How am I supposed to remember what this is when I can't even spell it? #2

After what seemed like an eternity and absolutely getting no answers yet, the doctor came back to Isaac's bedside and told us that after reviewing the films he had called in pediatric surgery so they could evaluate him. I asked why SURGERY would need to review the xrays and evaluate him and he said they saw "abnormal gas patterns", "loops in the bowel", and a "narrow transition zone." Well what the heck did that mean? I asked him if he knew what the diagnosis would be or if he had any idea of what it could be. He said it could be a mucous plug that was blocking his intestines or some type of rare congenital disorder. He said but that's why he wanted a pediatric surgeon to take over his care. So now we waited for another doctor to come and talk to us. Meanwhile, all we did was sit by his bedside and watch him. He had a suction tube down his throat to keep his stomach clear of any fluids. He had a catheter in his rear end to drain all the gas and stool out since basically he had an obstruction and nothing could get out. He was hooked up to the standard ICU machines, pulse ox which monitored his oxygen levels, heart monitor, and a respiratory monitor. Along with all those things, he now had 2 IV's in. He had wires, leads, lines coming from every where. And all I kept thinking was how did this happen? And why? 

When the pediatric surgeon arrived, which was VERY quickly, I was so nervous but so eager to hear what, if any, news. She introduced herself, Dr. Stephanie Acierno. She explained to us that she wanted to do a barium enema which was a contrast dye xray of his abdomen because of what she saw on the regular xrays. Ok, but what did that mean?? She said that from looking at the films she suspected Hirschsprung's Disease. HUH? What IS THAT? Our question exactly. I had NEVER heard of that. We looked at each other and basically asked at once, "Is this something he can live with?" 

So what is Hirschsprung's Disease? 

The following information is from the Cincinnati Colorectal Center - Cincinnati Children's Hospital. 

Hirschsprung’s disease is a type of colorectal condition that occurs when nerve cells that are normally present in the wall of the intestine do not form properly during fetal development. During digestion, intestinal muscles move food forward in a movement called peristalsis, requiring special nerve cells called ganglion cells. Because these nerve cells are missing in children with Hirschsprung’s disease, normal peristaltic (bowel movements) movement cannot occur. Consequently, stool backs up, causing either partial or complete bowel obstruction. All children with Hirschsprung’s disease require surgical treatment.

So after hearing a brief description of what Hirschsprung's (HD for short) was, we wanted to know what we were dealing with. Dr. Acierno told us that she was almost certain that's what we were dealing with because of the amount of distention that was showing on the xray and because the transition zone was narrow. WHAT DOES THAT MEAN? Basically it was like trying to poop through a straw. Because he did not have the proper nerves that the muscles needed to function to push the poop out, it caused a narrowing and obstruction, and that caused him to get all backed up. She told us that she was hoping there would be only a small portion affected so they could do a procedure called a pull through. However, some children suffer from total colonic Hirschprung's Disease which meant their whole large intestine is affected.. NOT GOOD. But she told us that 80% of infants with this problem are lacking the cells in the sigmoid colon or rectum. GOOD. The less amount affected, the better the outcome. This is where we learned about SHORT SEGMENT HIRSCHSPRUNG'S DISEASE (or SSHD) and LONG SEGMENT HIRSCHSPRUNG'S DISEASE (or LSHD). Determining if he had short segment or long segment would determine what type of surgery he could have. A pull through or end up with a colostomy bag.

So now we needed to know if he had short segment or long segment. I asked if there was other conditions that looked similar to Hirschsprung's on film but unfortunately there wasn't. The only way to get a positive diagnosis was to do this contrast xray and then if they still suspected Hirschsprung's they needed to do a suction biopsy. The biopsy was the only way to give us a for sure confirmation. It was an easy procedure done at his bedside and did not hurt him. First we needed to get this xray done to see what we were really dealing with. So we started to prepare ourselves for this journey.