It may be time for an update.

 

At the pumpkin patch, October

I know you can't believe that I'm actually posting an update!  Ha!  And some random pictures that are only related to this post by virtue of Della being in them!

First a couple of housekeeping items.  One is that I changed the domain and the description of this blog.  When I first began this blog I chose those based on Della's initial diagnosis.  Since that diagnosis was changed I've just been putting it off due to pure laziness on my part.  But since I wanted this blog to exist to potentially help others with children with similar heart defects I thought it was important to have all the titling correct.

Now for the actual update on Della.

Today we are almost 8 months post surgery. Della is 24 months old.  She is doing fairly well, though I have to admit she isn't doing as well as we had hoped.  For the first few weeks after the surgery we expected things to be pretty rough.  And they were.  Della was in a lot of pain from the incision and from the bruising where they had to spread her ribs apart. She was doped up on medication for the entire first 2 weeks post surgery.

We were told that the all the stitches used were the dissolving kind.  But the stitch that was where the chest tube was did not show any signs of coming out so after 10 days we had my dad cut it out.  (He was a medic in the Air Force so he knows what he's doing...don't worry! :) )  Then around four weeks post surgery Della developed what looked like a small pimple at the very top of her incision site.  We looked at it and poked at it and it seemed to be only mildly painful but it also appeared to be a stitch coming out of the incision.  When we called her surgeon he said that was probably exactly what it was and to just wait and it would pop out on its own.  And that is exactly what it did...eventually.  As in on July 4th! It finally popped out and with some luck that one little spot will no longer bother her as it has been (I think it must have just poked her just wrong sometimes).

With Daddy, petting a donkey!

The main issues we seem to be having with her are all related to her breathing.  We had such high hopes that this would be a fix it and forget it kind of thing.  Well, at least sort of forget it.  But as it stands right now we were off a little there.  Della still has very noticeable stridor (the gurgley breathing, it basically sounds like she needs to cough, but coughing does nothing to help her).  So she is still on Pulmocort twice a day which she inhales using a nebulizer. Right now she has a cold so she also is on Albuterol twice a day and we do chest percussion to try to help her clear the mucus from her lungs.  We also have a supply of Prednisone (a steroid) for if she gets a particularly bad cold.

In September we had a flexible bronchoscopy done at the recommendation of her pulmonologist.  This was to check out her airway and see if they could see exactly why she still struggles with stridor.  It turns out she has "significant tracheal malaise" right where the trachea splits to enter each lung.  Tracheal malaise is a fancy word that means her trachea is crushed and squishy right there.  It is really bad right above where the branches split and then the tube going into the left lung is ok but the tube going into her right lung is also crushed. 

So what does this mean?  Basically it means that she can't cough effectively.  Think of a cough as a violent wave that originates in the lower lobe of the lung.  When a healthy person coughs that way pushes all the mucus up and out of the lungs into the trachea and, with luck, out into your mouth so you can get it out of your body.  When Della coughs the 'wave' disperses and loses all its power at the site of the malaise. So she essentially never is able to clear her lungs. 

We have been so extraordinarily lucky because she has never had pneumonia!  But this does make our job this winter to keep that statement true.  She was also approved by her insurance company to receive the RSV vaccination as getting it (again) would pose significant risk to her.  We are very fortunate because it is so expensive (around $1000 a shot and she'll need 5) and insurance companies rarely approve it for kids over 2.  Thank goodness  her doctors were able to successfully appeal the original decision and demonstrate to the insurance that it was medically necessary.   

 

Della as Little Red Riding Hood on Halloween, with Mommy.

 

As for her heart she still has an ASD (atrial septa defect, or whole between the atria).  She had another echocardiogram at the cardiologist last week.  It appears that it is a tiny bit smaller than it was at her last appointment with him in May but that is to be expected as they close naturally the first two years of life.  But hers is still too large and the right side of her heart is slightly enlarged as well.  We will go back to him next November just after she turns three and he will look at it again.  He said there is a very small chance it could continue to close but that it's not very likely.  Probably we will be making a plan at that point to go in and have it closed.  This procedure will be outpatient and will be done with a catheter.  They may wait and do it after she is four as long as her heart is not getting more enlarged on the right side, just to let her get a little bigger. 

And speaking of her size brings me to the final point of the post.  Della is still very small.  It was her not growing between 12 and 15 months (at all!) that got her pediatrician worried and set us on the path to her diagnosis.  At 2 years old she is 30.5" and 23 lbs 6 oz (although the weight isn't entirely fair because she has  a full arm cast right now because she broke it!).  But she is on the chart for weight.  She is no longer on the chart for height.  She had gotten back on the chart post surgery.  So now her pediatrician is concerned again.  We will go back in 6 months and have her weighed and measured again.  If she stays on her curve, even if it's off the chart, then it's ok.  But if she has flat lined on growth again we will have to look at why.  Again. 

So excited watching the pig race at the festival!

That is pretty much it in a nutshell.  I know it's been so very long, it's hard to keep up with this!  I really admire people who find the motivation to keep blogs updated so well! 

*And a special thanks to Aunt Brie, who took all these pictures! :)

You take the good with the bad....

Della had her first appointment with her new cardiologist yesterday.  We learned some potentially good news and we learned some bad news.  I'll start with the bad news to get it over with. 

When the cardiologist first listened to her heart he mentioned that he thought he heard a small murmur but that considering that it hadn't been noticed before (and she has an excellent pediatrician) it was most likely due to the amount of Albuterol she is on (and no, I have no idea why that would cause a murmur?...should have asked).  But after taking her history and talking to us about her symptoms he wanted to run a transthoracic echocardiogram.  This test was not at all painful for her but she wasn't too happy about it either.  Even the Baby Einstein movie they had showing wasn't interesting enough to keep her from trying to get away. 

The bad news is that Della has an Atrial Septal Defect (or ASD for short). 

(You can read more about this here)

Della's ASD is considered "moderate" and will be monitored.  Over time if it is left untreated the right atrium will become enlarged due to the extra blood flow into that chamber.  Most likely when she is 3 or 4 years old they will go into the heart with a catheter inserted in an artery in her leg to fix the hole.  This will be relatively easy and will not involve a stay in the hospital.  In the cardiologists words "She'll go in for the day and go home fixed with a bandaid on her leg."  That is pretty amazing.  So really, the "bad news" wasn't really all that bad.  

As for the potentially good news.  While the doctor was looking at her heart he said that he was not able to locate a left aorta.  What this means is that she may not, after all, have a double aortic arch.  He said that doesn't mean it isn't there, just that he couldn't see it with that test.  There is definitely something causing all these troubles to happen.  Based on what he was able to see he is suggesting it might be something he termed "Right arch with aberrant left subclavian artery".  This is still a type of vascular ring defect it's just different from a double aortic arch, and as I understood it, involves a much less invasive surgery to be corrected.  

(Image credit)

If this is the defect that Della has they will still have to fix it surgically but they will only have to go into her chest and cut ligamentum arteriosum (which you can see in the picture above).  Cutting this will allow the left subclavian artery the flexibility to move back and stop putting pressure on her esophagus.  This is obviously much easier than having to go in and remove a piece of the aorta.  

So where do we stand right now?  Basically we are still waiting.  We have the doctor who ran the Barium Swallow test who thinks she saw a double aortic arch with the x-ray, and we have the doctor who ran the echo test who thinks he saw only a right arch with aberrant left subclavian artery.  We don't really know for sure either way.  Both tests have large error margins.  We also don't know for sure, but we were told the doctor who did the Barium Swallow thought part of the aortic arch was going between the esophagus and the trachea rather than around them both.  So we don't know what will be involved if the left subclavian artery is between those structures?  Surely it will have to be removed, but how?  And how risky is that?  We simply don't know.  

From here we will have to wait a couple of weeks until we can have the CT with contrast run which will answer all these questions for us.  It is so incredibly frustrating feeling that you finally have an answer from doctors who don't make it sound like there is a question as to what her diagnosis is, to going back to not knowing what is wrong with her. 

Silly baby, just playing with her shoes and waiting for the doctor!