CT Scan Date

We now have a  date for the CT Scan.  It seems like it's a really long time off... March 8th?!  But there is a particular doctor the cardiologist and the pediatrician want to have do it and she is on vacation.  *sigh*  I'm sure it will go fast but it seems impossibly long. 

The test will start at 7:30 AM and she can't eat for 6 hours prior.  She will be completely put under general anesthesia.  Hopefully this test will be clear and definitive enough to give her surgeons all the information they need to make her well!

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!

The Diagnosis

The first visit with the Pulmonologist went fairly well.  He confirmed that he did want to do the Sweat Chloride test and that he wanted to do the Barium Swallow.  He assured us that the most likely explanation for all her symptoms was that the RSV had caused asthma.  He ordered the tests and prescribed Pulmocort (an inhaled steroid used to treat asthma) and told us to follow up with him in 3 weeks.  He said (like her pediatrician) that CF was an unlikely diagnosis because she had never fallen off her growth curve.  Still we were nervous and her 15 month well baby visit then next week did not encourage us because she only gained 3 ounces between 12 and 15 months.  Fortunately the Sweat Chloride test was negative. 

We were feeling fairly confident at that point that she just had severe asthma as the steroid seemed to be helping a great deal.  On February 8th she had an appointment to do the Barium Swallow.  Basically they strapped her to a rotating board under an x-ray machine and fed her a drink laced with Barium (which shows up bright white on an x-ray) through a syringe and watch her swallow it.  This checks for any reflux or swallowing issues.  It also checks to make sure, as her pediatrician explained it, that her esophagus is nice and straight.  My husband and I were able to see the x-ray on the screen as it was performed.  We talked about it as we were leaving.  "Did you see that part that looked like a dent?" we said.  We had both seen it.  But we tried to ignore it.  We aren't doctors after all. 

Later that afternoon we received a phone call from the Pulmonologist.  This is odd for two reasons.  One being that he said we 'should' have the results by our appointment with him on Friday.  It was still Wednesday, just hours after the test was run.  Two being that we were told we'd have the results of the Sweat Chloride test the same day and it took us two days to get the result.  He told us that the Barium Swallow had shown what's called a "Double Aortic Arch".  Something I'd never heard of.  Something we weren't expecting.  Basically it's a rare congenital heart defect where her aorta didn't form correctly and she now has two branches of it.  One of these branches is going between her trachea and her esophagus and putting pressure on them.  It makes it hard to breath.  And it makes it hard to swallow.  It makes her more at risk of lung infections and choking.  It has to fixed by surgery. 

As of today we are waiting to see the pediatric cardiologist.  This appointment is scheduled for Wednesday February 15th.  We will learn more then.  We are still a bit in shock.  I will write a post later explaining what Double Aortic Arch is when we know a little bit more about her specific anatomy. 

Right now it seems as if the prognosis for a full recovery are really good.  She should come through with a scar but with few other long term effects.  We are still nervous and upset that she will have to have a major surgery to fix her heart, which never goes without some inherent risk. All we can do is put our trust in her team of doctors and help her be strong.

 Getting ready for the Barium Swallow

The First Year+

Della was born at 36 weeks gestation weighing just 5 lbs 10 oz.  Other than her rather rushed and early arrival she was perfectly healthy.  She was breathing and nursing beautifully.  We took her home with us 6 hours after she was born.

The first several months with Della were the same as they are with any newborn.  She slept.  She ate.  She pooped.  She gained weight and while she was always small she was always on her growth curve.  There was nothing at all to worry about.

In February of 2011 her brother Liam, who is only 14 months her senior, was diagnosed with RSV. We didn't even know. I took him for his well baby visit at 18 months and the doctor listened to him and surprised me with this news.  He listened to Della and said she sounded great but to try to keep him away from her as much as possible.  We did try.  But with a toddler it was next to impossible.  Within 2 weeks Della was also diagnosed with RSV.  She had a much harder time kicking it but we were fortunate to not have her hospitalized and she did not develop pneumonia.  She was never hospitalized and did well with Albuterol breathing treatments through a nebulizer.  Her doctor said it would take weeks for her to be completely well and that's when the trouble started.  Della never got "well".  For the next 11 months she would be called "Vader Baby" because she always breathed like Darth Vader from Star Wars.  Her pediatrician listened to her every time we were in his office, even if it was just a visit for her brothers.

Over the summer Della sounded slightly better.  She has always been healthy and active and happy.  She was small, but growing steadily.

Just before her first birthday Della came down with her first cold of the season.  It was nearly as hard on her as the RSV had been the spring before.  She was having trouble breathing and was wheezing and coughing.  We increased her Albuterol treatments and her doctor put her on Prednisone (a steroid) for 5 days.  The steroid didn't seem to help much though she got slightly better on her own after several weeks.  She was at the doctors office several times and while she never developed pneumonia she never seemed quite 'right'.

Come December and we take our noisy baby on a road trip to Idaho to visit her paternal grandparents for Christmas.  Christmas Eve we were driving from Las Vegas to SE Idaho and we pass by Mesquite in the morning.  We were handing out cookies to the kids.  We know we shouldn't but we had lots of excuses which seemed justifiable at the time.  The normal things, we can't stop every hour to let them have a snack, it keeps them quiet and makes the trip go faster.  Della is rear facing still.  We can't really see her.  We handed her a cookie which she took willingly.  A couple minutes go by and she is fussing and crying and she throws the cookie.  We decide she probably doesn't want a cookie so we hand her something else (I can't even remember what it was).  She immediately threw it and was trying to cry.  My husband turned around to look at her and our whole world nearly stopped. She was choking.  She wasn't breathing.  We stopped as fast as you can pull a van over that is going 75 MPH down a freeway.  It's all such a blur now but we got her out, we tried everything and finally after what seemed an eternity (2, maybe 3 minutes?) my husband gave her mouth to mouth and got her to breath again.  A ride in an ambulance to the ER followed by chest x-rays (which were declared clear by the ER technician) and we were on our way again, terrified and upset, to spend Christmas week with the family.  On our way home we were much more vigilant (no food in the car. Ever. Again.)  but Della would seem to get more distressed the longer we would drive.  She would sleep then when she'd wake up crying she'd start coughing and it would sound like the classic "seal bark" cough associated with croup.  We'd have to get her out.  Calm her down and sometimes give her Albuterol to get her breathing right before we'd limp our way closer to home.  It was a very long and stressful trip.

The same week we were traveling and getting home my maternal grandmother passed away.  We were home with Della only two days (though she did see her pediatrician to follow up with the ER and was declared healthy...though still a loud breather) before we were off on a plane to Washington for the memorial service.  The cold wet air did nothing to help her and she spent the entire weekend fighting to breath normal making everyone nervous and precipitating late night phone calls to on call nurses and middle of the night trips to Walgreens for a humidifier to try to help her breath.

It was with relief that I got her back to Arizona to our warm dry climate.  She seemed to improve and the next time she saw her pediatrician was mid January when she was running a fever (we always have taken her in with a fever because we are so leery of pneumonia).  This is where our life got interesting.  The pediatrician makes some comments that he isn't comfortable with the fact that she never seems to quite get better.  He says he wants to refer us to a Pulmonologist because he wants to put her on an inhaled steroid but he thinks we should get a second opinion from a specialist first (one of about a thousand reasons that I love her pediatrician).  He tells us that the Pulmonologist will most likely want to run a test called Sweat Chloride that will test for Cystic Fibrosis (CF) and possibly a test called a Barium Swallow to test for any reflux or structural problems with her esophagus.  We schedule the appointment for January 20th.  We spend the 10 days or so in a near panic practically convinced that CF is the answer to almost all her symptoms.

This is where I'll break off to the next post... just to keep things from getting too cluttered.