Taking a step back

"Optimist: Someone who figures that taking a step backward after taking a step forward is not a disaster, it's a cha-cha.” -Robert Brault

Thank you to everyone that has been asking about Landon. Unfortunately, the reason I haven't been updating on him online the last few days wasn't because things got better, it was because we were plotting our next move.

The random vomiting spells are only getting worse. It has happened every day since Tuesday- 4 consecutive days now. Landon's GI system is so out of whack there's no point in even trying the botox. We are over a year post pull-thru and it seems that things are getting worse instead of better. Some may remember this past summer when we were entertaining the thought of going back to an ostomy. I felt pretty strongly about doing it to get him off the Flagyl (which he has had to take over a year total now) and to improve the quality of life for Landon and the rest of the family. Landon should not have to deal with 3+ hours of irrigations a day, and it takes away time from our other kids. He shouldn't have to deal with projectile vomitting all the time, the belly aches, not ever wanting to eat and pretty much being force fed. But despite all that, having an ostomy stinks for him because it makes him "different." So we tried the Botox, and it helped. Just not enough. Landon spent his first Christmas eve and first birthday in the hospital.. but he is not spending his 2nd or his 3rd. After the way the last 3 months have played out, I have complete confidence now that going back to an ostomy is the right thing to do.

So the 30th we will head back up to Cincinnati, but instead of doing the botox, Dr. Levitt will be giving Landon an ileostomy. We will then have to stay in the hospital for a week and possibly a few additional days. This time we know what to expect post-op. We already know how to do bag changes, and even though they are no fun for us, Landon had a much better quality of life with an ostomy. I'm looking around for the coolest pediatric ostomy belts that I can find to conceal it. I learned from our last go round that many, if not most people are very freaked out by the appearance of it. After all, it is live human intestine hanging out of the abdomen, and even I had a hard time looking at it and touching it at first. But Landon is completely aware of what goes on around him now and I don't want him to experience any dirty looks or unnecessary rude questions. He will just have some sort of cool belly band he wears around his waist barely peaking out of his pants.

Since it is another temporary ostomy we know that he will have to undergo at least one additional surgery eventually. I want to emphasize the word eventually. We are leaving things alone for at least a few years. There is a good chance he will go to kindergarten with an ostomy. Personally, I'm tired of making decisions that affect his quality of life. I can't even explain how bad I feel when I think that we could have done something differently thatmay have stopped him from going through even a little bit of the adversity that he has faced. I hope that we can leave things in place until he is old enough to want to reverse the ostomy, and to understand the risks and challenges that will come along with it. Who knows, maybe within the next few years modern medicine will catch up to these complex HD kids. There is some promising research going on in Boston right now where they have found that injecting cells from the intestines of healthy mice into the intestines of mice with HD caused the aganglionic segment of the intestines of the HD mice to gain ganglion cells and function. That's terrific news, especially for the children affected with total intestinal aganglionosis. In Landon's case, that particular study may not have much use since his affected segment has already been removed and he continues to have poor function of his colon for unknown reasons.. but hey- at least they're getring somewhere!

I hope everyone has a Merry Christmas! I will update soon on how the surgery and recovery goes. As always, please keep Landon in your prayers.

The night I poured my 17-month-old a Coke...

I'm sure you can tell by the title that this is going to be an interesting post. I really don't know what else to do, though. Landon is sick- he has been having a horrific vomit fest all night. Our closest children's hospital is 2 hours away. On one hand, I don't want to let this go to the point where we miss Carson's birthday and Christmas Eve because we are in the hospital. On the other hand, it is late at night, the other boys are in bed, we would have a lot of packing to do, and the roads are icy and expected to get worse. We don't want to take Landon in unless it is completely necessary, and the key to that is to keep him hydrated while we hope and pray he responds to things we can do from home. It's safe to say at this point that his last round of Botox is completely worn off, and even the full dose of Flagyl and 2 irrigations per day isn't keeping enterocolitis away. Why does this have to be the weekend? If I call Cincinnati they will put me on the line with a resident and he or she will tell me to take Landon up there. Unlike Dr. Levitt, the resident will not be aware of how much experience we have with enterocolitis, irrigations, and managing this stupid disease while doing everything we can to keep him hydrated.

Which brings me to the title. A little background: we are a very healthy lifestyle family. We eat mostly natural, organic, and unprocessed. Carson and Austin have never tasted a caffienated soda. Maybe twice a year as a special treat we will let them order a rootbeer or Sprite when we're out to eat, but that's it. Unfortunately, though, my title is a true event. I poured Landon a Coke in a sippy cup and he drank about half a can. What else is there to do when your son is vomitting profusely and you desperately need to get clear liquids in him. And he refuses Pedialyte. And he refuses water. And he refuses apple juice. You bet he guzzled down the Coke though. Thank God he did because it was my last resort.

Please keep Landon in your prayers the next couple weeks. He has another round of Botox scheduled for the 30th and so we need him to hang on through the holidays without landing in the hospital AGAIN. This poor child spent the better part of his first 3 months of life in the hospital, and his 1st birthday, and as most probanly remember we had to rush him to the hospital on Carson's 4th birthday last year and missed Christmas Eve and drove all night just so we could be back for the boys to get their gifts from Santa Christmas morning. I want nothing more than not to have a repeat of last year, so please keep him in your prayers!

Update

Everything is going pretty well with round 2 of Botox so far. It has been nearly 2 months and it seems to still be working. About a week ago there were some irregularities that made me think that it had worn off (enough that I started the process of scheduling round 3 for the end of next month) but everything seems to be working pretty well again. We haven't been successful in getting Landon off Flagyl this time around. We are getting ready to wean him off and try again starting next week.

Landon still takes 15 mg of Senna (one Exlax square) along with Klaire Labs Therbiotic Complete probiotic (adult dose), one capsule of S. Boulardii, a gentle iron supplement, multivitamin, and extra fiber with built in probiotic support. We no longer see the natural medicine specialist due to the cost and very little observable difference now that things are working more smoothly with the Botox injections.

We are still very careful about what Landon eats. Constipation doesn't seem to be an issue, more of the opposite. Laxative foods tend to run right through him and so we are now limiting most of the foods that Landon originally was living off of. He drinks 2-3 Pediasures a day for nutritional support and extra calories, which for right now is all he drinks aside from an occasional sip of water. Fruits and vegetables are the most difficult foods to get in because they often pass undigested and cause mild obstructions- which thankfully at this point we have been able to dislodge during rectal irrigations. Landon can eat peaches, apple, carrots, and sweet potato.. as of right now that's it. It's unfortunate because he is a very good veggie eater, but we have had some close calls with everything else. We have been lucky that Landon does fine with small to moderate amounts of cheese (which I love and add to most dishes that I make in some form!) He eats everything I serve for dinner which includes chili without beans, various pasta dishes made with pasta with extra fiber, meatloaf, chicken breasts, and sloppy joes on whole wheat buns. Other foods that he does well with are scrambled eggs, Applegate chicken sausage, plain Almond yogurt, peach-applesauce, thin-sliced deli turkey, and any kind of chicken- but especially nuggets and tenders. We go on Monday for a check-up with his pediatrician to confirm this, but Landon appears to be growing and gaining weight very well. At 16 months old he is outgrowing 18 month clothing.

We are doing one rectal irrigation per day. We do this before bed every night. Landon tolerates this very well and isn't at all bothered by it, despite how terribly invasive it is. My first goal is to get him off daily antibiotics for good before we worry about discontinuing the irrigations. If he doesn't come off of the Flagyl this time, I want to discuss either trying a super low maintanence dose or possibly giving Rifaximin another shot. We are approaching the one year anniversary of Landon's pull-thru surgery and so things should be regulating as much as they will within the next few months. Thoughts and prayers are always appreciated!

I'll leave you today with a picture of Landon at the park yesterday with his brothers. He loved this ride and although I was afraid he was going to get a brain injury and/or broken bones, his brothers couldn't make it go fast enough for him!

Round 2

After 4 weeks of absolute perfection with Landon's health, the botox wore off. Before there was even a noticeable change in his bowel function he already had a low grade case of enterocolitis. They had hoped that we would get at least 3 months out of the last round, but Dr. Levitt said they could go in for another round after 2 months. So basically for the past month Landon has lived off of Flagyl, frequent irrigations, and Pediasure since his appetite is nonexistent when things aren't moving along very well.

Today we were finally able to repeat the botox injections. Landon did great and we were in and out of the hospital in under 3 hours. Dr. Levitt recommended that we start putting him on a potty chair to encourage him to push more on his own, but other than that didn't have any suggestions other than repeating the botox treatments and treating enterocolitis as needed. I asked about the possibility of doing a myectomy- a surgery to permanently remove some muscle in the sphincter to create the same effect as the botox permanently. He said that he doesn't like doing those surgeries due to the risk of incontinence later on. For now I'm fine with trying a few more rounds of botox to buy us some time, but if things don't improve and Landon continues to experience recurring bouts of enterocolitis we will definitely weigh the pros and cons and reconsider.

Now we wait and hope for similar results from this round of botox as we experienced with the last. Other than our continued Hirschsprung's related struggles, things are going really well with Landon. Big news- he is officially walking! He took off walking 2 weeks ago and has made great progress becoming more stable ever since. He is also saying and repeating more words every day. This weekend we came up to Cincinnati early to visit the zoo and children's museum with the boys, and during that time Landon was around a lot of children his age. I noticed two wonderful things- compared to his peers, Landon is 1) big! and 2) equal. He talks, walks, and climbs as well as other children his age that we saw. No one would ever guess there is anything out of the ordinary going on with him other than the slight clue that I'm holding him down pretty much force-feeding bottles of Pediasure just to get calories and hydration in him.

I'll update with results of the botox soon. I hope everyone is enjoying the start of fall. The weather is absolutely beautiful here right now!

FINALLY!

After being on this roller coaster much longer than I would have liked, I think we have finally turned a corner. Many times we have seen a slight improvement here or there for a few days or even weeks at a time, but never before have we seen such drastic, steady progress.

So I guess I have some back-tracking to do to catch everyone up..

On Monday, the 15th, Landon had his Botox injections. We were told that it would start to work over the next few days, and that in a week if we had not seen any changes then it was not successful. After 2 or 3 days we saw very subtle differences. Landon was passing what appeared to be normal looking stool (which was a first, his stool has always been completely liquid and very undigested looking.) We could tell during the irrigations that the Botox had loosened things up like it was supposed to, but despite the improvements, Landon's bowels were not functioning any better. We still had to do 2 very long irrigations of 3000-4000 mL of sodium chloride and it still took 45 minutes to an hour to get him cleaned out. After waiting the full week, I emailed Dr. Levitt and let him know that we could see changes, but nowhere near to the extent that we had hoped. He forwarded my email to GI and they decided as a team to try something new. They recommended we start a low dose of a stimulate laxative called Senna once a day.

For those that know me, they know how crazy this makes me. I'm a very natural parent. Since having the boys, I stopped using household cleaners containing chemicals, starting buying all organic, steroid, hormone free foods, and the boys use special chemical-free bath products. Carson or Austin neither one take medications on a regular basis, and when they do become sick we don't "over medicate", we only use Ibuprofen if a fever is very high. So you can imagine what a change this has been having a child with a chronic medical condition requiring some form of medication his entire life. Medication has just became something I've had to learn to deal with. I do my best to avoid what I believe to be the more potentially harmful medications in favor of safer alternatives, but at the end of the day there is only so much I can do.

I had to push my worries aside and just deal with the Flagyl, as much as I didn't want to, because without it Landon ended up so sick on his birthday. Laxatives were another area I wasn't thrilled to get into, because it's great if they work, but then you are likely looking at long term use. I researched Senna, and found it to be safe enough that I had no objections. It is something available over the counter, and to my surprise (and delight!) it costs less than $5 for a 12 day supply. We are used to every trip to the pharmacy costing anywhere from $40-$200, so $5 is such a great surprise!

The particular form of Senna that we chose is Exlax chocolate squares. Landon takes one square (15 mg) at bedtime following his irrigation. After starting this, it was like a miracle. I could mistake Landon for one of our other boys when they were this age! His freqency decreased from 10-12 tiny BMs per day to 4-5 large ones. We have used the Senna for almost 2 weeks now and this is continuimg to improve. Yesterday, Landon only had 3 large bowel movements. He stayed clean throughout the morning and afternoon (which also helps his diaper rash issue) and then had his bowel movemnts over the course of the late afternoon and evening.

Since the Botox, we have been tapering off the irrigations and Flagyl. We dropped the morning irrigation and only do one at night, which takes less than 800 mL of solution because Landon is already nearly empty! We are down from 4 doses of Flagyl per day to only one, and next week we are attempting to go off of it altogether. This is the first time in a long time that I have been optimistic that this could work. Even if it doesn't,  we can at least go back to a small maintenance dose which is an improvement from where we've been.

Landon's development is also coming along. He is still struggling with the balance and strength to walk, but has made several other major acheivements. He is eating 100% table food, with only a few dietary restrictions. We introduced some dairy and small amounts of whole wheat, so he is no longer completely dairy or gluten free. We are still avoiding constipating foods, but letting Landon at least try everything he can and then trying to add more laxative foods to balance it out.

Another great accomplishment Landon has made is drinking from a sippy cup. He still takes a few bottles a day to stay hydrated, but he is taking in about half of what he needs through a cup. I'll end this post with a cute picture of what else he has figured out he can do with a sippy cup ;)

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.