I am, once again, a day late. I knew the letter was ‘G’ and had no doubt what I would be writing about. This one is emotional and personal to me. I am sure that most people think GERD is a minor problem that most people deal with from time to time. Who dies from heartburn? Well, this is the cause of my son’s death when he was thirty years old. And why I have this blog, to shout out to the world that children are not treated well in our current healthcare system and to remind all practitioners working that our children are people too. Also, that their families who have been living through it with them are overwhelmed, exhausted, and confused. There is another way and it is my mission to introduce my vision and continue fighting for change until it is realized. That is another subject for another time.
Alex, my son, was born in 1977. We were told that he was perfectly healthy. When we came home with him two days later, we noticed that his breathing was so loud that you could hear it all over the house and that he had a sunken chest (Pectus excavatum), a congenital disorder that could be an indication of certain diseases or could cause problems on its own. I would bring it up to the physician on our monthly visits and he would place an infant sized nasogastric tube down into his lungs to show me that there wasn’t any blockage. By the time he was two years old, he was diagnosed with asthma after I had made another trip to the ER to find out what was wrong with him. This time they put an endotracheal tube down with a ventilator and kept him for three days. You are probably wondering what this has to do with GERD. But you will soon understand.
The Emergency Room visits and hospital stays continued for his asthma. When he was ten years old, he had a problem swallowing while he was eating a plum. He was in pain and there was frothy saliva coming from his mouth along with mucus. The doctors took him into surgery and did an endoscopy (placing a tube with a tiny camera at the end) to determine what was stuck in his esophagus (a tube that carries food from the mouth to the stomach). When they came out to talk to us, they told us that it was a small plum seed and that his esophagus was inflamed (called esophagitis) with strictures and he should see a pediatric gastroenterologist. We were referred, and from there Alex was sent from test to test. There was a Barium Swallow with hamburger, a 24 hour ph test, and another endoscopy. We drove miles because some of the tests were only offered in distant cities. Before all of the testing was done, Alex developed an ulceration in his esophagus. That was his first of multiple blood transfusions. He was first put on a liquid diet that was sold to us by the doctor at our well known HMO. He thought that if Alex lost weight, he would be fine. He continued going to the ER for his asthma and his esophageal problems. He was eventually taken off the liquid diet and put on a diet eliminating foods that might trigger GERD such as citrus fruits, spicy foods and fried foods and sent to a nutritionist. It was then that the multiple medications started. There were H2 blockers to help block the production of stomach acid, proton pump inhibitors that reduce the amount of acid the stomach produces. Nothing was helping so we went on to stronger drugs called prokinetics to reduce the number of reflux episodes by helping the lower esophageal sphincter (LES) muscle work better and the stomach empty faster.
I admit that it is a rare case that one is not helped by at least one of these medications, but Alex was a rare kid. During the time that he was having his allergy shots, I asked the doctor if he believed there was a correlation between the asthma and the GERD. Of course, he said yes. I wondered why they could not have made the diagnosis much earlier and he told me that if he was a 50 year old man, he would have known right away. Babies are diagnosed with GERD everyday. Alex’ asthma was caused by the acid from his stomach spraying into his lungs. So we continued with the many hospital stays and ER visits until he was 18. At that time they were forced to do a surgery called a Nissan Fundoplication to create a valve at the top of the stomach by wrapping a portion of the stomach around the esophagus. Now, we would be able to send him off to The Boston Conservatory to play the clarinet, his dream since fifth grade. We were so excited. We left him off after getting him settled and attending the parents day thinking that all would be well. Within seven days I received a call from Beth Israel Hospital with a doctor telling me that Alex was bleeding, they started a transfusion, they did an endoscopy and saw numerous ulcerations and strictures and they felt that he should come home. Eventually we learned that the surgery had failed. It fell apart in two weeks.
Needless to say Alex refused to come home. He felt at home there. Everyone at the school loved music as much as he did and the best part was that he could play the clarinet all day and all night. So he stayed and made his frequent ER visits and hospital stays, but when he was needing endoscopies once a week just to dilate his esophagus enough to swallow food, we all knew it was time for him to come home. I did some research and asking around if anyone knew a good gastroenterologist. I was working as an RN and had a lot of resources. I was told about a doctor at USC that had written books on esophagitis, esophagectomies, and colon transpositions. I picked Alex up from the airport and went straight to the doctor’s office. Another surgery was scheduled. This time Dr. D. would put a better valve at the sphincter muscle that was loose and causing the troubles. Alex was in the intensive care unit recovering when we were told that they were not sure if the surgery would be successful or not. This surgery was an open one, not laparoscopic as the first one was. So the surgeons were able to measure the esophagus and decide if it was worth a try. Though his esophagus was shorter than it should have been, they continued on. Alex had a long recovery period after that, and things seem to be going fine. He was eating better, had more energy and believed that he was left with only asthma to worry about.
It was only a couple of months before he was back in the ER for an endoscopy to dilate his esophagus. We were on the same track that we were on before the surgery, only he was so much worse off now. We made an appointment with Dr. D. and I was shocked when I was told that Alex would have to have his esophagus removed because the cells were turning to Barretts Esophagus, and that turns to cancer.
There were doctors that had treated him for most of his life and found his case fascinating, but now they were giving up on him. Alex was twenty two and had to make this decision on his own. As usual, I thought this would be it, the answer to our prayers. Nobody really knew what to expect because there weren’t too many people as young as Alex who underwent this type of surgery. They pulled the esophagus through his neck, then opened his stomach and removed a portion of his colon and positioned it to take the place of the esophagus. While they were finishing the surgery, Dr. D.’s nurse brought my husband and I to look at his esophagus. It was about five inches long and looked like hamburger meat. I had to wonder how he had survived this long. The normal length of an adult esophagus is about 10 inches and the lining is usually moist pink tissue called mucosa. We knew that it was the correct decision to have this surgery. But after everything that Alex had been through, we knew that there are no guarantees. With every procedure and every surgery, he is left with more pain, another unexpected side effect, and the knowledge that nothing lasts forever.
After a long, difficult and painful recovery Alex went back to live his ‘normal’ life. Not long after that , he was experiencing the bleeding from new ulcerations from the anastomosis (a connection between the top of the old esophagus and the newly placed colon.) So more transfusions, more night long visits to the E.R., stays in the hospital, and endless procedures. They were not able to continue with the endoscopies and Alex was unable to swallow, unable to play his beloved clarinet and unable to get rid of the pain. I was told by his doctor that he needed a PICC line (a peripherally inserted central catheter) used for prolonged periods of time. In his case they were used for total parenteral nutrition (TPN). He went through clotting and infections in the lines. Later they put a permanent G-Tube in his stomach to deliver cans of predigested formula.
His mental status was failing and was diagnosed with ‘failure to thrive.’ Then his doctor told me that Alex was eligible for hospice. I couldn’t process that. That couldn’t be possible. It was two weeks after that I took my beautiful boy to the Emergency Room for the last time. We came home and went to bed. I went to his room to wake him up the next morning. He was cold, his lips were blue and I started CPR and called 911. This time there wasn’t an ambulance to race him to the hospital. Instead my house was filled with policemen, firemen and the coroner. That night was the last time I saw my baby alive.
This is Alex. No one ever knew that he was sick. He kept it well hidden.