Long time no update! I can't believe I didn't update before Landon had his surgery but I have been so busy that I didn't get a chance. Everything went well.. now let me back track-
Saturday we left to come up here around noon. For some reason we have been having bag leaks about every 6 hours for the past couple of weeks even though I do everything the same every time. I have my suspicions that about half of them are defective.. But anyway, even though we put a fresh one on before we left we didn't even make it to Louisville, KY (3 hrs away) before he was leaking all over the place. We stopped there to clean him up and walked around their mall (a really nice mall, by the way!) and then we were back on the road.
Two hours later we made it to Cincinnati. Our first pleasant surprise was our hotel. I had booked our room through the hospital and had looked at their price range chart and selected the cheapest one I could find within 10 miles. Even though we had gotten a discount, you don't usually get much for $50 a night and so I was prepared for the worst. It was actually one of the nicest hotels/rooms I've ever stayed in, and we usually spend $120-$150 a night on rooms! It had an indoor pool and was walking distance to tons of shopping and restaurants, plus due to unusual weather it was 60 degrees out and so we were able to take advantage of that and explore the area. Fortunately I had already booked the same hotel for the few days following Landon's release from the hospital and so we can look forward to returning.
Sunday morning we came in and Landon was admitted to the hospital. He was put on a clear liquids diet and they inserted an NG tube to give him GoLytely, which should really be called go violently since he kept blowing off bags every 15 minutes no matter how often we emptied them. His bowels got cleaned out and then we went down for surgery yesterday morning. His surgery was scheduled for 11, but having been through surgeries in the past it was no surprise that they were just taking him back at 12:30. It took an hour to prep him and then 3 hours for the surgery, as expected.
At 4:30 we were called in to one of the conference rooms to talk to Dr. Levitt and find out the specifics. The first thing that we found out was that the part of his colon used in the ostomy was in fact good. He said there was a tight band around the ostomy and thought maybe that was why it had not been emptying like it should causing Landon to keep getting enterocolitis. That turned out to be great news because it meant less colon to lose. Landon was able to keep two-thirds which is more than was expected. Once you lose more than half you run into problems with dehydration and so it was good that Landon was able to keep so much.
Everything has been going well so far, but Landon is in a lot of pain. He had a tap block which wore off around 3 AM and ever since has been pretty sore. He is getting IV pain medication and Valium. He has an NG tube in suctioning his stomach, and currently has a nasal canula for extra oxygen which should be gone by the end of the day. We were warned that today will be the worst day for Landon as far as pain goes, so we are trying to keep him resting. After making it through today we will wait for him to have his first bowel movement sometime between tomorrow and Friday and then they will do an x-ray to be sure that he emptied his bowels. If everything looks good with that then he will begin eating again, which is the first step working our way towards leaving the hospital.
The only thing that has not gone well so far was the plan for genetic testing. Babies with Hirschsprung's often have RET gene defects, one of which is known as Multiple Endocrine Neoplasia Type 2 (MEN-2). Just under 5% of children with Hirschsprung's have that specific defect, which causes malignant tumors on the thyroid and adrenal glands. If the defect is discovered then there are steps that can be taken to minimize the risk of cancer, but it is not currently standard practice to perform genetics testing on Hirschsprung's disease patients. I had requested that the vials of blood be taken during surgery to avoid extra sticks and blood draws and that was the plan, but for whatever reason Endocrine decided not to do it without telling me. They are coming by any time to talk to me about the tests and I assume to try to tell me that they are not necessary, but I want this testing done and now I'm frustrated that Landon has to have extra sticks because they randomly decided to deviate from the plan and didn't feel the need to talk to me about it first.
I'll update again soon and let everyone know how Landon does. Here is a picture of him being his normal happy self right before going back for surgery.
Hello again! Today I had a phone appointment with Dr. Levitt, Landon's colorectal surgeon in Cincinnati. This was my first time speaking to him aside from exchanging a couple of emails nearly 2 months ago when Landon was staying so sick and I contacted him for a second opinion. I was pretty intimidated by the thought of speaking to him, he is pretty much a "celebrity" in the Hirschsprung's community. People travel from all over the country- even other countries to see him because he is by far the most experienced surgeon when it comes to Hirschsprung's. Anyway, I guess I expected him to be arrogent, and to my surprise he was very down-to-earth and friendly. He didn't make me feel like the crazy, over-protective mother that I am.
Rather than typing a long paragraph that's difficult to follow, I'll list the questions I asked and his responses:
Me: After reviewing Landon's records, what is your opinion on the reason that Landon went into cardiac arrest on day 6 of life?
Dr. L: It is hard to say with the information that is provided and without seeing him around the time that happened. There are several types of complications that can arise from Hirschsprung's associated enterocolitis (HAEC) that could have led to that event.
Me: So you do think it was related to HAEC, though?
Dr. L: Yes
Me: That leads to my next question- all the literature available states that HAEC is nearly impossible to get while a leveling colostomy is in place. On the other hand, it is fairly common after the pull-thru procedure. Since Landon has had so many issues with the colostomy when the risk is so low it makes me nervous for what will happen when the risk is significantly higher....
Dr. L: I suspect that there is a problem with the colostomy, that the passageway is too narrow to allow him to empty completely. I would expect improvement after the pull-thru, you may or may not have to continue doing irrigations.
Me: The irrigations have been very effective, doing them 2-3 times a day has kept him healthy. But I'm concerned with how quickly he accumulates bacteria. Is there any danger of bacteria going into his blood stream when you open up his large intestines for surgery?
Dr. L: As long as the proper procedures are followed through before the surgery, no. (Referring to giving him nothing besides clear liquids the day before and doing frequent irrigations in the hospital before surgery)
Me: Which type of pull-thru procdure will you be doing?
Dr. L: Swenson. In my experience it has shown to be the cleanest approach and the best way to be sure that none of the affected colon is left behind.
Me: Landon will be having the surgery right at 5.5 months, but he is currently approaching 4 months and getting to the point where he could start solids. Do you have any recommendations on whether it would be better to start solids and get him used to them while he still has the colostomy or if it would be better to hold off?
Dr. L: Either is fine. You can do the same as with your older children, it shouldn't make a difference.
That concluded our conversation. I feel reassured that everything will be okay and much more prepared to go do the surgery now. I can't believe it is barely more than a month away. At least now when we have bad days with the ostomy (bag falls off in public and leaks, huge messes, bags won't stick, etc.) there is light at the end of the tunnel. If Landon's colostomy was functioning like it was supposed to and kept enterocolitis away I really don't think I would be willing to do the pull-thru yet. I would do it eventually since it is a quality of life issue for him, but not yet, not at 5 months. As difficult and expensive as the colostomy care has proven to be, I would do anything for him to just get to be healthy for a while without uncomfortable interventions. Who knows, maybe that day will come sooner than expected after the pull-thru. That child has done nothing but beat the odds so far, why would he stop now?
We officially have the date for Landon's pull-thru/colostomy reversal surgery- Dec. 10th. We will drive up there on the 8th and stay in a hotel, and then first thing the morning of the 9th he gets admitted and placed on a clear liquid diet with frequent irrigations through the colostomy and his rectum. The surgery is sometime mid morning on the 10th, and then he stays in the hospital 7-10 days before discharge. After discharge they recommend we stay in Cincinnati a couple more days, and so again we will stay in a hotel and explore what there is to do in Cincinnati. I'm hoping to be home by the 23rd, which is Carson's (our oldest son) birthday. In case we aren't back in time we are having his birthday party on the 1st before we leave. It will be sad if we don't make it back for his actual birthday, but that was the way the scheduling had to happen. Aside from all of the other reasons that Landon needs prayers for his bowels to wake up and for him to have a quick recovery, that is another reason. So please keep him in your prayers until then.
On October 25th I have a phone appointment with Dr. Levitt, the surgeon doing Landon's procedure. I'm asking a long list of specific questions about everything. One concern I have is that anesthesiology wants to do a phone consult with me on Dec. 5th, just 3 days before our scheduled departure to Cincinnati. They are concerned with Landon's history of a previous cardiac arrest. Landon did go through over 4 hours of general anesthesia for his colostomy surgery and did fine when he was only 2.5 weeks old, but that was considered a life saving surgery and so they were more lenient on the conditions they would perform the surgery in. This surgery isn't absolutely necessary, yes Landon is doing perfectly, yes he could live his whole life with a colostomy, but this is a quality of life issue. I'm hoping to gain insight from Dr. Levitt on how likely he feels that it is that anesthesiology will either shoot this down or scare me out of doing it by discussing risks. I'm pretty nervous and anxious for both phone consultations.
Landon is still doing great. Still off antibiotics, still no infections. We are still doing irrigations 2-3 times per day, but we haven't tried to stop. Doing them is manageable and so it isn't worth the risk to stop doing them for experimental purposes. He is still gaining weight like crazy. All 3 of our boys had these matching shirts, but Landon's was in size 6-9 months. I put the other 2 boys in theirs, but figured Landon's would be huge on him. My mom was over at my house and insisted that I try it on him anyway and it fit! He's only 3.5 months old and it actuually fit! He is right on track with the size his brothers were at the same age, despite everything he has been through. Not to mention that most Hirschsprung's babies have some degree of difficutly absorbing nutrients and growing. We have been blessed 100 times for every unfortunate event that has happened and I am so thankful. I will probably update next after the phone appointment next week and let everyone know how it goes and what Dr. Levitt has to say.
Hello again! Since I last updated everything has pretty much been the same. I guess it's good that I don't have a bunch of updates, the past month has been pretty uneventful. Landon is off antibiotics now, and so far so good. Dr. Levitt in Cincinnati has reviewed Landon's records and developed his plan of care. No surprises there, he will have a colostomy reversal/pull-thru procedure sometime within the next few months. They should be contacting me any day now to schedule the surgery. If we can't schedule over Christmas break we might have to wait until March for Spring break. I'm really trying to avoid taking FMLA at work, and with this only being my second year at my job I don't have much sick time to work with.
More good news, I've won several battles with our insurance company. I appealed Cincinnati being billed as out-of-network, stating my case that mistakes made in St. Louis due to lack of experience would be more costly than a trip to the leading colorectal specialist in the country. I'm still working on the whole colostomy supplies issue, though. Whoever decided that these bags should last 3 days clearly never had a child with a colostomy. Even when I tape and paste around the edges for extra reinforcement, they always spring a leak within 24 hours. Not fun!
Last time I updated I mentioned that Landon had started laughing without sound- well 2 days later I was getting ready for work and Carson and Austin were playing with Landon in his bouncer. I heard a laugh- one that was clearly not Carson or Austin's. I looked in there and Carson and Austin had Landon laughing out loud! At the time he was still barely 2 1/2 months old! I even managed to record it on a video on my phone, until Landon spotted my phone and immediately gave it a hard stare and forgot what was so funny. Landon has also continued to become stronger and stronger. He has awesome head control, and when he sits in his Bumbo seat he can easily turn his head back and forth to look around the room.
So overall, everything is going very well. Last weekend we even managed to go on a weekend trip to Nashville, Tennesee- colostomy and irrigation supplies in tow. While we were there, we purchased a new vehicle unexpectedly. We were pretty crowded in our Toyota Highlander with 3 car seats squeezed in the middle row, and I just wasn't comfortable putting any of the boys in the 3rd row in case we were ever rear-ended. We managed to find a great deal on a Chevy Suburban that is only a year old. It is huge, and has plenty of room for the boys. I feel much safer driving it, with all the worrying I have done lately it's really nice having one less thing to worry about.
Well, that's it for now. Hopefully everything stays uneventful and I can just check in every now and then and talk about how well everything is going!
Since we have been back from Cincinnati we have had the longest stretch of uneventful time so far, and it is great! Landon is down to just 2 doses of Flagyl a day (down from 4!) and he is staying healthy. This Thursday we are removing another dose and we will be down to just one a day. We are doing irrigations twice a day, and increase the frequency up to 4 times a day if he seems to be getting sick. One day mid last week Landon vomitted once right before we left the house in the morning, and so my mom and stepdad brought him to me at work during my lunch and I did an extra irrigation in the front seat of their truck! It cleared everything right up and Landon has continued to tolerate the decrease in antibiotics well. Just a side note for those who aren't from the Hirschsprung's support groups who aren't familiar with what irrigations are, it's the process of inserting a catheter through the rectum (or ostomy if the patient has a colostomy-like Landon or an illeostomy) and then feeding it up through the intestines. You then attach a syringe and slowly insert sodium chloride solution and let it drain out, taking all of the stool and bacteria out with it. Trust me, it is not a fun thing to do to your child. And I'm sure it's not especially pleasant for Landon, either.
Besides the Hirschsprung's-related issues, Landon is doing very well. He is still gaining 3-4 times the amount of weight that is expected for a typical baby his age, which is absolutely amazing for a child with a disease that is often closely related with poor weight gain/growth. He is smiling more each day, and has started making laughing faces without making any sound. He has also gained enough head control to sit in his Bumbo seat and watch his brothers play. So far at 2.5 months he is on track developmentally and I will continue to stay cautiously optimistic regarding his future development.
Pretty much everything is going well for our family. The colostomy care was difficult at first, but has gotten easier. Same with the irrigations. It is tough getting up at 5:15 in the morning just to complete all of Landon's care before I go to work, but I'm hoping that gets easier as he continues to sleep for longer stretches of time. It will also be difficult any time that he gets sick and requires extra irrigations. I work 25 minutes away from our home town and I work 8:15 to 3:30 with a 30 minute lunch- not enough time to run home in the middle of the day. I wouldn't ask my mom and stepdad to bring Landon to me during my lunch on a regular basis in their gas guzzling truck, that would be too much. All I can really think to do is take little chunks of sick time to tack on to my lunch and run home, but my sick time is already running low and I still have to plan to take off for his 2nd surgery sometime in the next few months. Not to mention the nature of my job- I'm a speech pathologist in a school and so every time I'm out my students miss their speech that day. Working is definitely tough right now, but I have to make it work. I love my job, and my older two boys love their daycare. They both have friends in their classes and love the socialization, and they learn so much. I know if I were to stay home with them I wouldn't be able to read to them or teach them like they get at daycare with everything I would have to do for Landon. Every day would be crazy rather than structured, which is what they need. Not to mention, as I said before, I love my job. I work a teacher schedule and so I still get plenty of time with my kids, in addition to working in a profession that is very rewarding to me. I would really like to fast-forward to next summer when I'm off for 3 months. By then Landon will be 1 and things will hopefully calm down even more.
We went home with an excellent prognosis and everything was going great. That was until Landon took the last dose of his antibiotics. After 4 days, his stool developed a foul odor. Next he become inconsolable, and cried constantly, and did not want to eat. He started throwing up, first plain vomit, and then bile. I was convinced the cycle was beginning again and we rushed him back up to St. Louis Children's Hospital which was 2 hours away. They started him back on antibiotics, and within a few days, everything was great again. We went home shortly after on a longer course of antibiotics.
Again, everything was going great. He even gained 2 full pounds in less than 2 weeks! Everything was picture perfect... until he took his last dose of antibiotics. Within 4 days, the cycle began again, in the same order. This time it was caught early enough for us to treat it at home and avoid the 2 hour drive each way to the hospital. Again, he improved right away. But it was mentioned that he might have to stay on the antibiotics until his next surgery....
...but wait a minute! At this point I was pretty educated on Hirschsprung's Disease. You are not supposed to get enterocolitis while you have a colostomy in place! It is supposed to be next to impossible! Something didn't sound quite right. Why was he sick constantly? And how would the next surgery (to close the colostomy and reconnect his bowels) change anything if he was already having issues during the time that he shouldn't be?
Time for a second opinion. I did my research, and found that the best and most experienced doctor on Hirschsprung's Disease is Dr. Levitt at Cincinnati Children's Hospital. Fortunately for us, that is only a 5 hour drive away, so I contacted Dr. Levitt. I summarized Landon's story, and within 2 hours he personally emailed me back. I was very impressed with his response, especially considering our very own surgeon that had done Landon's colostomy was impossible to contact. I had to speak with a receptionist who took a message for the nurse, and then the nurse looked at the message, tracked down his surgeon, and this process usually took a couple of days! Forget about speaking with his surgeon personally! And that is the surgeon that only operates on 2 cases of Hirschsprung's Disease a year. In the other corner, in Cincinnati, we have the famous Dr. Levitt- who does several of these surgeries a week- in which Landon is not even a patient of- and he emails me back within 2 hours!
He agreed that something didn't sound right and had us come on up and admitted us to the Cincinnati Colorectal Center. Even with the 5 hour drive, it was amazing to be in a place devoted entirely to children like Landon. All of the nurses and nurse's aides were all experts on Hirshsprung's Disease. They ran several tests and determined that nothing was wrong with Landon's colostomy that posed an immediate threat, but something was definitely off and he shouldn't be getting enterocolitis over and over. They also didn't think he should be on such a high dose of antibiotics, and felt that he should wean off of them rather than repeatedly attempting to quit cold turkey.
So, as you can probably imagine, we decided that from now on we will drive the extra 3 hours each way to take Landon to Cincinnati. We have higher out-of-pocket expenses since they are out of network with our insurance, but it only amounts to a few thousand which is NOTHING compared to our child's life. They still suspect that something is wrong with his colostomy, but they recommend following a strict protocol of irrigations and some antibiotics and then just going ahead and doing the pull-thru sooner rather than later, rather than revising the colostomy and then having to put Landon through an additional surgery to do the pull-thru. So for now that is the plan!
