The Upside of Down: Hirschsprung's Disease and Surgery

After finally getting out of NICU, we started to get into a routine at home. It was nice. Me and Carson's day mostly consisted of eating, pooping, sleeping, pumping, and then the cycle started over. I was just starting to forget what hospital life was like when all of a sudden we ended up back in there!
Carson was just over three weeks old and I already had learned his feeding habits, how much he would take, etc. The morning after my husband's 30th birthday, Carson would not take his bottle, and he started spitting up everywhere. I was a little concerned and I had Bert call the pediatrician because I felt like something was wrong. Our awesome pediatrician got us in that afternoon. Here I was, the crazy first-time mom that rushes her kid to the doctor because he spit up! I felt slightly crazy but had this nagging feeling that something wasn't right.
When we got the doctor, he felt around on Carson and laid him on his belly and asked us if we thought Carson looked distended. I hadn't noticed until that very moment and boy, did he! What we thought was just his little "frog" belly was really him being distended. He sent us right away to HealthPark (what would become our second home) to get an x-ray done. He told us to go see the pediatric radiologist there. He had already called him and they would be expecting us.
By this time I'm getting a little concerned but in the rush of things I really didn't have time to freak out. By the time we got to the hospital and up to radiology it was almost 4:00 in the afternoon. They took us back pretty quickly and did a few x-rays of his stomach. The radiology tech was super nice and wanted to show them to Dr. Weiss and see if he wants us to do anything else. When he came back he said Dr. Weiss wanted to do a barium enema on Carson. I didn't know what the heck that was but, it didn't sound like it was going to be too much fun, especially for a tiny baby like Carson. Carson screamed his head off when the barium enema went in and during the entire scan. They couldn't use any lubrication to help get the enema in because it can mess up the picture, just to make things worse. After this we were starting to get even more concerned. What was wrong with our precious baby??
We got Carson cleaned up and went to this little waiting room in the radiology department. We were sitting there slightly frantic when a doctor came up to us. This was Dr. Mon, who would become a very close doctor to us and somewhat of an advocate for Carson. He had just happened to be walking by when Dr. Weiss was looking at Carson's barium enema results so Dr. Weiss consulted with him. The results were leading them to believe Carson had Hirschsprung's Disease, which is where nerve cells don't form in part of the intestine. This could be affecting a small part of his intestine all the way to the entire intestine. He did not believe that it was affecting his entire intestine, in part, because he had gone to the bathroom. It just appeared that he was not able to get everything out and was getting backed up. He said that he wanted to admit us to the hospital and do a biopsy to confirm the Hirschsprung's diagnosis.
Wow, that was a lot to take in. Here we were completely unprepared to be spending the night(s) in the hospital. I was scanning my brain for any recollection I had of Hirschsprung's Disease. There it was, I remembered reading about it when Carson as in the NICU. There was a small part about it in the book I had "Babies With Down Syndrome". It is more common in babies with Down Syndrome and a big sign of it is not having your first bowel movement within 48 hours. It took Carson 72. I just felt like we were in the clear with HD because once he started going, he was going! We called our families and they were in just as much shock as us. Everyone rushed over to the hospital as quickly as they could and brought us stuff from home so we didn't have to worry about that.
Two days later on Sept. 16, 2010 Carson's biopsy was performed. I wasn't too apprehensive about it until that morning when they wheeled us down and we had to pick out a little scrub cap for him. Could this be real, was I really picking out a scrub cap for my almost 4 week old baby?? Something was not right with this picture. The night leading up to the enema had been difficult because Carson could not eat anything so he was up nearly all night long. We just took turns trying to keep the pacifier in his mouth and keep him soothed as much as we could. All the while, I was still a pumping machine because I did not want to switch Carson to formula yet! The biopsy went fine and two days after that we were discharged from the hospital. Dr. Mon told us he would call us with the results and we would go from there.
A few days after we were discharged was my follow-up appointment with my ob/gyn. There I was telling her about this whole Hirschsprung's Disease mess we were going through. She asked who our surgeon was and told me if he couldn't do it to go to Tampa! Did I need to go ahead and go to Tampa? She reassured me that Dr. Mon was great but the next best option after him would be in Tampa. That made me feel better that we had a great surgeon on our side. The next day Dr. Mon called and Carson did have Hirshcsprung's Disease. He would need to have surgery to remove the part of the intestine that did not work. Once he got inside he would have to keep biopsying to see where the nerve cells started. If it was really bad and he got too far he would have to close him up and we would have to make a decision on what to do, most likely he would have a colostomy if that happened. The surgery was scheduled for October 13.
Unfortunately, Carson would not wait until his surgery date. Not even a week later he was not eating, spitting up, distended again! We called Dr. Mon and he wanted to go ahead and admit us to the hospital and we would move his surgery up. His surgery date would now be Sept. 30, 2010. Carson had to have yet another IV, it took the nurses 4 tries this time. Poor little guy having to go through so much at such a young age. He wasn't able to eat but Dr. Mon did allow us to give him some Pedialyte so at least it would give him the feeling that he was eating even though he wasn't getting full. There were more sleepless nights leading up to the surgery. The night before I could barely sleep a wink. Dr. Mon said it was difficult to say how long it would last because of the biopsying and not knowing how much he would have to remove. He felt like Carson's did not go too far because he was able to get some out but, obviously not all.
Once again, we were wheeled down to the surgery prep area. There the nurses and anesthesiologist went over everything with us. We picked out another scrub cap (the same print we picked out last time). As I sat back there waiting, I noticed that all the other children back there were, first off, much older than Carson, and all seemed fine until it was time for them to drink this medicine that relaxes them some before they get put under. I joked that they need to give that medicine to the parents because it's pretty tough on us too!
They took Carson back and Dr. Mon said he would call the waiting area to update us on his progress. Having to hand over my baby and watch them take him away was one of the hardest things I've ever had to do. When we got out to the waiting room our families were waiting for us. They were all so upset they didn't get to see Carson back. Thank goodness I completely trusted Dr. Mon and knew he was in great hands :)
The waiting was excruciating. I didn't even want to leave to go to the bathroom, much less eat lunch! Finally the phone rang and the lady at the front desk said it was for me, I answered and Dr. Mon said it was what he had suspected. He was going to need to remove about 6 inches of his intestine. That was a good thing that it wasn't more than that. It had already been a few hours and he was just about to start the surgery part, now he was done with biopsying.
After a little while longer we are still sitting in the waiting room eagerly anticipating hearing something else, when my sister Valorie walks in! She wanted to be there for me so much that she drove all the way from Stuart just for the afternoon. It was such a great surprise and a nice relief from all of this waiting we were doing :)
Six excruciating hours later we got a call to the front desk for me, Dr. Mon said that Carson was out of surgery and heading to recovery. He said that when he took him off the ventilator he immediately started breathing on his own! That meant when he came to he could come back up to the room with us and not have to stay the night in PICU!! That was the most amazing news! I was not looking forward to having to spend the night without him. About thirty minutes later a nurse came and got me to come back and see Carson. There was my tiny, little baby boy laying in a huge hospital bed. He was so swollen from them pumping him with air because surgery was performed laparoscopically. But he was absolutely perfect as always :)
They were ready to take us up to the room so I called everyone in the waiting room and told them to meet us up there. It's hard to look at pictures of him after the surgery now because he barely looks like himself and he was pretty pitiful after. He would just make grunts when he moved or wince in pain. Changing his diaper became very difficult and I wouldn't do it until the nurse brought him his pain medicine.
The night of the surgery me & Bert both got probably the best sleep of our life. Carson was still out from the anesthesia and we had been through so many sleepless nights leading up to this that one little break was graciously welcomed!
The next few days he became more and more aware, and more and more hungry! Unfortunately, because Carson's surgery had to get moved up and was not performed when it was scheduled, Dr. Mon had to go out of town following his surgery and a colleague of his had to watch over Carson's progress. We became very close with some of Carson's nurses and aides, some we had our last stay even. We had a life now at the hospital, eating downstairs, sleeping on our air mattress. Occasionally we would get Carrabbas and have a little date in the waiting area. We tried not to eat around Carson while he wasn't able to eat because we didn't want the aromas to make him hungrier than he already was. Eight days after we had been in the hospital the doctor told us that we could go home as long as Carson was taking his bottles good. We were SO ready to get home! I forgot what it was like to sleep in my bed and cook dinner! Carson was doing good eating but he had one bottle that he left an ounce or so. The nurse came in and told us that we would not be able to go home because he hadn't finished that bottle. Here we were again, all ready to go, car loaded up and we couldn't go because of Carson's feeding! Once again, I know you guys are probably getting tired of reading this, but I was crying, heartbroken about not getting to take my boy home. Bert's poor dad was in there and he felt so terrible for us. The next day Dr. Mon would be back and I was hoping that meant we could go home.
Dr. Mon came in the next morning and quickly discharged us. We had to see him in a few weeks and he drilled into us signs of a bad infection that Carson was susceptible due to HD. Constipation was a big no-no and could lead to enterocolitis.
Following his surgery, it's hard to remember all the times we were in and out of the hospital. My birthday dinner we came home from the Melting Pot and the ambulance ended up picking us up and taking us to HealthPark where we stayed a few days. We were back again after Valentine's Day as well. We had our same room we usually got and all of our nurses we love. Just when we would think things were going well, we'd be back in.
Luckily our last hospital stay was in mid February. We started to get things under control and with a little help from Miralax we have him going pretty good now. It's a constant battle, not only due to HD, but also low muscle tone due to Down can cause constipation issues. Dr. Mon also told us that due to HD and the surgery it might be harder to potty train Carson bc the connections are not as good as they once were. All in all, once we got a year out from the surgery we should be in the clear for the risk of enterocolitis.

We really are lucky that this is all Carson ended up with. I couldn't imagine going through heart surgery with him, this was difficult enough. Once again, Carson proved his strength to us all. We are also so fortunate that it brought Dr. Mon into our lives. He has been such an awesome doctor and friend to us all. He has even helped us recently get quicker appointments with our newest health scare with Carson. We went through a phase of not wanting to leave the area for fear that Carson would end up back in the hospital. I am glad those days are over, but so thankful for the amazing staff at HealthPark and Dr. Mon for making things so easy on us :)