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Logan's Story

by: Logan's mom, Mara Lee

What a wonderful way to celebrate our anniversary! Just a year before, Fred and I were married in our beautiful mountain town of Breckenridge, Colorado. A year later I was shaking nervously as I completed the pregnancy test. We had just returned from our dream vacation to Europe when the EPT test confirmed that the hat I bought at the Monaco Grand Prix wasn't the ONLY souvenir we had returned home with.

Since we had waited until we were well into the "Advanced Maternal Age" category to start our family, we decided it would be easier on us if we could get pregnant along with our best friends. We excitedly called our good pals, Dick and Heidi, and being the good friends they are, they were pregnant within five weeks. Heidi and I were just giddy with anticipation. Our amnios told us we were both having perfectly healthy babies. Not a week went by that our husbands didn't catch us comparing our new cup sizes and tape measurements of our bellies.

About half way through my pregnancy, I remember the look of concern in Dick's eyes as he wondered if his wife was going to be as large as me in just five weeks. My robust size was the result of an excessive amount of amniotic fluid. It was during the first of thirteen ultrasounds that my doctors, William Fuller and Richard Perrico, suspected a possible TEF and told us we would be having a special child. Because of strong contractions, I spent the next 14 terrified weeks completely bedridden. My only adventure off the couch was to have Fred or a family member drive me to Denver for therapeutic amnios.

It was during the first of these that Logan let us know just what a fighter he would be. We watched as our son was shown to us on the ultrasound screen and the doctor explained he did this so as not to hit him with the needle. About twenty minutes into the procedure, Logan had had enough of this hollow instrument invading his space. We watched in horror as Logan reached up and grabbed the needle with his tiny little hand. I had a total of five "draining" and I lost 25 pounds before Logan was even born.

Logan was delivered during the worst snowstorm in years via an emergency C-section. The perfect ending to a delightful pregnancy. Since all of you reading this know the fear, anger, excitement and questions associated with having a child with a birth defect, I will skip that part and get to the reason of this letter; to explain the amazing corrective surgery performed on our son.

As Logan laid in the NICU for the first two months of his life, we waited in hopes that the 51/2-cm. gap between his upper and lower esophageal pouches would grow closer together. When barium tests showed that the gap was getting larger, Fred and I turned to the library at the University of Colorado Medical Center in Denver. We re- searched everything we could find on EA and through our research, found an article written by Dr. John E. Foker at the University of Minnesota. Dr. Foker detailed how he corrects severe EA by stretching the two ends until they meet and then sewing them together. His article stated that he had not done a colonic interposition since 1973 and has had a 100% success rate. Armed with this information we got a second opinion, then a third and a fourth. All of the pediatric surgeons in the Denver area told us the only way to repair Logan's extreme gap was to resort to a colonic interposition. I showed them the research I had done and only one, Dr. Jack Chang, was even remotely optimistic. Dr. Chang showed us the respect of calling Dr. Foker and after several phone calls, gave us his blessing and ordered the air ambulance to Minnesota

Logan's barium swallow showed a gap of 71/2 vertebral spaces. Dr. Foker explained that he would do his best to get the two ends together on the first try, but if this was not possible, he would then put Logan's esophagus ends on traction and pull them towards one another. The gap was too severe and Dr. Foker turned to Plan B.

With Logan sedated, Dr. Foker took the end of the suture attached to the upper pouch and threaded it through his chest cavity and brought it out a hole near the lower portion of Logan's back. He then took the end of the suture attached to the bottom pouch and brought it out through a hole in the upper portion of Logan's back. The sutures crisscrossed in Logan's chest and, when tightened, pulled both ends towards one another. Dr. Foker then threaded the ends of the sutures through button-like restraints over the holes in Logan's back, pulled the sutures tight and secured them with knots. The restraints acted like stoppers that prevented the sutures from being pulled back into his chest.

Logan was then put into a drug-induced paralysis to keep him from moving and a respirator breathed for him. During the next ten days, Dr. Foker would pull on the sutures atop the "buttons" until he could insert an ordinary piece of plastic tubing. When he had inserted enough tube sections under the sutures to stretch the two ends of Logan's esophagus far enough, Dr. Foker and an assistant took Logan back to the operating room and successfully attached his two ends together.

Fred and I consider Dr. Foker to be a bit of a miracle worker. Although many in his specialized field will not believe a procedure such as his can work, we have proof in a handsome little six month old who is enjoying the pleasures of eating for the first time in his life. Many surgeons are taught that anything over a 31/2 cm. gap cannot be pulled together. I am writing this letter to give hope to other parents whose child is considered an "Ultra-long Gap."

We read articles from every country in our research and they all told us the use of the child's own esophagus is best. The esophagus is a muscle and appears to have a substantial tolerance for being stretched, if done properly. Dr. Foker's procedure is elaborate but Logan's experience proves what can be done.

It has been hard work to teach him to eat and use his newly attached muscle, and this is still ongoing. Each day he relies less on his Gastrostomy feedings, and after 70-80 days of therapy, he is now receiving 70-80% of his food through his mouth.

We are thrilled with the outcome, as it has been 21/2 months since the procedure and he hasn't even needed a dilation yet. Logan will undergo heart surgery in the fall to correct his VSD and we should be on our way to normalcy.

Logan's Story - The Medical Community Responds

Comments from Logan's surgeon - Dr. Foker

The story of Logan and his parents' efforts is very heartening for a number of reasons. The problems that may accompany infants born with esophageal atresia are potentially great. Understandably, the reactions of parents to this situation vary widely. Some decide the issues are so complicated they leave everything to the doctors while the other end of the spectrum is represented by Logan's parents. As with any approach that seems to violate basic principles, this operation is rightly greeted with skepticism. The details of the repair are extremely important and a clear understanding of them and the potential pitfalls is necessary.

Not only were they unwilling to leave the decisions to the doctors, they actively sought out the available information on the subject. They read much of the current medical literature and even waited on articles when the surgical volumes were at the bindery. As a result, they made the basic decision regarding the method of repair for their son who was born with pure EA and a very long gap between segments. They made the choice that their son would have a true primary repair. His esophagus would be joined together without pulling the stomach up into the chest or using circular incisions in the upper pouch to cut muscles of the esophagus (circular myotomy).

What was remarkable to me was the amount of faith they showed. Their advice, with one exception, was that the goal of primary repair was impossible. Their faith extended to going to another state for an operation by someone they had never met, which makes it all the more remarkable.

As can be seen by the accompanying x-rays provided by Mara Lee, Logan had a very long gap of over seven vertebral spaces. Elsewhere, his choices would have been stomach pull up, gastric tube, or colon interposition. He underwent a primary repair which began with placement of traction stitches and, when the esophageal ends came together, a true primary repair was carried out without difficulty.

As is often the case after EA repair, and particularly with a long gap, reflux from the stomach up the esophagus seems to be increased. An anti-reflux operation (Nissen Fundoplication) was later done. As can be seen from the second x-ray, Logan's esophagus has been joined together and the stomach remains below the diaphragm. I believe that the control of reflux is important for these children and with the stomach in the abdomen, acid reflux should not produce damage to the esophagus over the succeeding years.

There are two major principles regarding EA surgery that virtually everyone believes to be true. The first is that the infant's own esophagus will be best if it is at all possible to use it. The second is the surgical principle that the anastomosis - or joining of the two ends of the esophagus or stomach or colon or whatever - should not be done under tension. Breakdowns and leaks of saliva are thought to occur if the repair is under a lot of tension. I believe the former is correct but not the latter. I have proceeded under the assumption that in order to use the infant's esophagus, the anastomoses will often be under considerable tension but the quality of the surgical repair will determine the result. Thus far, tension has not proven to be an obstacle in
achieving a good primary repair. Although this is extremely heretical to many, and mildly objectionable to others, the results should be the arbiter.

A child who preceded Logan proved in my mind the validity of this approach. His gap was over eight vertebral spaces but even his very small esophageal ends could be stretched into a position of primary repair. He was the first infant in whom I placed traction sutures into the esophageal ends and brought them out to the skin's surface. Traction can be increased over 7-10 days' time and the ends stretched sufficiently so that they can be joined together. Although these repairs were done under considerable tension, the anastomoses were feasible and held together without leaks.

As with any approach that seems to violate basic principles, this operation is rightly greeted with skepticism. The details of the repair are extremely important and a clear understanding of them and the potential pitfalls is necessary. Nonetheless, I am very enthusiastic about this repair and the results speak for themselves. I believe it will meet the important goals of a good quality of life over the expected life span. Of course, time will tell.

Sincerely yours,
John E. Foker, M.D.
Professor of Surgery

P.S. Most parents wish to learn about the basic issues of EA and the specific problems relative to their child. Recently, I have published a chapter on EA which may serve as a reference source for your support group. I believe it is the most complete recent article on this subject and contains almost 300 references which will allow original articles to be easily located. Not only are all of the preoperative and short-term problems discussed, but I made an attempt to assess the long-term consequences of the various operative solutions for EA. As any parent knows, but doctors sometimes forget, the goal for these children is to have 70 or more good years of life.