Harlie Update

Hi!  It has been a really long time since I gave you a general update on how Harlie is doing.  I appreciate all of you who have emailed me, asked me and prodded me to sit down and write.  I am grateful that you care as much as you do. 

Since her hardware removal surgery in May, she's been quite busy.  Well, we've all been a little busy.  Since June 1st, I have taken myself or my kids to 31 in-person doctor's appointments.  In addition to that, I've had 13 virtual appointments.  I'm lucky to still have my job!  Anyway, I'll focus on her medically (will update on general life later) and summarize by area:

Hearing

Back during Harlie's jaw surgery in March, we had an issue with her right ear (her hearing ear).  Her jaw surgery created a lot of bleeding, and the blood filled her ear canal.  I noticed that week of post-op that her hearing was way worse than normal.  Surgery was in early March and I couldn't get her in front of her audiologist until June!  This was so frustrating.  I guess someone (with good hearing) said hearing wasn't essential during the beginning of Covid.  Communication is so incredibly important and wow, life is so much more difficult when communication is limited.  

Anyway, her hearing results were not good.  Her hearing decreased, diminished, got worse?  We were hoping that it was due to a blockage in her ear canal (dried blood, hopefully?).  So, we scheduled a follow up for a few weeks away and started putting drops in her ear to help dissolve what was there.  

She ended up having a DLB (direct laryngoscopy and bronchoscopy) with her ENT in DC and I asked him to take a peek in her ear while she was under anesthesia.  He did and said he removed a clot.  Awesome!  

So, we returned to have another hearing test.  Insert sad face here.  No difference.  Dammit!  Her audiologist said that sometimes when kids have growth spurts, their hearing can get worse.  I'm assuming that she's talking about kids with hearing loss only.  As a bonus (said so sarcastically) - I didn't see any evidence of a growth spurt, either.  

So, she ordered her a new hearing aid.  That was kind of hard for me - I had to decide between a rechargeable hearing aid or the kind we had with batteries.  I know that sounds dumb, but I always have hearing aid batteries with me.  The thought of having to remove her hearing aid and let it charge seemed like it would likely be a problem.  But, she told me that eventually all of the hearing aids will be rechargeable.  So, I guess I should try to adapt anyway.  I will say that the new one has bluetooth.  That is very cool because now Harlie doesn't have to wear headphones when she is on her tablet.  So, we lose having to charge her headphones, but gain having to charge her hearing aid.  

Her hearing aid charger is a case that you insert the aid into and the case has a plug.  We have one case.  And we went to my sister's lake house for our first weekend away with the new aid - and you can probably see where this is going... we left the charging case at home.  Ugh.  See, I would have had batteries with me.  And, even if I forget batteries - they are the ONE medical thing you can buy almost anywhere.  

The audiologist also went on ahead and reprogrammed her current hearing aid for her new hearing needs.  So, we at least have a back up now, which is nice.  Of course we didn't have it with us when we went out of town.  Rookie mistake!  Luckily, my niece was coming to the lake the next day, so she stopped at our house and grabbed her stuff for us.  So, I've ordered another charging case to keep in her bag.  And as I'm typing this I just remembered that it is in and ready for me to pick up.  Like weeks ago...

Her hearing loss has always been a true loss - it dominoes into more losses (speech, communication, language delays, etc.) so it is scary to think about it continuing to get worse.  For example, if we need to comfort her in the middle of the night - she now cannot hear a thing we say without her hearing aid.  She used to hear if we spoke a little loud, slow and clear.  Now, she hears nothing.  One morning a couple of weeks ago, she came into our room at 4am.  She woke me up and said something, but I couldn't understand her.  So, I said, "What?"  And then she said, "What?" And then I said, "No, I'm saying what to YOU, tell me again."  And she said, "What?"  And then she walked out of the room. Ugh.  It makes me so sad when she feels like she isn't heard.  

So, yes, I think her hearing is really important. 

Eyes

Back in the fall of 2018 we saw her opthamologist.  She recommended that we put eye drops in her left eye (the one that doesn't close all the way) several times per day.  This might not sound like a big deal - especially if you only have this one task to do.  But, man, you should see all the crap we have to make her do everyday.  And she hates eye drops.  Anyway her doc said that she has some scarring on her cornea (in her left eye) in several spots - due to her being in several medically induced comas (over 30 days total).  It was a constant battle keeping her eyes closed when she was in a coma.  All it takes is one piece of dust and when they close her eyes for her - they've closed it right in there.  Anyway, that was in 2018.  

So, a few months ago, she started telling me that she needed glasses.  I'll admit - I was suspicious.  This girl loves her accessories, and I fully believed she just wanted another accessory.  After she kept on complaining, I figured it was time for a follow up anyway.  I'll also admit, that I was not the best at putting those drops in her eye and I didn't want to get scolded by the doc.  

Anyway, we went and as you might've guessed - she needs glasses.  Insert face palm here.  Luckily, she is nearsighted and she only really needs them when she is watching tv (or if she were in a classroom, which she isn't). And now, she needs one kind of drops 3-4 times per day and a different kind of drop at night before bedtime.  

Her doc didn't scold me, either.  She was very nice and empathetic.  I really like her because she looks at Harlie as a whole person - and not just at her eyes.  She can see that we have our hands full and that Harlie is well loved and cared for.  

Airway/Jaw

So, as I mentioned earlier, we had that DLB with her ENT in DC.  This was her first one with Dr. Preciado since her jaw surgery in Boston.  Here is where I talked about her airway and how I was looking forward to Dr. Preciado giving me his opinion.  

Anyway, he didn't think her airway was as small as Dr. Lee (the ENT in Boston) said it was.  So, that is promising.  He tells me he has hope that he will be able to get her decannulated one day (get the trach out).  And I want to believe him, I really do...

He also gave me his blessing to go on ahead and start working toward the next surgery.  The next surgery is essentially joint replacement surgery.  And after the jaw distraction surgery, we were thinking we needed to give her (and us) a big break.  However, we can't wait too long because any growth we might've gotten out of her distraction - we don't want to lose.  And it takes months to prepare for this next surgery.  Basically, if you want to make it simple, distraction was phase 1 and joint replacement is phase 2.  Pointless to start the process and not finish it.  So, proceed we will.  She had a CT scan a month or so ago and the company will use that scan to fabricate custom joints that are made to fit in her jaw.  

The fabrication takes several months.  Her Temporomandibular Joint Reconstruction with patient-fitted TMJ Prosthesis (you should've seen my reading material last week!) is scheduled for April 12 at Boston Children's Hospital.  

Spine

We also had her follow up appointment with her orthopedic surgeon in DC.  

He said that he thinks her quality of life will improve if he does another spinal fusion surgery.  Her kyphosis scoliosis forces her forward, into a bent over position.  This makes it very hard (honestly, impossible) for her to stand for any length of time.  I'm talking a few minutes, at most. This also makes it very difficult (honestly, impossible) for her to walk any distance.  I'm talking a couple house lengths, at most.  

But, he wants her to grow as much as she can before he does it, since after he fuses those vertebrae they cannot grow anymore.  So, he told me to think about it.  We scheduled her normal follow up appointment for some months away and I've been thinking about it.  He said it will be a pretty brutal surgery.  And, not only are we coming out of that horrible jaw distraction surgery, we are gearing up for her joint replacement surgery in April.  Regardless of her growth, I just can't fit spinal fusion in her schedule right now.  So, it is going to have to wait.  

Growth

We went to her appointment with her GI doc and they always look at her growth.  I guess since things are relatively "calm" right now, she said it was time for us to see Endocrinology regarding her growth - or lack thereof.  

So, we did.  Because it was a new specialty to her, I had to fill out new paperwork.  I hate medical paperwork.  I am so over writing the same information over and over again.  Plus, I mean, come on...

That was a pretty interesting appointment.  First, it was pretty impressive that they had taken the time to review her medical history before coming in to meet with us.  That doesn't always happen.  The first thing they saw was her growth chart.

The doc we first met with was a fellow (in her final year, I believe) and she really wanted me to know that she knew her stuff - about hormones, etc.  She was pretty thorough.  After going over the basics of growth plates, bone age, puberty, hormones, etc., her attending came in.  She said she wanted to meet us.  She said Harlie's growth chart was "striking" and after stumbling over her words in an awkward few moments, I stopped her.  I knew what she wanted to say.  So, I said, "You want to know where we've been."  Relieved, she said, "Yes!"  

I told them both that we've had other priorities.  I'm sorry, but there's only so much we can fit in!  And when you've got concerns about breathing, how tall you are just doesn't seem to matter that much. 

I honestly never thought anything else was wrong.  I know that must sound funny.  What I mean is that I just always assumed she was so small because that was all her heart and lungs could do.  She has clubbed fingers and toes because there's just not enough oxygen in her blood by the time it gets to those extremities.  So, how is there enough left over to let her body grow?  

But, they talked about puberty and how important it is for one to go through it.  Duh. She went on to explain to me that it really helps female bone growth since after menopause, women lose a lot of bone density.  I let it go the first time.  But, it was as if she thought I didn't get it or something, so she repeated it but changed a few of the words.  Then I looked at her said, "I don't think that is going to be a big concern for Harlie."  This is why I so appreciate a doctor who is able and willing to step out of their specific specialty and look at the whole patient.  Regardless, I, given all the current life-threatening, life-impacting concerns I have for Harlie, cannot possibly give bone density after menopause a second of my energy.  Moving on...

She went on to talk about her growth and puberty - where we are now and what we can do about it.  Let me see if I can break it down more easily for you.  

Puberty: Girls stop growing after they start their period. Periods usually start about two years after breast buds develop.  Harlie has no breast buds. She is 14. So, does she have at least two more years of growth potential?  To find out, we needed an x-ray of her hand to tell us her bone age.  If her bone age was less than 14, then that would mean she has more growth potential.  And we needed to get some blood work done to see what her puberty markers were and all that jazz.  If she had low to no puberty markers, then we might have the option to give her some growth shots and force some growth and then force her into puberty after that.  

So, we got the x-ray and then did the bloodwork.  

A few weeks later the doctor called me with her results.  

Bone age:  Her bone age is 14.  So no real growth potential there. 

Puberty: According to her lab work - she is in puberty right now.  Haha.  

So, no growth shots and no forcing her into puberty.  There is nothing to do. She is done growing and I don't know what this all means for her breast buds or her period. We have a follow up appointment scheduled in February, I think. 

She is 14 years old.  She weighs about 70 pounds, give or take a pound or two.  She is 4'4" tall.  And she wears mostly size 10/12 in clothes.  She wears size 3 shoes.  

Before you say anything or think anything - this is what I want you to know...

Our children are supposed to grow.  And when they can't do that, it sucks really bad. 

As parents, our jobs are to feed them - so they grow.  It is literally the first thing we do after they are born.  One of the more painful things for me as a mom was the inability to feed Harlie.  I mean, we did, by her g-tube.  But I'm talking about feeding her by mouth and then turning her over and burping her.  I never could do that.  She doesn't know what a cookie tastes like.  She doesn't know what it is like to chew food.  She has never had her favorite meal and then said, wow, that was really good.  

We tried for many painful years to get her to eat by mouth.  But, it just wasn't meant to be. Maybe one day we will be able to try again.  But, chewing is likely a pipe dream.  I have accepted that.  Luckily, you don't have to eat by mouth to have a good life.  

Anyway, we work really hard to feed her via her g-tube.  And I know that sounds weird - I mean, how hard can that really be?  Well, Harlie cannot tolerate a lot of volume.  So, that has always been a big challenge.  We have always given her as much as she could tolerate - sometimes more so.  But it wasn't enough for her to grow. Her heart and lungs are working way harder than they should have to - there's just not enough left over.  

There is no positive to this.  There is no silver lining.  There is no parent on this planet that says, I'm fine if my kid can't grow.  It's all good.  

Will she have to shop in the kid's department for the rest of her life?  

I don't know.  Her body is so crazy, she could have a growth spurt next year for all I know.  For right now, it makes me sad.  But, then I think we need to be grateful for what her heart and lungs are doing.  That is pretty amazing. 

She is happy and she is hilarious and keeps us laughing.  I overheard her during her virtual speech therapy appointment (insert eye roll here) the other day.  

ST:  Harlie, last week you really struggled with ______.

Harlie:  Because it was hard!

ST:  So, we are going to work on that again. 

Harlie:  Can't we do something else?

Just this morning, Harlie asked me if she could make and bring me my coffee.  We told her she could turn it on.  The next thing we knew, she made herself an espresso - and drank it!  I had to laugh because it took me YEARS to train myself to drink coffee.  I had to start with a sugar-filled coffee drink at Starbucks until I could handle less and less sugar in it.  And this girl wakes up one day and chugs a freaking espresso.  

She also breaks my heart on a daily basis.  She had the opportunity to play with a baseball bat and tee a couple of weeks ago.  Apparently she loved it.  So, now she tells us that she wants to play baseball in the spring.  She wants to be on a team. She googled it and found a local little league and showed it to me and said she wants to go there.  She actually said her life would be better if she could play. 

Sometimes, it is as if Harlie has no idea she has heart and lung defects.  The girl gets out of breath walking up the stairs!  And - for real - you think we want her around a ball that could hit her face/mouth?!?!?!  Or her pacemaker?  I mean, we aren't even supposed to let her play any kind of sport where another person or ball could hit her in the chest/pacemaker.  

Look, we let her in the pool, in the lake, in the ocean - all areas of grave danger (and immense joy).  We've put oxygen on her bike/trike.  Tom has taken her "hiking" and we take her camping.  We've let her ride roller coasters (the more gentle ones). We do our best to give her all the experiences we can give her.  Oh, she wants a freaking scooter for Christmas.  Oh, the fear we have of all the bad things that could happen!  Why oh why does she have to want to do things we shouldn't let her do?!  

Luckily, we know a girl. And she runs Jacob's Chance and if we are lucky, they will have Buddy Ball this spring.  Alexa, remind me to call Kate. 

All of this reminds me of this quote I came across a few years ago:

What is stronger than a human heart, which shatters over and over and still lives... ~ Rupi Kaur

To end on a really positive note, one big thing happened in our house last week.  Harlie changed her own trach for the first time ever!  We are so proud of her!

That's it for today.  As always, thanks for reading and for caring about our girl. 

Much love,

Christy xo