An Esophagus Story

Although able to eat, Ari's food repertoire is limited.  It can't be too fibrous, too chewy, too stringy, too sharp or too sticky... and we had to learn this all by trial and error.  It's a lesson in irony for our family... our child born without an esophagus wants to eat everything and our healthy child has an extremely limited palate.

One of Ari's favorite foods is apples.  I have tried every possible way of serving them to him.  Outside of thin applesauce, the chunks got stuck.  After the third emergency procedure to remove apple from his esophagus, his GI sternly told me "NO MORE APPLES."  Point taken.

On the contrary, fluffy scrambled eggs are easy for Ari to swallow.  The eggs that got stuck over the weekend were fried, not scrambled, and stored in the balloon-like section of his esophagus over the connection site, which narrows significantly.  Think of it as a kitchen funnel.  The first piece of food that is too big for the funnel blocks the opening and the rest of the food builds up on top of it.

To exacerbate the situation, Ari has something called a Nissen Fundoplication (Fundo).  Since he did not have an esophageal sphincter, his stomach is wrapped around the esophagus and secured in place, like a loop on a roller coster.  This keeps the food he swallows and his stomach acid from coming up.  The problem with a Fundo is two-fold.  First, Ari's peristalsis (the wave pattern of the esophagus during swallowing) is inconsistant and slow, if present at all.  He has to chew, swallow and then drink with enough force to flush the food down.   The second problem is that the Fundo limits the ability for him to throw-up.

When food gets stuck, Ari can make himself gag to regurgitate the food above the Fundo.  Water also helps carry the food back up.  He drinks and regurgitates water over and over again until the food dislodges and he swallows it or it comes out.  It's exhausting for him.

If the food does not come up and he has gone too long without fluids or if he starts to show signs of aspiration, we have to go to the ER.  Like all of his procedures, he has to be intubated on a ventilator while the surgeon picks out the food piece by piece, or as in this case, has to try to scoop it out.

Below is some history to fill in the blanks on how we got to this point and what a true miracle it is that he can actually eat.

When Ari was born, he did not have an esophagus, a condition called Esophageal Atresia.  Instead of a connection to the stomach, his esophagus ended in a balloon high in his throat.  There are 7 variances of this condition, Ari's being the most severe and called Pure Long Gap Esophageal Atresia because of the distance between the pouch and his stomach. 

From the moment he was born, he had a tube in his mouth that went into the pouch to suction his saliva.  If he did not have that tube, he could fatally aspirate.  At 1 day old, he had his first surgery- the placement of a G-Tube (gastric tube for feeding) and a colostomy.

For 11 weeks in the NICU, his surgeon came in daily to stretch his esophagus from the mouth with a red rubber catheter.  I hate that catheter.  To this day, every time I see one I cringe, flooded with the memories of seeing the tube stuck down my baby's throat, sharing space in his mouth with the suctioning tube and a breathing tube.

Ari's time in the NICU was unstable.  Ideally it should have been wait and grow period, but not for Ari.  His de-stat episodes were frequent- 20, 30, 40 + in a day.  Alarms would sound and the nurses would rush over to help him breath again.  Each time we wondered if "this was it".. the time we has to say goodbye forever.  We would  brace ourselves mentally, unable to take our eyes off the scene surrounding him.  We prayed for the day his surgeon would tell us "It's time to make the connection between his esophagus and his stomach."  When that moment finally came, we were ecstatic.

After 6 hours of surgery, I will never forget the feeling I had when his surgeon walked into the waiting room, surgical scrubs on, goggles resting on his head, asking us to meet with him in a private consultation room.  Immediately assuring us that Ari was fine, he explained that there was no way Ari's esophagus was going to reach his stomach.  Other surgeons joined him in the OR and they concurred.  As I mentioned in my last post, our worst case scenario came true... Ari had to get a spit fistula.  

A spit fistula is extremely rare.  Most doctors have never seen one and most nurses have never heard of it.  Ari's esophagus was literally pulled out the left side of his neck into a stoma (hole) to allow for drainage of his oral secretions.  Every time he swallowed, spit would come spurting out of the hole.  There is a video link on this blog called "Ari's Spit Fistula".  Check it out and you can see what happens when he drank juice.

Larry and I made the decision to teach Ari how to eat and drink at the same time he received his G-Tube feeds in an effort to create the association of a full tummy and oral satisfaction.  Oh boy, was it messy!  I think this early training contributed significantly to Ari's ability to eat today.

Due to other anatomical defects, we did not have any options for Ari to ever have an esophagus.   His surgeon will always be my hero for taking the risk to recreate a not-well-accepted procedure and make it work for Ari's anatomy.  In June of 2009, Ari "grew" an esophagus... it was nothing short of a miracle.

Despite the new esophagus, Ari was still tube fed.  His esophagus just wouldn't stay open for food to pass through.  He was going to the hospital to get an esophageal dilatation every 1-3 weeks, having to be intubated every time.  We ran out of options and discussed redoing the entire connection surgery again, this time removing the area that had narrowed.  One of the surgeons came up with the idea of 2 dilatations per week for 12 weeks.  Without any other options, we gave it a try... 24 times in the OR in 1 summer and no results.

Out of ideas, we scheduled the re-sectioning surgery for October 2010.  About 7 days before the surgery, Ari told me he was hungry.  Cautious, I fed him a snack.  He asked for more and I obliged, carefully monitoring every bite.  He was eating, and it was going down.  The power of a Miracle, the strength of a Lion, the grace of God.  Call it whatever you want... I call pure joy.  We canceled the surgery and worked on introducing new foods and textures to Ari.

I can't paint a prettier picture than our actual reality, though.  Food gets stuck, his peristalsis halts, and his esophagus randomly closes for no good reason.   Episodes like this weekend are frequent.  We continue to work on his chewing and swallowing and live with the fear that the choking could happen at a friend's house or at school, where the adults in charge will not be comfortable dealing with it.

And speaking of dealing with things... In the time it took me to proofread this post, Ari accidentally pulled out his button.  Calmly, I gathered the gloves and surgical lube, made saline, had Larry hold him down and Liv hold his hand.  I reinserted it, inflated the balloon inside his intestinal wall, and exhaled.

"Bed time kids!"  It's as if it never happened.  We certainly do live a different type of normal.