Summary of our Options

I am going to try to summarize in simple terms the choices we are dealing with in Audrey's medical situation, which will hopefully explain why we are heading to Boston.

Her inability to swallow is caused by a thick, complex scar in her throat from the early surgeries to connect her short, oddly shaped esophagus to her stomach.  This fast-growing scar has needed to be dilated, or stretched, every four to six weeks since she was released from the hospital last September, because it keeps growing closed so tight that even her saliva has a hard time getting through.    Because of this, she has gone back and forth from trying to learn how to eat and drink by mouth, to being completely dependent on the G-tube pumping infant formula directly into her stomach.  A dilation is a simple outpatient surgery done under anesthesia, and she usually comes home that day and recovers over the next three days.  We've done it eleven times since September.  Once, last winter, she had a small tear form in her throat during a dilation, which healed over on its own with heavy antibiotic treatment over the next two weeks.  (The antibiotic Clindamycin that was given to resolve the tear, caused her to develop a nasty intestinal infection called C-difficile, which gave her horrible pain for three weeks until it was finally resolved by probiotics.)

In June, during her eleventh dilation, the thick scar in her throat split and a wider tear formed in her esophagus, leaking bacteria into the protective membrane space around her throat (called the mediastinem).  The resulting inflammation in her chest caused a dangerous buildup of fluid both around and in her right lung.  A chest tube was placed to drain that fluid, but a tiny nick in her lung caused a new buildup, 60 mls of air around her heart.  A second chest tube was placed to relieve the air, and over the next three weeks she received heavy antibiotics and tests that have shown the leak beginning to heal.  We were released to home on the 21st of June, and have since then been giving her two strong antibiotics, one through her G-tube (once daily) and one through an intravenous "picc" line straight to her heart (every 8 hours).  We opted to go home with the picc line still in place, first because it was the only way to avoid being re-prescribed the Clindamycin drug that gave her such a terrible reaction last winter, and second because they can draw blood for her labs through it rather than putting her through the inevitable four or five-poke drama of trying to get blood out of her tiny veins.  Fortunately, it has worked and she hasn't had to go through all of that again like she did in February.  She is still not allowed to take anything by mouth, in order to protect the leak site so that it will heal over as completely as possible, and is also receiving only the minimum constant drip of milk through her G-tube rather than a faster pump of intermittent "meals" as we used to do before this hospitalization.

On this coming Monday, a test will be done to take a picture of her esophagus.  It will tell us two things.  One, whether her leak has healed, and two, whether her throat is getting tight again in spite of our precautions against any potential reflux.  The fact is, despite her having been medicated for it her entire life because of the commonness of bad reflux alongside her defect, we don't know whether she actually has bad reflux or not, but it could explain the reason for her scar growing back so quickly.  She doesn't have outward signs of reflux, but there is a so-called silent reflux that is also common.  The test that is usually done to determine it has not yet been done for Audrey because it is somewhat involved and we have had setbacks that prevented her receiving it earlier.  We anticipate that she will receive that 24-hour test at the hospital some time very soon, once the leak is determined to be completely healed.  If it turns out she does have bad reflux, it will explain why her throat keeps stricturing so quickly, but it will also tell us that despite essentially an overdose of Prevacid twice daily, she is still producing too much acid and should probably receive a surgery called a Nissen, where the stomach is wrapped around the base of the esophagus and sewn tightly to prevent any backflow up into the throat from the stomach.  If that happens, it could solve the problem of her scar closing down so quickly, but it could also cause a whole set of new problems from the Nissen itself.  We'll cross that bridge when we come to it.  It may also turn out that she does not have bad reflux, in which case we'll know that her scar growing so quickly is caused by something else.

Regardless, her scar in the meantime has become so thick and tough that dilations are becoming as dangerous as they are necessary.  Our surgeon had felt that an instrument called the Tucker Dilator was the best way to handle Audrey's particular needs, but now that we have had such a big problem develop with the tear, he plans instead to use a very gentle balloon technique to dilate her only as much as it takes to let her saliva pass through, not attempting to make room for any kind of eating or drinking through her mouth.

Our options are very limited at this point.  I researched every technique I could find for dealing with Audrey's problem, and they are few and far between, but five of them show the most promise, and the two of those which I feel are the most promising are not available in our region.  The choices are:

1. Placing a stent in her throat to try to force the scar to regrow in a wider circular pattern.  I discuss the technique in this post and explain that because we learned about a little boy in California, very similar story to Audrey's, who passed away as a result of a very similar stent procedure, we felt that it was not right for her.

2. Using opposing magnets on either side of her scar to try to push through and cut a circular opening in the scar.  This technique would be performed by a friend of our surgeon, who would travel here from Japan to make the attempt.  As innovative and fascinating as it sounds, I can see many reasons it might not work and could be in some ways very messy and involved.

3.  There is a technique called a "needle knife" procedure, where a tiny knife is inserted down the throat to carefully cut away scar tissue without needing to make any chest incisions.  (Think of it a little like cleaning out a green sweet pepper.)  This technique is not available in our region, and is typically performed by a gastroenterologist, or GI specialist, rather than a pediatric surgeon.  I know of one GI who performs this procedure at Cincinnati Children's hospital, as well as the GI Dr. Manfredi at Boston Children's Hospital Esophageal Atresia Treatment center.

4. Another technique similarly available only out of state, is often done in conjunction with the needle knife procedure, where small doses of steroid are injected into the scar tissue.  The steroid stops the overgrowth of the tissue and softens the existing tissue, allowing it to be stretch open with a dilation and stay open much longer.  It doesn't always work, but often does.

5. Our last resort is called a resection, and it could be done both here and of course back East.  We are trying to do everything we can to avoid another open-chest surgery, because her small body has endured so much and the recovery of a surgery like that is lengthy and intense.  But if all other methods fail, they will cut into her chest wall (usually needing to break a rib in order to reach the right area) and disconnect her esophagus again.  They would cut out the bad section that is causing the problem, and attempt to sew it the two pieces of her esophagus back together again to give her a fresh start.  Here is the problem.  Her first connection (the surgery is called an anastamosis) was done under a lot of tension, because the two pieces were still quite far apart.  The surgeon said it barely came together.  It has now been almost a year since that surgery, so we don't know what it is like in there.  Sometimes the esophagus quickly grows in a way that relieves that tension and would allow for enough flexibility to be pulled back together after a part is cut out, but it varies how quickly that happens, and sometimes it doesn't happen quite that way at all.   Let's say a resection turns out to be her best hope for eating normally.  The surgeons could find, once they get in there, that the good parts of the esophagus are too short to reconnect.  If that occurs, they will have a few choices to make.
       A) They could pull her stomach up into her throat.  This is called a gastric pullup and has been done numerous times successfully in many patients.  A person can actually go their entire life with their stomach in their throat (and you thought it only happened on roller coasters!) with the added bonus that they will always be skinny, because they have to eat small frequent meals and physically cannot overeat.  From what I've read, the biggest drawback is that stomach acid doesn't have far to go before it spills over into the airway, and the resulting damage over time can be devastating.  For reasons that are hard to explain, I don't feel good about this option for Audrey.
      B) A portion of her intestine could be used to replace the esophagus.  This used to be done frequently, but there are a number of problems with it that I won't go into here.  It works well in many cases but again, I don't feel it is right for Audrey.
      C) She can be medically paralyzed for two to three weeks while doctors in Boston use an innovative technique to stretch the two good ends of the esophagus closer until they can be sewn together.  The "Foker technique" is designed to rapidly grow esophageal tissue, and has been fairly successful for the surgeons at Boston, but unfortunately as of yet almost no one has been able to recreate their success elsewhere.  The doctors at our hospital in Salt Lake have seen problems with the technique and were very concerned about it for Audrey, but of course the Boston group says it is highly successful.  Personally, our jury is still out on this.  I will say that being paralyzed by drugs after her anastamosis last August, literally nearly killed Audrey.  Both of her lungs were almost completely collapsed for a time because saliva had pooled in her lungs, which she had been unable to cough out because of being forced not to move.  It is our most painful memory of this entire experience and is very challenging for me to think of revisiting.

So, those are our choices.  I hope it's clear why we are going the direction we are.  We want to save her esophagus in the least invasive way possible, so we are going to try everything we can before considering the major surgery for a resection.  The bottom line is, our best options for that approach are not available here.  This is why we are taking her East.