Scope Results

So, Harlie and I drove to DC this morning. We had to be at the hospital at noon. But, she needed to have water at 11am. Her procedure was scheduled for 2pm. And because of her cardiac issues, they want to make sure she's hydrated.

Oh, how different traveling in a car is with her now that she's older and so much more independent! I just place her suction machine so I can turn it on and she suctions herself! And, she can tube herself water, too.

Traffic was light and I made it in less than two hours! Woohoo!

We got all checked in and waited for a while. This is her new wheelchair I haven't been able to tell you about.

We got it about two weeks ago. It has a smart drive thing that I can click into place under her (a motorized 5th wheel). I didn't have it on today because she's still learning how to use it. She wears a bracelet and it connects via Bluetooth. When she taps her wrist to the chair twice, it moves and will accelerate in speed until she taps it once to lock in the speed. And then she just has to steer it by applying friction to the wheels. Then, to stop it, she taps her wrist twice again.

It is going to be pretty cool, I think. She just needs more practice. And the smart drive weighs a lot less than a motorized chair. So, I can still lift the chair and get it in the back of our van, which was a must!

Anyway, her pacemaker has to be adjusted before and after every procedure. This is her holding the pacemaker thingy while Carol made the adjustments.

They finally took her back at 3pm. She told me she was scared, but she really didn't look like she was at all. She went away smiling and signing "I love you" to me. She is such a trooper!

Sometime after 4pm, Dr. Preciado came out to talk to me. I'm struggling to fully understand what he told me, so relaying it to you is especially difficult.

He said he understands why I'm having a hard time doing trach changes. There is a ridge of some sort off the back of her trachea that the trach is getting caught on. It requires a bit of pressure for the trach to finally break free from it before it goes into place. This clearly hurts her, causes a lot of irritation and I guess can build up scar tissue. And we can't let it stay this way. He doesn't want us to have to do many trach changes. Man, I am feeling so good about my decision not to change it these past few weeks!!! Go me!! I absolutely love it when my instincts are right because so often I do dumb things. Like this morning, on my way here, I went the wrong way, and had to drive several miles before I could turn around and go the right way. I've only driven here a thousand times, so that's totally understandable, right?

Anyway, he said that when I do a trach change, I need to stand behind her head while she is laying on her back and insert it in a forward motion, vs. standing in front of her and inserting it in a downward motion. Basically, I need to stay to the front of her trachea. That probably doesnt make any sense to you. Well, it sounds strange to me, too. And I'm nervous just thinking about it. Ugh!!!

I asked him if this ridge could be removed, but it is not that simple. Honestly, I don't remember what he said, but it wasn't easy to understand. He said that what she really needs is a stoma revision. The stoma is the hole in her neck. And the whole area is friable and easily irritated. She has a couple of keloids now, too. All new territory for us. Anyway, he said doing an emergency tracheostomy is very difficult and her body is not reacting/healing well. I'll say. That emergency trach was done almost six months ago! So hard to believe it has been that long. I feel like so much has happened in such a short time. Anyway, he wants to re-do it, basically.

Of course, there are no guarantees it will work. And the back up plan is that he might have to put an adult trach in. An adult trach has an inner cannula that you take out and clean, while the outer piece stays in. That way we are not having to insert one all the time. That has its negatives though, so we are hoping it doesn't come to that.

So, we have to give this stoma revision a try. This will require a week's stay in the hospital. And he says it needs to be done sooner vs. later. This is a major bummer. Anyway, he is looking at adding her on in mid February. We will see how that turns out.

He ended up putting a Bivona 4.5 in. The Bivona is more flexible. He said that a 4.5 is plenty big enough for her. So, I'm a little worried about her exercised induced intolerance. Maybe the stoma revision could help? I kinda feel like we're grasping at straws here. I must admit that I'm getting a bit worried that we aren't going to find answers.

He said I can take her home tonight. So, I am just waiting for her to wake up. She is bleeding and is in pain when she coughs. Like with big fat, silent tears. So they gave her some Fentanyl. The nurse asked me if she was awake or if she had fallen back to sleep. So, I said, "Harlie, are you awake?" And she shook her head "no." She's so funny. 

Oh, how I am not looking forward to spending another week in the hospital. Things really need to start going her way, for crying out loud!

Okay, it is after 7pm and she still looks pretty sleepy...

Thanks for reading and for loving us. We appreciate it more than you know.

Much love,
Christy xoxo

January Update

Hi! I have tried to start this post so many times. But, sometimes, it isn't easy to write.  I'll just try to jump in and tell you what's happening now...

After Harlie's heart cath in mid December, she showed no improvement in her exercise induce intolerance. You might remember that her cardiologist there said we should go back to her airway.

As things are now, she still gets out of breath with little activity (like walking to the bathroom, or going up the stairs, or answering the front door).  We are also having a hard time with trach changes.

We did weekly trach changes for nine years and never had an issue. You pull one out and you quickly put a new one in.  No biggie. I've done them alone more times than I can count.

Except now. I wouldn't dream of trying to do one without someone with me. Her anxiety is terrible, which makes her far from cooperative. And I can't blame her. We are hitting something when we put a new one in and she bleeds. That's gotta hurt.

The trach tube is soft and flexible, so in order to put it in, it has to have a hard plastic insert in it (called an obturator) to guide it in. Once its in, you take the obturator out. If you leave it in, she can't breathe. While doing the trach change, it takes so long that she runs out of air and starts to panic. So, I have to remove the obturator before it is in place to allow her to breathe. That is not the way it is supposed to go.

Anyway, I joke about having PTSD. But there are times when it isn't funny. And when I envisioned doing a trach change, I could see me not being able to get it in and her dying. So, I decided I wouldn't do a trach change that week. Or the next. Or the next. I weighed the risks versus the benefits and decided it just wasn't worth it.

I scheduled an appointment with the local ENT that her DC ENT wanted me to see. And I told her what was going on. So, she did a trach change for me. She still bled, but it went way better than when I do it. And while that makes sense (she's the expert after all) it still feels terrible to know that I'm not able to do it myself. After being able to do it independently for nine years, it isn't easy to accept that I'm not doing it right, or not as well, or whatever. I want to be what Harlie needs me to be. And she needs weekly trach changes. I should be able to do that. I want to be able to do that.

So, for now, we are doing saline breathing treatments several times a day (as often as necessary) to keep her trach free of plugs. And I haven't changed it.

She is scheduled for another scope on February 1st in DC. Her ENT (Dr. Preciado) is going to dilate her stoma and upsize her trach to a 5.0. Hopefully this will take care of her breathing issues. If it doesn't, you might have to pick me up off the floor.  It is so hard to see and hear her be so out of breath after doing so little.  I am also praying that she will be able to talk with this larger trach. It isn't easy for her with a 4.5 trach. I'm definitely worried how it will be after going up in size. But, when faced with choosing between breathing or talking, you don't really have a choice.  I'm trying hard to remain positive.  But, sometimes, I'm pretty angry at what life throws her way and the "decisions" that we have to make as her parents. 

Last week I had Harlie with me in the car when I picked up Cooper after swim practice. We were going to go to my sister's lake house that weekend. So, we were talking about that. We hadn't been since July! Anyway, Harlie asked me if she would be back to normal by summer. I asked her what she meant by normal. Her and Cooper spoke back and forth for a bit and then Cooper said, "She wants to know if she will get the trach out before summer so she can go swimming."

I took a deep breath, told myself to be strong, and said, "No. I'm sorry, Harlie. The trach isn't going to be out by summer." And she started to cry. I decided since she was already crying, I might as well go and tell her the whole truth.

So, I explained that when they gave her the trach to help her breathe, her trachea got damaged. That's why she needs the trach to breathe now, even though she used to be able to breathe without it. I told her we are all so sad for her. And that Dr. Preciado is sad, too, and he is going to try really hard to see if he can fix it. But that is going to take some time, maybe a long time. So, we don't know when it will come out. Her and Cooper were both so sad. It was so hard to have to tell her that. But, I couldn't let her think it was coming out soon, when it isn't.

We got home and I put her in front of me and I told her that we all love her and that none of us are giving up on making things better for her. But, for now, we have to try and accept the trach and find happiness anyway.  We all hugged and cried and she even hugged Cooper, who was so upset for her. Surprisingly, she has seemed okay since. Which is kinda crazy, really. I feel so bad for her. She has such grown up, complicated things to have to try and live with and understand.

Honestly, it is amazing, but she seems so grateful. She tells me she loves me when I'm feeding her. She tells me she loves me when I'm giving her a breathing treatment. And she will tell me at random times throughout the day.

I am trying hard to focus on finding happiness anyway.  I've noticed that I've taken so few photos in the past few months.  I tried to find one to put with this post, and I don't really have one to share. So, I'm going to try and change that.  The fact is that life is hard right now.  And somehow, we have to survive it and find happiness anyway.  Wish me luck!

Thank you for caring and for your unwavering support of us all.  You have no idea how often it helps me get through the days!

Much love,
Christy xo

Heart Cath #? Results

I've been sitting here with my tablet open in my lap for about 20 minutes trying to figure out how to start...

I think I mentioned in my last post that Harlie had "exercise induced intolerance." Which, in her case the word exercise means activity of any kind, like walking from one room to another.  This has really been life limiting and has prevented her from having the energy to play or do much of anything. 

We moved the Xbox down from the 3rd floor to our living room (oh, the sacrifices we make for our children!) because she couldn't go up there to play. We give her piggy back rides up the stairs at bedtime. She has moments where she plays, but they wear her out. Most days, just walking to the bathroom leaves her out of breath.

It is amazing to me how one problem seamlessly morphs into another one. Weeks later, I'm left wondering when "this" started. With that said, I think this activity intolerance started about 6-8 weeks ago. Probably around the same time we took her to see her ENT and he upsized her trach and discovered she has tracheomalacia (collapsing of the trachea). Once he upsized her trach, he said if we still had issues with her breathing we should see pulmonary. So, we did. And once we saw him, he said her lungs were good and we should see cardiology.  She's had an echo, chest xrays, EKG and she's had her pacemaker interrogated. The only thing left to do was a heart cath. 

 So, here we are. 

Yesterday, I had Harlie and Cooper in the car after swim practice. He asked if we could do something tomorrow (which would have been today) and in that moment I realized that I had not told Harlie about the heart cath yet. Oops!! So, I said, "No, buddy. I'm sorry, but Harlie and I have this thing in DC tomorrow." She looks up and says, "What?" 

So, I told her she had to have a heart procedure called a heart cath.  Without a worry, she said, "Mom, my heart is fiiiiiine." I explained that they were going to see if they could help her get more energy to play. She was cool with that. 

Last night at bedtime, she asked, "How long am I going to be in the hospital?" I told her hopefully just one night. She smiled and said, "A night without two annoying brothers..." That girl can be so rude!! ☺ Her brothers are so good to her!

I had to have her in DC at 6:45 this morning. So, Tom woke her up at 4am. She told Tom she had a great night's sleep. She cracks me up. What kid sleeps great the night before going into the hospital for a heart procedure? Anyway, she was all smiles and silliness waiting to go back. 

As for the results...

Two main things. 

1. He found some narrowing in her Fontan. In simplest of terms, her Fontan is the name of the procedure where the surgeon re-plumbed her circulation to return blood from her body into her lungs (normally blood goes into your heart, and then gets pumped into your lungs). They use Gore-Tex and it was sutured in when she was three or four years old. Since then, she has grown, but of course, the Gore-Tex has not. So, it has narrowed, kinda. This increased pressures a bit. Here's a couple of pictures. 

So, he put a stent in it to open it up more. I think he said it was 10mm and now it is 13mm. 

He said this might help a little. 

2. He noticed during her cath that her pressures changed with her heart function. When her heart was in sinus rhythm (the way your heart is supposed to beat with your atrium and ventricle working together, in rhythm) her pressures were 16. But when she went into junctional rhythm (not the way your heart is supposed to work, with the atrium and ventricle working separately, or out of rhythm) her pressures were 19.  

Her heart is really hard to pace, with beats initiating from all over the place (sometimes from the atrium, sometimes from the ventricle, sometimes the middle, etc.) and at random times. So, they are going to come and take a look at her pacemaker at some point. And they are going to send her home with a holter monitor to see if it does it again. 

So, overall, her heart is "good." Mine, however, is another story. 

I don't think anything he did today is going to be the fix she needs to live her life like she was living five months ago.  His recommendation was to look again at her airway. Sigh... 

Needless to say, it is frustrating. To look at just her airway, although it is far from typical or ideal, it is "fine". 

To look at just her lungs, although they are far from typical or ideal, they are "fine." 

To look at just her heart, although it is far from typical or ideal, it is "fine." 

But, put them all in one little girl and it isn't "fine." You've got yourself subpar results that you just can't fix. 

I told her doctor that I still feel like I'm in shock. I STILL can't believe this is where we are. I said, "She is getting so complicated." He laughed and said, "Getting?!" Haha! Wow, is my perspective skewed. This new normal is so much worse than the old one. 

So, at some point soon, we have to go back to the beginning and talk to her ENT.  

I just want her to be able to play and be happy. I know other parents out there who want their kids to be the best at stuff. Me, I am not that greedy. Sorry Tiger moms and dads! But being happy is where its at. And, the less it takes to make you happy, the more often you can be happy. 

Anyway, we are here for the night, in the recovery room. But, we have the whole hallway to ourselves, (the very unhappy child next to us just left) so I'm happy about that! See how easy it is to be happy? And this is where I'll be "sleeping" tonight...

Tomorrow morning, she will have her pacemaker checked again. She already came and made an adjustment. They will see how she does overnight with that change. And she'll have an echo and xrays, and then we can go home. She's fiestier than normal this time around. We just had to remove two dressings (jugular access and groin), while she yelled "leave me alone!" All's done now and she's resting. 

Okay, this has taken me all day to write. I need to be done and get something to eat. 

Thank you for all the love and support. I was talking with a friend this afternoon and she said she wished she could do something for me. I told her to just love me and cut me lots of slack. She said done! Honestly, you just don't know how much you reading this, caring about us, and cutting us slack does for my spirits. Thank you, from the bottom of my heart. 

Much love,

Christy xoxo

November, so far.

It has been two weeks since her scope in DC. For a while, I was so negative that I didn't want to write. Then, I only got brief moments to write. So, here's what I started after we were discharged on Friday, November 3rd.

I got such little sleep while in the hospital. And Harlie was sad, and so was I. We left the hospital at about 1 or 2pm on Friday. And I cried the entire drive home. The last time I did that was when I was pregnant with her and we learned that she had serious heart defects, in addition to her lung disease.

To say I was feeling down would be an understatement. As far as this trach goes, there is no light at the end of the tunnel. We wished for so long and worked so hard to get it out, it still feels like a shock that we are here.

The trach wasn't a choice when she was born. But it didn't feel like a life sentence. It felt like we were going somewhere and that we had time to get there. Now, it feels like we were sentenced to live a life we didn't want. It is a major loss of freedom. And I'm having a hard time coming to grips with it.

I know that we have to accept it. I know we have to make the best of it. I know we have to live our life differently and we have to think differently. We need to focus on what she CAN do, not what she CAN'T do. But that's all easier said than done. And it is complicated when she has her own feelings and she lets us know that she is not happy with the trach. The other night she said something that ended with, "this stupid trach."

I forgot to tell you that when we were in the ER getting admitted in DC for the DLB two weeks ago, they wanted to give her an IV. She said no, of course. I asked them if they could wait till she was in the OR (I knew they wouldn't go for it) but they said no. They did promise to only try one stick. If they didn't get it, they would wait. They called the IV team and that guy was AMAZING. He got it in so fast! Best stick ever. But, of course, we still had to hold her down. After they left and Harlie was still calming herself, she looked at me and said, "Why didn't you tell them to stop?" Ugh. Kills me that she could think that I'm not protecting her. Heck, in her  eyes, I'm even helping them hurt her!

Anyway, back to leaving the hospital... it was quiet when we got home. Tom took the boys and the dog on our fall camping trip. I was so sad for Harlie and I to miss it. But, there was no way she could go.

A friend of mine had dinner delivered to the house that night. That was amazing since I don't cook and I really didn't want to have to make any more decisions for a while. Thanks Marcy and Patty!

On Saturday night a bunch of my family came over for a little "party" to cheer Harlie up since she couldn't go camping. My mom brought Harlie a Lego Friends set to build. She didn't get up until she was done.

We ate dinner, watched the movie Sing and laughed a lot. We also acted like we ate all the boys' Halloween candy and sent a photo to Tom to show them.

Me, Brandy, Sandy (my sister), Kelly and Maggie (my nieces).

I love my family. We are crazy loud, but really, we have so much fun when we are together.

Sunday, Nov. 5

The boys got home from camping. It is so weird that we missed it. Been going there twice a year for years. It was not easy to have to sit it out. You could tell that Cooper missed Harlie. This is how they were the rest of the day.

Harlie is really gaining independence. She hooked up her own breathing treatment, including putting the saline in.

Monday, Nov. 6

Harlie's IEP was that morning. I was not up for that. I knew I'd cry, and I don't like to cry in front of people if I can help it. So, for the first time ever, I didn't go to an IEP meeting. I conveyed my wishes and let them handle it. I believe we really have a great team, so that helps. All went fine.

I had parent teacher conferences with four of Murphy's teachers that night. I'm happy to report that all is well there. They all said that Murphy is a really good kid.  Counting my blessings there.

But, for Harlie, the nights are always harder than the days. Her sadness breaks my heart. If only I could make it all better.

Tuesday, Nov. 7th

We saw her pulmonologist.  We love Dr. Schmidt. 

Dr. Preciado (her ENT in DC) said that if her sats/oxygen requirement didn't improve within a few days, then we should see pulmonary.  Unfortunately, they didn't. At this point, she was not tolerating the speaking valve at all. Her strong voice I heard initially was gone. She was back to talking while holding her breath. But her lungs look good and are not part of the problem.

I can't remember if I've already mentioned that she is still desatting when she walks any distance. Just walking to the bathroom gets her out of breath. So, Dr. Schmidt said we need to see cardiology for her exercised induced intolerance. Sometimes, I feel like we are chasing our tails.

Wednesday, Nov. 8th

Harlie had school picture retakes that morning.  When I told her we were going to school for pictures, she asked if she could take her backpack.  She wants to go to school so badly.  But, her body is just not ready.  Anyway, she did fabulous and I cannot wait to get her photos!

We also saw her wound care NP for her sacral ulcer (bed sore). She said we don't have to come back unless anything changes. And she told me I did a good job treating her wound. It healed really well. It is still sensitive, but it is closed, which is awesome. The new skin will remain more fragile and sensitive for a while.

Thursday, Nov. 9th

Tom and I had a date night and went to see a showing of the movie Le Ride. It was really good. Very enjoyable to watch.

Then, that night, we had some trouble with Harlie.

I never showed you a picture of the fenestrated trach.

The top trach has the hole in it (fenestrated). The bottom is a normal trach, different brands. This is how it works.

Ideally, air can get past the tube and then go through the vocal cords to make sound. In Harlie's case, there is some collapsing around the trach, not allowing air through. So, the fenestration allows air to pass.

It is not perfect though. One of the problems that we had was that when you suction, the suction catheter would get stuck on the hole, not allowing us to safely suction her. Another negative, is that stuff can grow through the hole.

This night, I think that's what had happened. Because when we suctioned her that night, and hit the hole, she flinched and started crying telling me that hurt. After that, she wouldn't let us near her trach, which is terribly unsafe. She has a strong cough, so we left her alone through the night.

Friday, Nov. 10

That morning, this trach issue got worse. She was very upset and her work of breathing increased dramatically. So, when Brandy got here, we did a trach change. I only had one fenestrated trach. The rest had been ordered, but weren't in yet.

She said she was very afraid of me changing her trach. And frankly, so was I. Especially since I had to use a different technique to avoid scraping the back of her trachea. It went okay, I guess, but she bled. It seemed to stop after a few minutes.

I went to work, which has actually been really good for me. I don't usually work on Fridays, but I've been trying to catch up from  missing so much time in August and September, so I've been working whenever I could. Plus, I never know how my week is going to go. I plan to work two days, and then something happens, and I can't.

Anyway, I came home that afternoon and Harlie was still bleeding. And she was still very protective of her trach and wouldn't let us suction her.  And she was still working pretty hard to breathe. It didn't make any sense.

We were supposed to go to my brother's (Bruce and Nancy's house) to draw names for Christmas. There was no way she felt up to going. So, Caylee came to stay with her. It took us a while to get her settled, so we went late. It was so good to be with my family. Sometimes things are so tense at my house. I feel like I don't ever take a deep breath when I'm around Harlie. It is very difficult to hear her breathe like that.

Anyway, we got home and it took three of us to suction her. It was terrible. We got her settled for the night and we were really hoping that she would calm down and sleep okay.

That is not how it went. Her work of breathing just got worse and we were very worried about her all night. No one slept. I thought about taking her to the ER. I don't understand why she couldn't breathe with that trach. But, after thinking about it, I realized it didn't really matter. She couldn't breathe, and the first thing you do, is change the trach. But I was very worried she wouldn't let me. She was so protective and wouldn't let us near it. And if I had to fight her, I thought that was too risky. My thought was that maybe she should get something to calm her down (like Ativan or something) at the ER and we could change it where I had back up and we could better help her. Also, since I had just changed it less than 24 hours ago, if the trach wasn't the issue, they could move on to the next step.

So, at almost 5am, I got up and got dressed. Tom started the car and got oxygen ready to go. I went to get her up, and saw that she had wet the bed, something she NEVER does. That made me even more worried. She told me she couldn't catch her breath and she could only say one word at a time. When I got her up, she completely fell apart and was in full respiratory distress. I realized there was no way I could drive her to the hospital. I was seconds away from calling 911, which I really didn't want to do. So, I said to Harlie, "Will you let me change your trach?" And surprisingly, she nodded yes. 

She signed that she was scared. I told her that me and daddy were right here and I asked her to be brave.  So, we put her on the floor, and I put in a non-fenestrated trach. This change actually went better than yesterday's. We sat her up and she took a deep breath, and smiled.  And the respiratory distress was gone. Thank God. Because that was awful.

I put her in bed with her clothes on, and she fell fast asleep.

We are so, so thankful for comfortable breathing and restful sleep!

She didn't tolerate the speaking valve with the fenestrated tube. And she certainly isn't tolerating it without. But her speaking is the same. She talks while holding her breath.

For some reason, I am feeling a bit less sad now. I'm so grateful that she's here, and that she's finally breathing better for the first time in months. I just can't see her in respiratory distress anymore.

I will never stop missing the sound of her sweet voice and laugh. No amount of gratefulness will ever take that away. And I hope and pray that one day we will get to hear her again.

I told her ENT that one day, I hope to have a road map to possible future decannulation. I need something to give me hope again. Honestly, I think it is very far away. If all goes well, in 8-10 years, we will be faced with a heart and/or lung transplant. Early adulthood sounded so far away when she was a baby...

Anyway, I have spent most days having to put forth a concerted effort to not cry. But I'm better now. If we can manage this trach without constant worry, we might just survive this. She seems a bit happier, too.

Just a few nights ago, when I was putting her in bed, she told me she wanted to do it by herself. I said, "you want to suction yourself during the night?" She said, "Yea, give you a break." She's hilarious and oh, so sweet. But, suctioning yourself while you are sleeping is a lofty goal (that she did not achieve). But it sure was nice to see this sweet smile again.

She is still having issues with this exercised induced intolerance. After speaking with her cardiologist, the next step is to do a heart cath. Just walking around the house causes desatting and increased work of breathing. Today, she got up to feed the dog. When she sat back down, she was out of breath, and her sats were 70. She is too tired to play or go to school.

So, we are working on getting that scheduled now. Her last heart cath was four years ago, on December 13th. I remember it so well. At that time, we couldn't get her off oxygen. Anyway, hopefully we can get a date soon.

I think life with the trach will be a touch easier to accept when she is fully recovered and well again. She was so good before this surgery. Good in every way. After the trach, it's just been one issue after another - the bed sore, the belly infection, the trach stoma issue, the too small trach, the plugging, the respiratory distress, the fenestrated trach, this exercised induced intolerance, the oxygen requirement, etc. Heck, having the trach is supposed to alleviate many of these problems. To have breathing issues WITH the trach just feels like adding insult to injury.

We are scheduled for a follow-up appointment with her ENT in DC this Wednesday. But since she seems to be breathing easier now, I'm going to see if he will be okay waiting another week to see her. It is the day before Thanksgiving and traffic on 95 is going to be horrible. Like more horrible than usual. So, I'd rather not travel that day.

Well, that's it for now. Thank you for all the love. We appreciate it more than you know.

xo,
Christy

DLB Results.

So, we are still waiting for them to take her back to the OR. I swear if I could add up all the minutes I have spent waiting on doctors, hospital rooms, surgeries, etc., in the last 11.5 years, it would be a scary amount of time. Maybe my super power is patience.

Anyway, I keep thinking that later today I will be happy or really sad. I signed the consent form for a DLB and for him to upsize her trach from a 4.0 to a 4.5. This is what I wrote about on October 11 about what it was like when she had a 4.5 in:

After talking it over with a few people, we decided to try it.  So, Brandy and I did the trach change and upsized her from a 4.0 to a 4.5.  After I put it in, I sat her up and Harlie put her finger over the trach to say something.  Not a sound came out.  Her lips moved, but she was completely silent.  She looked at us, hung her head and started to cry.  I sat her on my lap and tried to tell her that it was really important that she can breathe safely.  I tried so hard to be strong and not cry, but I just couldn't help it.  It was killing me.  How could I ask her to be okay with losing her voice?  It's just too much.  So, Brandy said, "We'll work harder to make the 4.0 work."  So, we put her back on the bed and took the 4.5 out and put a 4.0 back in.  And her weak voice sounded strong again compared to nothing.  And I was grateful.  So, we are now giving her scheduled saline nebulizer treatments to help prevent the plugging and that is going well.  Whew!

So, here are the possible scenarios from today's DLB:

1. He finds something in her airway like a granuloma or scar tissue, and he removes it or dilates it. Hopefully, if this is the case, this solves the problem and gives her more air flow. Not sure if he would leave the 4.0 in, or if he would still upsize her. Either way, I am hopeful that she would have enough airflow to be able to talk.

2. He finds nothing and her jaw and tongue base is occluding her airway. He would definitely upsize her in this case, because her only air is through the trach. In my opinion, this would be a bad situation, because of all the things I talked about in my last post and the fact the she would not be able to make any sound.

So, we just have to wait and see. It is now 1:17pm and we are still waiting in the pre-op for them to take her back. Ugh. I thought I would take her pic and she would NOT cooperate! She keeps on doing this crazy face when I try to take her pic!

They took her back at 2:00pm.  He said it would take about 45 minutes. I only looked at my watch 1,092 times.

3:30ish, he came out to talk to me.

Here's what he found:

1. A granuloma. He removed it.

2. He upsized her trach to a 4.5.

3. Her trachea is collapsing on itself. It isn't so severe that the trach tube is keeping it from closing entirely. It stayed open without the trach in it, it just narrows. I don't know if this can get worse on its own. I suspect it can. It was caused by being re-trached. It just weakened it. This is very bad news. For several reasons.
    A.  It means she cannot go back and forth between being untrached and trached.
    B.  It doesn't necessarily mean she can't be decannulated sometime in the future, but it makes it way harder.
    C.  She definitely won't be decannulated until they feel good that there are no more surgeries ahead.
    D.  She could potentially need tracheal reconstruction. But, we can't even wrap our heads around that, so I won't speak further about that right now.

4. Something about the positioning of her stoma, and/or changes in her trachea has caused us to hurt her when we do trach changes. The trach is basically scraping/hitting the back wall of her trachea when we put a new one in. I am so glad she got this scope now, instead of any later, potentially allowing us to damage her more. We have to learn how to change it in a different way. No pressure, there.

5. The collapsing doesn't allow air to go past her trach and through her vocal cords. So, he put a fenestration (hole) in the top part of the cannula to allow air to escape and go through her vocal cords. He said he feels confident that she will have a stronger voice and that she will be able to wear a speaking valve. I am very thankful for that. And so appreciative of him doing whatever he could to help her. He had to experiment with many trachs and different size holes and placement of those holes to find the right fit. We will have to have custom trachs made to those exact specifications.

6. Her stoma and the skin around it and inside it is very fragile. I don't remember exactly what he said, but he mentioned that he might have to do a stoma reconstruction. The stoma is the hole in the neck, just in case you didn't know.

It would have been perfect if that list stopped at #2. But, it didn't.

I really don't know what to say. I am thrilled that she can talk. As she has recovered from anesthesia, she's been speaking more and I feel like she can tell she can breathe and speak better. But, she also asked me what it was (as she pointed to the trach). I think she was a little pissed that she still had it. And she could tell it was different/bigger, which I think is pretty cool.  She told me tonight that she wants her trach gone forever. I told her she still needs it. She said, "No, I dont." I told her she does, but that we are going to keep trying. She cried.

Her voice is stronger and way less robotic now, so I tried to focus on how great that is. I think it is a hard sell considering she had that just fine without the trach. Sadly, I can't remember what she sounded like before she was re-trached. It is as if parts of our life just diappeared from my mind. But, I think she sounds way more herself now.

But, I am devastated that we have new, permanent, complicated problems with her airway. And that this trach is here to stay for the foreseeable future.

My friend Mona came to visit tonight and brought me dinner from Peter Chang's.

It was awesome to see her and to eat a good dinner. She also saved me by bringing me some hearing aid batteries. Somehow I lost a bag of some of Harlie's stuff, which had her batteries in it!

Then, my friend Stephanie's cousin, Yvonne came to visit! She is doing her fellowship in the NICU here at Children's National. She brought me some food, too! So nice!!!

Hopefully we can go home tomorrow. But, unfortunately, Harlie will not be able to go camping. Tom and the boys and dog left this afternoon.

Okay, I am falling asleep while writing this, so I have to go.  Thank you for all the love and support!

Much love,
Christy xoxo

October Summary

This is the blog post I've been working on and just hadn't finished. While I'm writing about what's going on now, I'll share this...

October 3

I had Harlie's IEP meeting at her school.  IEP meetings are never fun for parents.  But, I feel very lucky and believe I have a good team that really cares about Harlie.  Since she can't attend school right now, she is receiving home bound education, which means the teacher comes to our house to teach her.  Unfortunately, teachers can't come during contract hours (which means they can't come during the school day).  There are teachers that can come during the day, but Harlie needs her special education and hard of hearing teacher.  So, the higher ups were emailed and the situation was explained - she is young, very behind, has special education needs, and is recovering from a horrific hospital stay that left her with a bed sore that escalates in pain throughout the day.  So, the later you come to teach her, the more pain she's in and the harder it is for her to learn.  All the people that needed to make an exception in her case agreed and said yes, her teacher can come during the school day.  This was a big win!  I'm so thankful for all who thought about her case and wanted to do what's right for her!

October 5

Harlie got her ears pierced successfully!  So, that's a win.  She was so excited to get them done.

She definitely did not like the piercing itself, but recovered very quickly and jumped off the table to go check herself out in the mirror with a big smile. These are the earrings she picked out.

I almost cried, as I was a little overjoyed at the privilege of getting to do something so normal with my daughter.  And as little as she is, in that brief moment, I realized she's growing up (albeit at her own, very slow pace).  And I was really happy for her.

October 6

In doing Harlie's nightly care, I found a blood blister under her trach stoma.  It is so hard to find something on her and inside I'm screaming, "WTF is that?!"  But, outisde, I have to be all poker face and say with a smile, "Okay, let's go get your pajamas on."  I took a picture and emailed her local ENT to ask her for guidance.  The next day, the blister was gone and there was a little spot that looked like pus.  So, she called in a prescription for some ointment to put on it after we clean it with peroxide.  This area of her skin has really taken a beating.  It hurts to look at it.

October 7-8

Cooper had his first swim meet of the season and just moved up to the 9-year old group, which means he only swims 50s or more.  No more 25s for this guy.  He had five events and shaved off time in each event.

October 9

Two years ago today she had her stoma closed.  I remember how incredibly happy we all were. I still can't believe we are where we are now.

October 11

Her trach management has been difficult.  Much more so than it was when she had it for the first nine years.  I can't really remember her plugging before.  But, this time, she has plugged almost every single day, sometimes, several times in a day.  Plugging means that secretions get sticky and/or dry and clog the trach tube, making her airway even smaller, or sometimes blocked entirely.  We have to put saline in her trach to help break down the mucus so we can suction it out.  It is very stressful on all parties.  So I started to think that maybe her trach tube is too small.  Maybe if we went up a size, she wouldn't plug so easy.

This is something I had been thinking about for a couple of weeks.  My only hesitation was that I was afraid she would have no voice whatsoever.  While I know that breathing is the priority, her ability to communicate is pretty important, too.  And taking her voice away was a huge concern.

After talking it over with a few people, we decided to try it.  So, Brandy and I did the trach change and upsized her from a 4.0 to a 4.5.  After I put it in, I sat her up and Harlie put her finger over the trach to say something.  Not a sound came out.  Her lips moved, but she was completely silent.  She looked at us, hung her head and started to cry.  I sat her on my lap and tried to tell her that it was really important that she can breathe safely.  I tried so hard to be strong and not cry, but I just couldn't help it.  It was killing me.  How could I ask her to be okay with losing her voice?  It's just too much.  So, Brandy said, "We'll work harder to make the 4.0 work."  So, we put her back on the bed and took the 4.5 out and put a 4.0 back in.  And her weak voice sounded strong again compared to nothing.  And I was grateful.  So, we are now giving her scheduled saline nebulizer treatments to help prevent the plugging and that is going well.  Whew!

October 12

Our elementary school had their fall festival fundraiser.  Cooper really wanted to go.  So, we asked Harlie if she wanted to go and she asked us what it was.  We explained it and she said yes.  I was afraid it would make her sad to go to school for that.  But, I think it was good for her.  I heard lots of kids saying, "That's Harlie!"  And, "Mom, Harlie is here!"  It was really cute.  The best though is when we were standing in a line for her to play a game and the girl running the game came out of the classroom and went up to Harlie and said, "Harlie, want to play our game?"  Harlie said yes and the girl took her to the front of the line.  It was so sweet and thoughtful!

October 13

Kids got their flu shots and Harlie got her 11-year old immunizations.  The boys went first and neither flinched.  Harlie was crying just sitting there.  Her medical anxiety is off the charts.  I've never seen her like that before.  There is no reasoning with her and nothing anyone says makes a difference.  So, I had to put her on my lap and just hold her to get through the moment.  I knew she wouldn't like it.  But, I did not expect her to be that bad.  She really made it so incredibly difficult for the nurses.

Given her anxiety then, I decided to reschedule an appointment for her g-tube (how we feed her).  Back in May we saw someone about putting a different g-tube in.  Right now, we change her g-tube every three months.  It is a balloon type mini button.  But, they now make a balloon-less g-tube that has be to be inserted at the doctor's office and gets changed only once a year.  But, she said that it does pinch for a second when going in.  But, Harlie is no dummy and she will tell that it is "procedure-like" and will likely freak out.  And I just don't want to ask anything out of her medically that isn't completely necessary at this time.  So, I rescheduled it for January and we'll see how she is then.

October 14

Tom and I took Murphy to go see the Foo Fighters concert.

Tom bought the tickets a long time ago. It is hard for me to look forward to doing something like that.  A few weeks ago we went to see the Head and the Heart (also bought those tickets months ago).  I LOVE them.  But, looking forward to doing something out of the house makes me feel guilty, too.  So, my feelings can get complicated.  Overall, I'm not my happy self right now.  But, at the Foo Fighters concert, I made the decision to try really hard to live in the moment.  And that's what I'm going to try to do as much as possible.

Most of the time when I think of Harlie and what's going on with her, I look in the future and it is overwhelming.  And there are so many unknowns.  It occurred to me that she probably isn't doing that.  The other day she saw a commercial for a new episode of Teenage Mutant Ninja Turtles and she was excited about it.  Maybe she's living in the moment, too.  Although she does ask us when she's getting the trach out.  And we all get sad and I have to tell her that she's still using it to breathe.  And if we took it out, she wouldn't be able to breathe well enough.  So, she looked for her cap and tried again to put it on, and breathe without the trach.  No luck.  As of right now, we have no plan, medically speaking.  Which is kinda hard.  But, it is still too early to make one.

One of her surgeons emailed me a couple of weeks ago.  She said she'd been thinking about Harlie and hoping that no news was good news.  I had to email her back that I really believe that she has an upper airway occlusion again.  Put simply, she cannot breathe through her mouth and nose, period. She is trying, but she just doesn't have an airway above the trach.  This was her original problem and why she was trached for the first nine years of her life.

We climbed a mountain and it took us NINE years and FOUR brutal jaw surgeries to do it. She had a fifth jaw surgery and two days later, we fell down that mountain and we are right back where we started.  Frankly, I'm having a hard time picking myself back up.  I hope and pray that I'm wrong and that she just needs more time to recover.  I think about this almost every second of every day. I want to solve this problem like yesterday.  I want it to be the way it was.  The thought of living for months or even years like this, feels impossible.

October 18

We saw wound care today for her bed sore.  It is definitely a lot smaller and we are making a lot of progress.  But the new tissue around it is very sensitive and fragile.  And now she has a rash on the outside circle of the area.  Unfortunately, we can't really treat the rash while we're treating the sore in the middle.  Anything that we would put on the rash will prevent the dressing from staying on her skin.  And right now, the dressing over the sore is the priority.  We are still looking at weeks and weeks of healing.

October 19

This morning she had physical therapy for the first time in a really long time.  It was a 9am appointment, which means we have to leave the house no later than 8:30am and that really doesn't leave any time for traffic delays.  And I had no nurse.  As I was packing to go, I kept remembering more and more stuff that I should have with me, just in case.  I had to shake my head as I was carrying more and more stuff to the car.  How in the world am I back here, lugging all this crap around?

And I thought about a nightmare I had the other night.  In my nightmare her trach came out, she couldn't breathe and I didn't have her go bag with me (emergency kit with back up trach stuff).  I had picked her up out of her chair and carried her somewhere, so I didn't have the hands to carry her go bag.  Anyway, it was a terrible dream and so real in so many ways.  So, you can bet your butt I'm not going anywhere without that go bag.  And yesterday at her wound care appointment, her sats were rather low and I didn't have her oxygen with me.  So, I ran back inside and grabbed an oxygen tank, just in case.

Just walking to the room from the car left her breathing really heavy.  So I checked her sats and they were 73!  I went back to the car and got her oxygen and put her on it.  I guess any exertion right now is really hard on her body.

Tracy is awesome though and thought of things she could do anyway. And Harlie had fun.

October 20

Harlie had speech therapy today.  She actually had it twice - she had her school speech and her private speech after that.  Now that Harlie can open her mouth, she's been wanting try certain foods.  When we were in the grocery store the other day, she saw a box of macaroni and cheese that had Teenage Mutant Ninja Turtles on it.  She wanted to try it.  So I bought it.  Her nurse made it today so that it was ready when Amy got here.  Harlie got a spoon and wanted to put a big scoop in her mouth.  Of course we can't let her do that. So Amy found a small piece and let her put that in her mouth.  She gagged and had to spit it out.  She just doesn't know how to handle and move the food around yet.  But, she certainly tried.  I couldn't help but wonder if Harlie was disappointed. Had she looked forward to this and expected a better experience?  Maybe she thought she would just know what to do once the food was in there?  Maybe she thought it would be easier?  Amy put a bite in some mesh and let her chew on it so she could taste it.

October 21

We took the boys on a hike to Mt. Pleasant, Virginia.

November 2

I will pick up from there later. Still waiting for them to take her back for her DLB (direct laryngoscopy bronchoscopy).

Much love,
Christy

Airway issues.

Over the past week, Harlie has been having what I would call, episodes of respiratory distress. Just walking from room to room in our house was enough to cause her to start working very hard to breathe, retracting, abdominal breathing, and desatting (her oxygen saturation would drop from mid 80s to low 70s, sometimes high 60s). While she would recover fairly quickly once sitting down and catching her breath, it was still very unsettling. And it was very strange, because there wasn't a reason I could see. Her lungs sounded clear and she showed no signs of getting sick.

Her first episode was Thursday night. Since then, the episodes happened more frequently. And by a few days ago, she required oxygen around the clock, and her increased work of breathing was obvious, even when she was sitting calmly.

So, on Tuesday, I took her to see her pediatrician. She was not comfortable with her work of breathing, either. And she said it sounded all upper airway, like it was obstructed, and she needed to be scoped this week.

So, we went home and I emailed her ENT in DC. I told him what was going on and asked him if he could see her this week. He said I could bring her at 1pm the next day, Wednesday. Awesome!

We've also been noticing that her heart rate has been dipping unusually low lately. She has a pacemaker and it is supposed to keep her heart rate above 80bpm during her awake hours. We have seen her heart rate as low as the high 40s and low 50s during those hours. Since I was going to DC, I thought I would see if I could get in to see them, as well. Luckily, they could.

I was definitely worried about Harlie trick or treating that night. I didn't want her to keep getting out of her chair to go up to people's doors. I did not want her to have an episode while we were out. So, I asked our neighbors to please ask for candy for her. Which they were happy to do.

Harlie was Moana this year and Cooper was this pumpkin bobble head thing. Murphy was... well, I have no idea. He wore a silver drama looking mask , and was gone before I even got outside.

On Wednesday morning, we headed to DC for her appointments. We have a little superstition that if we are prepared to stay overnight, then we get to come home. But, if we aren't prepared to stay, then she will get admitted. So, we packed a bag.

Caylee (one of her nurses) went with us. Her ENT scoped her (put a camera down her trach) but saw nothing obstructing her airway below the trach. So, he said that there could be a granuloma or scar tissue obstructing around her trach tube. In order to know, she needs to be scoped from her mouth or nose. In order to do that, she needs to be under anesthesia. He said she was working too hard to breathe. He said he believes her trach size is too small. She's just not getting enough air.  So, he said he wants to scope her Thursday morning, and admit her today.

We weren't surprised. Listening to her breathe on the drive up was stressful. Honestly, I was relieved he was admitting her.

She wanted this baby care kit from the gift shop. She cracks me up.

Coloring in the emergency department (we had to get admitted through the ED).

We probably got to the ED about 4:30pm. And hung out there until about midnight. Then we got put on the heart and kidney unit about midnight.

Ahhh, finally some peace and quiet. That ED was pure chaos!

I don't know when she will go to the OR to be scoped, but they are assuming close to 7am. I really hope this is something easily fixable. I'll update when I can.

Thank you so much for your continued support. We would be lost without it!

Much love,
Christy xo