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