Our Ever Changing Life

I'm so sorry I haven't posted in so long. I wish I could say it is because we have been out having fun, living life to the fullest. Unfortunately, that isn't the case. A lot has happened over the past few weeks.

While coming/being home has been fantastic, reacclimating to our normal life has not been easy. I feel like I am trying to merge onto the busiest highway, in the crappiest car. Life doesn't care that you're beyond tired, or that your brain doesn't work as well as it used to. It doesn't care that you went through something traumatic. The boys still have cross country practice, swim practice and homework. The house still needs to be cleaned, laundry still needs to be done, pets still need tending to and I still have work to do at my part-time job. Life doesn't stop so you can catch up.

Here's what I started to write a couple of weeks ago:

Thursday, April 26

I don't even want to write this post. But, I suppose it does give you an idea of what this roller coaster of living a special needs life is like. One day you feel over the moon, the next, you're freaking terrified.

Things were really good for four days. Four whole days.

Therapy was going well and she was able to go three days, Monday, Tuesday and Wednesday. Then Wednesday night she had a fever. And she has had a fever every single day since. On Friday (April 20th), we took her to her doctor. She tested negative for the flu and strep. But, she put her on antibiotics to try and prevent a secondary infection.  She has missed six days of therapy now. It just kills me how everything is so difficult for her.

For the most part, she seems okay now. She spent several days in bed in her room. But, the past few days she's gotten up and come downstairs. Tonight, we took her with us for a walk with the dogs. The worst is that she has no voice. And she keeps asking what's wrong with her voice. It has now been eight days since her first fever and still no improvement in her voice. I guess I have to give her more time. But, I am so scared something went wrong. 

There's also been a new issue with her that I haven't been able to blog about. 

Back on February 8th, I started Harlie's breathing treatment and went into the kitchen. Keep in mind that at that time, we were unable to safely change her trach and were trying to keep her from plugging. Soon after the treatment started, she started coughing an awful sounding cough. It was a sound I had never heard before. It was so strange and alarming that I ran over to her. I could see something in her trach, like debris of some kind. I tried to suction it, but it was obvious that wasn't going to work. So, I grabbed it with my hand and pulled it out. 

It had a dark tip that was hard, like plastic but the rest was white, and rubbery and didn't break, like mucous would. It kind of looked like a worm. It was so odd, that I put it on a napkin and took a picture of it. I put it next to a business card for size comparison. 

Then, when we went to DC for her surgery on February 21st, I showed her ENT the picture. He said it looked like a cast and that I should talk to her cardiologist. 

So I did. She told me it is from Plastic Bronchitis, which can develop in patients with Fontan physiology and high cardiopulmonary pressures (Harlie has both). She also told me that the leading research for that disease is being done by Children's Hospital of Philadelphia (CHOP), and that we might have to go there. At that time, Harlie had bigger fish to fry (in the CICU) and we couldn't do anything about it anyway. I had to focus on other issues.

Fast forward to last week, our first week home. On Tuesday (the day before her first fever) she started coughing, making a weird sound. It sounded just like it did when she coughed up that cast in February. I tried to stay calm, but those casts can occlude her airway (which is terrifying). So, we worked really hard with saline in her trach and saline breathing treatments. The sound seemed to come and go. And in my scared mind, it was as if the cast was stuck below her trach. By Tuesday late afternoon, we decided to change her trach. There was a plug in the trach we removed and at first, I was relieved that maybe that was the problem. However, just a few minutes later, she started making that sound again when coughing. Ugh!

So, I emailed her cardiologist the next morning (Wednesday). At this point, none of her specialists here in town know anything about what happened in DC, or about the cast in February. Within 25 minutes, she called me. She explained some risks with Plastic Bronchitis (PB) and told me Harlie needs to see her pulmonologist asap. I told her I'm afraid that I'm being paranoid. She said I need to be hypervigilant.

Since then, I've been working on seeing him. We spoke via email the other day and he told me a little more about PB. He also included the Chair of Pediatrics, who is also a pulmonologist and is an expert in PB. He said this in an email to me:

The Children's Hospital of Philadelphia/Children's Hospital of Richmond plastic bronchitis consortium follows the largest number of PB patients in the world and the International registry for PB is located here at ChOr@VCU. I discussed management with Dr Schmidt this morning and have attached a brief chapter we recently published in the European Respiratory Society handbook. Happy to work with Dr Schmidt to keep Harlie well.

Our appointment is this morning at 10am. I read the paper he sent. And then I read some more about Plastic Bronchitis.

Here is a link:

http://www.chop.edu/conditions-diseases/plastic-bronchitis

Here is a picture of what a cast can look like.

I asked him if this was removed or coughed up. He said, believe it or not, adults can cough up something like this. This is what Harlie's looked like:

And this is the picture I took on February 8th.

So, are there more casts in there? Is this the cause of her exercised induced intolerance?

So, just to recap, Harlie now has heart defects, including a pacemaker, congenital lung disease, with only 3.5 lung lobes instead of 5, major airway problems and now Plastic Bronchitis (which is called a devastating disease in papers).

WHAT THE HELL?!

Any ONE of those things can be life threatening.

I just want my daughter to breathe easy and feel good way more than she feels bad. Is that too much to ask? Screw sports or other extracurricular activities, popularity, trendy, advanced classes, college, or anything else that most parents consider success in children these days. I want her to live and be happy. And honestly, that goes for my boys, too.

I have to pause. We have to go to our appointment. I'll let you know how it goes.

Friday, April 27

Yesterday we met with her pulmonologist, Dr. Schmidt and the Plastic Bronchitis expert, Dr. Rubin. Basically, he said if someone coughs up a cast, they almost always have PB. So, we have to assume she does. After reading the paper, and a bunch of stuff on the internet, I want to know if she has anymore casts in there, waiting to come up.  And I want to know, like now.

He said he was going to send a report to CHOP and ask for them to see her asap. I guess the first step is some kind of testing. The negative is that, according to what I read on CHOP's website, they use some special MRI. But, Harlie has a pacemaker, and cannot have an MRI. So, we'll have to see what they do in situations like this.

They want us to start one of the few treatments they think might help slow the production of these casts, inhaled Heparin (Heparin in a nebulizer). Dr. Schmidt gave me a really nice, heavy duty nebulizer. We have to give these breathing treatments every 4 hours for 48 hours, then we can go to 3 to 4 times a day. To put this possible diagnosis in perspective, Dr. Schmidt said he has never prescribed inhaled Heparin before.

Harlie's constant ability to take the path less traveled is exhausting. It makes me so fearful, because pretty much anything is possible. There is no comfort in the words rare, almost never or unlikely.

That night, I went in to say goodnight to Harlie. I could tell she needed to be suctioned, so tried to suction her. The catheter couldn't get through. Her trach was completely occluded. She grabbed the catheter out of my hand and tried to suction herself (as if I was doing it wrong). She got the same result, and looked at me like, "WTH?"

So, I called Tom and said we had to do a trach change. I had a very hard time getting the trach to go in. So much so, that I said to Tom, "I can't get it in." I could tell she was getting uncomfortable, so I finally just had to force it in. She bled and cried. It was terrible. And so unsettling. Tom hung out with her and they looked at photos on his phone until she was better.

I emailed her ENT and let him know what was going on.  It felt like all the positive from her surgery in February was gone.  It was crushing.  He said he wanted to see her the following Thursday.

~~~~~~~

Unfortunately, I don't have the time to give this post the attention it needs.  So, I'll be really concise to try and catch you up from here.

It is now May 10, and I've written this post over the past couple of weeks.  It has been hard to write because I've not been in a good place.  I have been so fearful of losing her, and that is very difficult to live with every day, month after month.  It wears on you.  And it has made me pretty angry.  I found myself thinking that what is being asked of her and us, is just too much (inhuman, really).  I mean, we are only regular people.  How much are we supposed to handle? I'm screaming uncle and no one is listening.  Anyway, it hasn't been pretty.

But, we went and saw Dr. Preciado last Thursday and it was a good appointment that made me feel a lot better.  So, I am better.  Plus, Harlie is better.  She is back to her cheerful, happy, more energetic self and so I am so much better. I am still fearful, but way less than I was.  I will fill in the gaps tomorrow while I'm in the hospital.  We are headed back to DC tomorrow for a scope of her airway and her lungs (to see if she has anymore casts in there).  Which means that I might get some answers very soon!!!  And I am very glad about that!

So, keep your fingers crossed that her lungs aren't a mess.

Thank you, as always, for your support!

Much love,
Christy xo