Blessed are you, Hashem, our G-d King of the universe, who fashioned man with wisdom and created within him many openings and many cavities. It is obvious and known before Your Throne of Glory that if but one of them were to be ruptured or but one of them were to be blocked it would be impossible to survive and to stand before You. Blessed are You, Hashem, who heals all flesh and acts wonderously.
blessing of “asher yatzar”.
“What’s this?” I asked my secretary Jo.
“You have a new consult, it’s the old chart.”
“Chart? That’s a phone book.”
Kathy was 18 when I first met her, on her way to college in a few months. She and her parents travelled from Connecticut to see me. Kathy was pale, quite thin and terribly shy, but she had a wonderful smile, mischievous and warm at the same time. I had not read her chart before and had no idea why I was seeing her so I asked her to fill me in.
Kathy was born with an esophageal atresia and a tracheo-esophageal fistula. Instead of being one continuous tube, her esophagus was incomplete, with a separate upper part that ended in a blind pouch and a lower part that had an abnormal connection, a fistula, to her trachea. When these children are born they are unable to swallow anything due to the blind ending upper pouch. They are at risk for lung infections because they aspirate their saliva into their trachea. Acid and gastric contents can travel into the lungs through the fistula between the lower esophagus and trachea. Kathy required an operation within the first days of life to connect the two ends of her esophagus and separate the fistula between her lower esophagus and trachea.
She had her operation at another hospital, performed by a very experienced surgeon; a leader and pioneer in our field. Nevertheless, she developed post-operative complications including a leak in the area where the two ends of the esophagus were sewn together. The leak never healed and the surgeon did not believe that the esophagus could ever be repaired again, so he chose to bring the end of the upper esophagus out through her neck. Something called a “spit fistula”. Now, when Kathy swallowed, whatever was in her mouth passed through the upper esophagus, out her neck and into a bag. It wasn’t pretty but at least she could swallow. In addition, a gastrostomy tube was placed into her stomach exiting out from her abdominal wall. Liquid formula was instilled every few hours to give Kathy nutrition so she could grow and develop normally.
Kathy’s trachea was very soft and would collapse when she inhaled. She required a tracheostomy in order for her to breath on her own without the assistance of a ventilator. By the age of 1 month Kathy had 3 separate holes in her tiny body; the spit fistula, the tracheostomy and the gastrostomy.
After several months in the hospital, Kathy went home and did well. She lived for the first few years of her life getting all her nutrition through the gastrostomy and the tracheostomy for breathing. She required more than the usual handful of care a baby her age needed yet she grew and developed quite well. At the age of 2 she had another operation to replace her esophagus in the hope that she would be able to eat “normally” and could be fed by mouth.
This time a different surgeon chose to replace her esophagus with part of her large intestine. One end of the intestine was sewn to the upper part of her esophagus and the other end to her stomach. The blood supply to the colon was left in place, originating from a single artery in the abdomen. Food would now travel from the upper esophagus through the colon to the stomach. It doesn’t quite approximate normal swallowing but is better than having the spit fistula and gastrostomy tube for the rest of her life. Kathy recovered from this operation and in subsequent years her trachea improved to the point that she was able to have her tracheostomy removed. Her new esophagus never worked as well as the surgeons had hoped and she wasn’t able to eat more than liquids.
As Kathy grew, so did the piece of colon in her chest. With time it became quite long and tortuous curling up and folding on itself. It became more and more difficult for food to pass into her stomach, getting stuck in the coils of the colon. Any liquids that Kathy drank would pool in these coils and not make it to her stomach. In order to compensate Kathy would have to bear down, as if to have a bowel movement, grunt forcefully, as if she was clearing her throat, increasing the pressure in her chest and “pushing” whatever she drank into her stomach. Eventually this stopped working and nothing would pass. Kathy stopped eating or drinking to try and minimize her discomfort and was now being nourished completely by her gastrostomy.
Despite this, Kathy lived a fairly normal life for an 18 year old. She attended a local public school and participated in several outside activities. Photography was her favorite.
“So, what brought you here today, what’s different now?”
“I am starting college in the fall and I want to be able to eat with the other students”. Kathy was a normal 18 year old on her way to college and didn’t want to feel any more awkward than we all do when we start. She was an accomplished photographer, having her own website, and was accepted to a prestigious New England university with a very strong fine arts program. She planned on pursuing her dream of becoming a professional photographer. Having to eat through a tube, secretly, and not being able to enjoy the usual late night “munchies” with the other students would, I imagine, make an already shy 18 year old all the more self-conscious and detached.
I reviewed her previous studies and ordered a new barium swallow. The problem was obvious. Kathy’s colon had become so long and tortuous that it resembled a rope coiled on itself sitting in her chest. There was no way any food would ever get through that. It was clear that Kathy would need an operation to replace her “neo-esophagus” and it was obvious to me that I was in over my head. Way over. I had minimal experience with this type of patient in the past and didn’t think it fair to undertake such a procedure on my own.
There was however a very senior pediatric surgeon who had recently retired from his position as chief at a major university hospital who did have a lot of experience with this type of problem. During his 40 plus years as a pediatric surgeon he had reconstructed several long standing, complex esophageal problems like Kathy’s. I called and reviewed the patient with him and asked if he would want to come to New York and help me. As luck would have it he was good friends with the surgeon in chief at my hospital, so getting him the necessary privileges to work with us wouldn’t be difficult. He arrived he following week, reviewed the patient’s case with me and offered several possible treatments.
Our choices were to remove the colon already in her chest and replace it with another piece of colon. Recent data looking back at patients who had their esophagus replaced with colon showed that they did not work well in the long run, which meant that Kathy might be right back in the same place in a few years. The next choice was a “gastric pull up”. Removing the colon, pulling her stomach up into her chest and sewing it to the beginning of the esophagus. This procedure seems to have better long term results than the colon interposition but still represents a major abdominal and chest operation. The third operation that we discussed was one that I had never heard of. My colleague had done it once before in a similar patient and was happy with the results. It represented less of a surgical “stress” for the patient and used neither a new piece of colon or the stomach, leaving those options available in the future if need be.
The colon is like a long piece of tubing composed of 3 layers. The innermost layer is called the mucosa and is responsible for much of the colons’ absorptive function. The next layer, the muscularis, is composed of muscle, and is responsible for producing the coordinated contractions of the colon known as peristalsis. The outermost layer is the serosa and acts as a protective cover. The operation my colleague proposed was to open Kathy’s abdomen and carefully deliver the redundant colon into the abdomen. We would then peel open the outermost two layers of the colon and scrap off the innermost layer, the mucosa, thereby shortening it from the inside without touching the outside. As the blood supply to the colon comes from the outside we would be shortening the colon functionally without compromising its blood supply. We did this for a length of about 8 inches.
Kathy’s operation was long but went as smoothly as we could have hoped for. Her post-operative course was somewhat complicated but she was discharged home about three weeks after her operation. She deferred her college admission for another semester so that she could recover fully before starting school. After about two months at home we decided to let her start drinking liquids. The problem was, it had been so long since Kathy drank anything, she had forgotten how. There are special occupational therapists that work exclusively with children and adults who, for a variety of reasons, have not eaten for prolonged periods of time and now need to be “taught” how to eat again. Kathy worked intensely with her feeding therapist over the next several weeks and showed up in my office with her parents about a month later. She looked great. They all couldn’t keep a straight face, smiling and giggling with big Cheshire Cat grimaces. It seemed like they were hiding a secret that only I didn’t know.
“What is it?”
Kathy opened her backpack took out a small bag of cupcakes and a container of frosting. She then proceeded to spoon some frosting on a cupcake and eat it. Three bites and it was gone. “Wow, is this good, do you want one?” I couldn’t believe it. For the first time in her life Kathy was able to eat like a “regular” kid.
I saw her again a month later and she was still doing great. Her whole disposition had changed. The shy, somewhat downtrodden and introverted teenager had become much more animated and happy. Now she’s always smiling.
Kathy fulfilled her dream and became a photographer. She has a great website with lots of her photographs posted on it. Many of the pictures chronicle her lifelong relationship with food. I look at it often and send her e-mails periodically to see how she is doing and if she still remembers me.
“Hey Dr Arkovitz
It would be pretty hard for me to forget you! I’m doing great. Eating is going well, having fun with it although I still have my G-tube. I’m also still in Boston going to school for photography. I’m in my junior year and loving it!
I was there for Kathy’s surgery, her post-operative course and her time at home. I think about the turn of events that came together perfectly to have such a remarkable outcome. My hospital employed eight pediatric surgeons, yet I was lucky enough to meet her and her parents. I was also lucky enough to work at a hospital that had an operating room and intensive care unit that could handle this case. By chance I had a relationship with a surgeon who, by some miracle, actually had experience with Kathy’s unique type of problem and was willing to help and, of course, the operation worked. Kathy’s life and her success was more a function of God’s providence than surgical skill, at least mine. I still consider the fact that I was able to meet this wonderful child and be involved in her care a blessing.