Harlie and she smiled, my heart broke a little more.  As sad as I was (and still am) I know that I cannot allow myself to miss out on the here and now because I'm sad about what might become.

And I have to look at how Harlie is now.  Today she is happy and well.  She is learning something every day.  And every day I am learning something new about what she knows.

Just the other night, Tom told the kids it was bedtime and Harlie signed and verbalized, "Ten minutes."  I couldn't believe it.  That was the first time she's ever asked for more time.  First. Time. Ever.  When I told her teacher about it the next day, she said that they have been doing that in "class."  For example, "Harlie, you have three more minutes and then we have to do something else."  So, she's taking what she's learning in school and applying it to real life.  YAY!

Most of the time, I'm okay.  But there are certainly moments (like the one during the half marathon) that catch me off guard.  And like Murphy's fourth grade recorder concert last week.  The kids were singing and I found myself thinking about Harlie and how she can't do that, too.  And then I was mad at myself for being sad about her when I should be focusing on Murphy.  Then the music teacher said that she tells her students to "dream big, because you never know, one of these students could find the cure for cancer."  And I thought about how different my dreams are for Harlie.  I can't tell her that she can do whatever she puts her mind to.  And my "big dreams" are for her to live, for as long as possible.  For her to be happy while she's doing it.  And for her to be able to go get frozen yogurt with friends one day.  And while I'm so grateful for all she can do, I can't escape my sadness for all that she's lost.  Even at a fourth grade concert for my son.

Anyway, that's the update on her lungs.  Since it was a crummy post, I'm going to end with some pictures.

Harlie loving Rooney.

Just because Rooney's so great.

See the abuse he tolerates from her?

I wonder where Harlie is hiding.

Oh!  There she is!  I had no idea.

I walked into her room last night to find her changing her baby's diaper.  This is a new development.  And it's a really cute one.  She actually holds her nose and says the old one is a "stinky diaper."

 

The other night Harlie was playing with her baby, putting her in a baby carrier on her back.  She proudly left the room and Murphy said, "Harlie's going to make a great mom one day!"  And it took all I had not to cry.  Oh, the things he doesn't know or understand...

Me and Murphy after his concert.

Rooney sporting his new raincoat.
Seriously, this dog gets me through some tough times.

As always, thank you for your support.  I'll have better posts soon.

Much love,
Christy xo