Curve Ball

Well, everything has pretty much taken a 180 degree turn since my last update. I received an expected phone call with very unexpected news last Monday. The biopsies of Landon's colon DID contain ganglion cells. Dr. Bernabe said that she wasn't comfortable removing what appears to be good colon that for whatever reason isn't working. She said that she could offer us Botox injections, but wasn't sure if that would help.

I contacted our nurse at Cincinnati to let her know what was going on and Dr. Levitt emailed me back. He said that he wanted to do a rectal exam under anesthesia with a sigmoidoscopy and possible upper GI endoscopy. We began the process of getting preauthorization from our insurance company and were prepared to wait until at least the end of the month before an opening would be available.

Next came another surprise when the nurse called on Thursday and said that they had an opening on Monday (today.) Then, an hour later called back and asked that we go up the next day for some testing beforehand. Fortunately, my stepdad was able to make the trip with all 3 of the boys and I. We made a round-trip in one day, and so way too much driving with way too many small children. It ending up being worth it to shed some light on the situation.

On Friday, we went down to radiology and had a variation of the contrast study done. They inserted the solution differently and took views from additional angles. Dr. Levitt felt that the narrowing that appeared was inflamation from enterocolitis, not colon that wasn't functioning. To be honest, I'm so exhausted, frustrated, emotional- you name it- that I pushed for doing an ileostomy and getting Landon healthy and his bowels healed. Dr. Levitt respected my wishes, but managed to talk me out of it. He convinced me that trying botox first could be the way to go to see if relaxing Landon's sphincter helps move things along. He left open the possibility that the ileostomy might still happen depending on what condition his colon was in once they took a look inside.

Last night, John, Landon, and I came back up prepared to stay up to a week with the possibility of being able to do everything outpatient and coming back home today. I'm happy to say that everything turned out best case scenario- upper GI endoscopy, sigmoidoscopy, and rectal exam all three were perfect. Dr. Levitt gave Landon 4 botox injections and we were happy to be out the door in under 4 hours. Now we wait a few days to see if the Botox works. If it does, then we will repeat it about every 3 months as long as necessary. If it doesn't work, then we will go back to our discussion on the ileostomy. We are also awaiting results of a some biopsies taken of the small intestine which are expected to be normal. I'll update when I get a chance on how everything goes.

Who, What, When, Where...

Hi everyone. I have a lot of updates. Let me start off with the good news:

Landon is officially a year old now! We celebrated his birthday on Saturday and he was able to enjoy his first cupcake during a Superman themed birthday party.

 

 



What a year it has been! Even though there are still gross motor and feeding issues that are being addressed, he is doing so well for what he has been through. He is getting stronger every day and is doing better at standing on his own and taking steps with a push-toy. The most amazing area of all, no doubt about it, is his communication. His receptive vocabulary is HUGE. This child understands everything. Any time we have a conversation about anything, he is looking around the room at what it is we are referencing. He is also starting to consistently use several words. He calls me "mama", says "hey!" to tell people "hi", and labels his favorite thing of all time- "fish" by saying "fffffsshhhh." What a blessing he has been to watch him grow and learn.

Now for the not-so-good news. Thank God we celebrated his birthday on Saturday, because on his actual birthday on Sunday he became very sick very quickly. He was projective vomitting and became lethargic. We rushed him to the emergency room at St. Louis Children's and they conducted testing and diagnosed him with enterocolitis. He was still taking the full dose of Rifaximin, but it turns out that it was no longer working so well. They started him on IV Flagyl and we noticed improvement after several days.

We were reunited with his surgeon at St. Louis, Dr. Bernabe. It made me realize that there are advantages to being in St. Louis vs. Cincinnati: 1) much shorter drive, 2) our older boys and other family can travel up to visit 3) they more thoroughly know Landon's history and take enterocolitis more seriously, 4) pretty much everyone knows us from his "episode" a year ago today, 5) we are able to visit the doctors that saved Landon's life and express our gratitude and show off how well he is doing.

Although those are all important advantages, number 3 turned out to be the most important. Dr. Bernabe decided to do further testing to see why Landon is continuing to have such a poor outcome following his pull-thru in December. The first test was a lower GI study that was done on Wednesday. It didn't take the radiologist long to see that something was abnormal. Landon had a narrowing in the rectosigmoid region and into the descending and transverse colon, consistent with Hirschsprung's disease before ANY surgery is performed. Dr. Bernabe then came in and explained that although it is rare, sometimes following the pull-thru surgery an area of the colon doesn't receive sufficient blood flow and can actually become aganglionic. We have biopsies from both St. Louis and Cincinnati to confirm that good colon with ganglion cells was pulled through back in December, and so there are no questions about there possibly being a surgical error. So we proceeded with a biopsy, which was done yesterday morning. Dr. Bernabe went in and performed a full-thickenss biopsy under general anesthesia to confirm that a section of Landon's colon is aganglionic again. Landon did very well during the procedure and we were able to come home last night.

So what does this mean for Landon? I've created a diagram to better illustrate what I'm about to explain in hopes that maybe it will be a little less confusing.

 

Here is a colon/large intestine. The area circled in blue has already been removed (15 cm), with the area directly above pulled through and reconnected with Landon's rectum. So the area circled in green is actually stretched and is placed where the area in blue used to be. Fast forward to Wednesday, when we saw the results of the lower GI study. The area in green (another 7 cm) is now narrowed and is not allowing stool to pass. Once removed, Landon's entire descending colon and part of his transverese colon will be gone. This is no longer consistent with short-segment Hirschsprung's disease, resection to this extent occurs with long-segment disease.

 

Those are the facts. Now we get into the opinion of the surgeon. Dr. Bernabe feels that since the ascending colon receives much better blood flow, we may have the greatest chance at a successful surgery if she removes Landon's entire transverse colon and connects his ascending colon. That would leave approximately 25% of his colon in place. That part sounds okay. The downside is that she uses the Soave method. Everyone should do their research on the Duhamel, Soave, and Swenson methods and form their own educated opinion. My opinion is that the Soave method is inferior to the Swenson method and yields a higher complication rate of enterocolitis and re-do operations due to what is known as the "Soave cuff."

 



For that reason, I can't say that I'm comfortable doing the pull-thru in St. Louis. I do feel that St. Louis is better managing Landon's care and is being more thorough, so I asked about the possibility of just going up to Cincinnati for the re-do pull-thru (Swenson method) and then coming back for Dr. Bernabe to manage his care. Dr. Bernabe stated that she would be comfortable with that, and so that is one option on the table.

Another option would be to return to another temporary colostomy. This would be a good option to keep Landon healthy for a while and off of antibiotics, and we would definitely do this in St. Louis. The disadvantages are that the colostomy was not easy to care for, and this would guarantee at least one more additional surgery to reverse it at a later date.

There are a few other options that we are not ready to consider (permanent colostomy, pull-thru with loop ileostomy). So basically what it comes down to is a decision to go back to a colostomy for a while to get Landon healthy and stay out of the hospital for long periods of time, or to go straight to a re-do pull-thru and keep trying to make this work. Both options have advantages and disadvantages that we are weighing. Right now we are deciding what to do, who is going to do it, where it is going to be done, and when it will be done.