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.

Results

As promised, I am finally getting a chance to get on here and update on what happened with our visit to Dr. Davis last week. We found out the results of two tests that we had sent off, a Genova stool study and a Genova nutrition evaluation. Both offered a lot of information- not necessarily good or bad news, just more information as to what is going on with Landon's body.

I'll start off with the results of the stool study. As expected, it showed severe dysbosis. Dysbosis is an overgrowth of bad bacteria and lack or absence of good bacteria within the digestive tract. Even while Landon was on the Flagyl, he still had a severe overgrowth of bad bacteria, and not enough good bacteria (flora) within his intestines to measure. All of that was the news that we were expecting, as Dr. Davis had already been treating Landon with a heavy dose of probiotics and many different herbal supplements for several months now. The news that we weren't necessarily expecting- although also not terribly surprising- was that the stool study also showed a severe overgrowth of several different types of fungus. So now we have added yet another herbal supplement to help eliminate the fungal growth, and if we don't see results within a couple of months she recommends that we do a round of oral Nystatin.

Now on the the nutrition evaluation... it was a train wreck! Landon showed deficiencies across the board of vitamins, minerals, and fats. And since these are things that we know for sure that he is consuming sufficient amounts in his diet, it means that he has malabsorption. The most relevant areas that came up were B vitamins and fats. Dr. Davis prescribed a very high dose of high quality B vitamins and increased his dose of cod liver oil.

Last but not least, was another surprise. We have been battling a strange rash all over Landon's back and stomach for a couple of months. When I brought him in to the doctor they diagnosed him with contact dermatitis and prescribed steroid ointment. The cream cleared it up, but after a few weeks it came back. Once again we applied the ointment and it cleared up, but it kept coming back. We couldn't figure out what was causing this. The usual instigators- laundry determent, fabric softeners, bath products- were out of the question because we already don't use products containing chemicals/dyes/perfumes. After we returned from Myrtle Beach he had the worst outbreak yet. I showed it to Dr. Davis and she was confident that it is eczema. She said that often it can be a reaction to wheat or dairy. The mystery was instantly solved. Although Landon doesn't consume wheat or dairy on a daily or even weekly basis, I am guilty of giving him small bites of these things occasionally. While we were in Myrtle Beach our whole family ate terribly, including Landon. Although I'm not sure yet if it is dairy or wheat that is triggering the reaction, now that we have completely eliminated both things again the eczema has cleared up. 

That sums up our visit with Dr. Davis. Otherwise, everything is still going well. We got our situation with our insurance company resolved so that we can affordibly get the Rifaximin. We actually only have to pay a $40 co-pay for a 10 day supply, which is the same that we were paying for the Flagyl. The only difference is that the Flagyl only costs $40, and so our insurance company was not having to pay a penny. Now, they are having to pay over $800 for their part of a 10 day supply, so $2400 a month. I expect that it will only be a matter of time before they try to deny coverage or force him to move to a different antibiotic although it is working so well. So hopefully next time we try to take him off antibiotics in August it will finally be successful.

As some already know, Landon is having his first birthday next Sunday! It is such a miracle that he is still here after all that he has been through and gets to celebrate something that so many people take for granted every year. He is really doing well with some things- like crawling, pulling up on furniture, waving hi/bye, understanding basic language, and finally babbling and starting to try to say words. Other areas are not so great. His low muscle tone is becoming more of an issue again. He can't stand on his own or cruise on furniture without his legs giving out on him. He is also having a lot of trouble with eating. He gags and chokes on everything that is not completely pureed. We will be bringing both of those issues up at his next appointment coming up, but I'd imagine we will be contacting Early Intervention again to re-evaluate and possibly start back up therapy. We will also be going for his one-year follow-up with neurology soon, but their office is so backed up with appointments that weren't able to get in until the middle of August.

I hope everyone is having as good of a summer as we are so far! Landon is such a little fish and loves to swim already. I've enjoyed the first month of staying home with the boys so much already and can't wait for the next two months!

Start of a Great Summer

Hi everyone. I apologize for the lack of updates, things have been chaotic around here! We just returned from our first vacation as a family of 5. Landon had so much fun seeing the ocean for the first time and crawling in the sand. We managed to bring all of his irrigation supplies, supplements, and medications and stayed on track with everything the whole week we were gone. I will write a longer update later this week to update on the new medication he started and what we find out at a follow-up visit with Dr. Davis on Wednesday. Meanwhile, I will post some cute pictures of Landon enjoying the beach.

Just an update

Hi everyone! First of all I would like to apologize for the lack of updates. Our oldest two boys have started gymnastics and one of them has also started t-ball and so things have been busy lately. Since my last update we finished the 3 month course of maintenance Flagyl and tried to take Landon off of it again. Unfortunately, it was not successful. I reluctantly called Cincinnati, knowing that they would want to put him back on another long course of Flagyl. I explained that although I understand that this is necessary in order to keep Landon alive, at the same time I'm uncomfortable with the damage that it is causing. Flagyl is known to cause neuropathy with long-term use and is also anticipated to be a known carcinogen. Landon has already been on it for 8 months total which is longer than I would have liked.
To my surprise, they actually had an alternative available. Dr. Levitt prescribed a new antibiotic called Rifaximin. Rifaximin is a broad-spectrum antibiotic that is not absorbed into the blood stream, meaning that it only affects the intestines- the location of the problem. It is a much safer drug for long-term use with no known adverse effects. So like myself, you are probably wondering why this drug isn't using more commonly as an alternative to the Flagyl. The answer: cost. I just spent over $800 for a 3 month supply of this medication. Just like any other situation we have encountered, our insurance does not want to cover it because like the Flagyl, it is compounded. Cincinnati is currently working to get them to authorize it so I can be partially reimbursed for the cost, but it is still up in the air. So unfortunately, this 3 month course could be the only course that we are able to afford if our insurance won't cover the cost.
We are still using all of our natural supplements and will be returning to St. Louis soon to find out the results of the stool study that was done. We will also possibly be returning to Cincinnati in another few months depending on the results of an abdominal x-ray that will be scheduled. Dr. Levitt has mentioned doing some more invasive testing to try to find the source of the issues that Landon is having, including a colonoscopy. I just wish that we could find an answer as to why he is having chronic infections to the extent that he is. Even Dr. Levitt, who sees the most complicated cases of Hirschsprung's disease in the world, is beginning to sound a little less confident.

Exciting News

Hello everyone! I'm happy to say I have all kinds of exciting news to share. First of all, we have a crawler! Three days after he turned 9 months old, Landon took off crawling. He loves to chase his brothers and to explore the house. He especially loves going in rooms that he is not supposed to be in (kitchen, bathroom...). Time to bring back out the baby gates!

Next is an update on the visit with Dr. Davis. She took a detailed health history and recommended that we do a nutrition urine analysis and Genova stool study. Both have been submitted, but we don't have results yet. We will be going back up for a follow-up visit at the end of May to discuss the results and if and how it will affect the treatment plan. To make a long story short, she agreed with me on the major points. She also felt that the flora in Landon's intestines is severely disrupted (which the stool study should confirm how and to what degree) and also that if we don't change the plan nothing will change trying to take him off of the Flagyl again in mid-May.

So we now have what I wanted- a new plan. Dr. Davis agreed to work with Dr. Levitt's recommendations from Cincinnati with continuing the maintanence Flagyl until the middle of May and then trying to wean off of it. She also put Landon on a lot of supplements.

 

Here is what all Landon is taking daily:

 

Digestive Enzymes- Gastro Calm 4 caps daily
Sacro B. 3 caps daily (over 9 billion CFUs)
Artic Cod Liver Oil 2 tsp. daily
HMF Replete Probiotics 1 packet daily (150 billion CFUs)
TruFiber 1 1/2 scoops daily
Gen Intrinsic 20 drops daily
Artemesia Int 40 drops daily
RegenRX Small Intestine 40 drops daily

 

The dosages are divided up so that he is taking 1/3 of the daily dose of all of the above in the morning, afternoon, and evening. I want to add a disclaimer that these are super high doses well above what most adults are recommended to take and that no one should give these doses to a child (or especially a baby!) without first consulting a doctor. But with that said, all of these supplements are working very well. Landon is staying cleaned out and it has drastically shortened the duration and amount of sodium chloride solution that we are using each irrigation. We are experimenting now with dropping the afternoon irrigation and only doing one in the morning and one in the evening. Keep your fingers crossed that this is successful because it would make life a lot easier, especially now that the boys have started gymnastics and Carson is starting t-ball.

 

A lot of people have asked how it's going with the donated milk. It is going really well! We have 5 ladies that have donated on a regular basis, and many others that have donated large quantities. Even those that have donated a few spare bags have helped tremendously. We have enough coming in that for over a month now Landon has not had to have formula. He eats pureed fruits and veggies and about 30 ounces of milk per day. If we stay on track with bringing in the milk, I'm really comfortable that we are going to make it until he is 12 months. If we could go beyond that it would be wonderful, but 12 months old is the goal. I'm not sure yet what he will drink once he transitions to something new. Almond milk or coconut milk seem to be the top contenders, but neither provide very many calories- which could be problematic with Landon's off and on struggle to gain weight.

My older boys are refusing to nap and terrorizing my mom's house as we speak, and so this is going to have to be all that I post for now. I hope everyone is able to get out and enjoy the weather now that it finally feels like spring!



Everything New

Some may remember that I had mentioned I had some exciting news about several things. Well, I figured I should jump on here and share it. The first subject, and most exciting, is Landon's development.

Two days shy of being 9 months old, Landon is now: clapping, following the command to "clap" when someone says "clap your hands" or "yay", waving bye-bye appropriately, getting up on hands and knees to crawl and rocking back and forth (but not quite crawling yet), scooting on his bottom to nearby objects, pulling up on objects and standing on his knees, imitating "clicking" sounds with his tongue, imitating "kissing" noises, feeding himself using a pincer grasp, and finally rolling over well enough in both directions that he changes positions in his crib overnight. He is also starting to experiment more with babbling. He was starting to babble in early Decemeber before his last surgery, but then it just stopped and hadn't picked back up. Lately, we're starting to hear him putting consonant and vowel sounds together again. Yay Landon!

As far as the other exciting news, we are traveling to the St. Louis area on Tuesday to see another new specialist named Dr. Davis. She specializes in using a hollistic approach, a combination of Eastern and Western medicine. In other words, she looks at the big picture and makes sure that the body is balanced and functioning how it should be, but also believes in using Western medicine when and to the extent necessary. She will be conducting a 2 hour interview on Landon's health and medical history and then doing some screening. We will also be doing a stool study in the near future. Several Hirschsprung's parents that I've met in support groups have had success using this type of treatment approach and have been able to turn their child's health around by keeping a balance in the intestines. I'm really excited to meet Dr. Davis and have high hopes that she will be able to do great things for Landon.

I'm hoping that the stool study is going to be the key to solving this complex puzzle, otherwise known as Landon's health. As most already know, Landon went in to cardiac arrest when he was 6 days old from overwhelming infection caused by (Hirschsprung's associated) enterocolitis. The reason that it caught everyone off guard, even when Hirschsprung's had been mentioned as a possibility as to why he was having the issues that he had, is that enterocolitis very, very rarely develops anywhere near as quickly as it did for Landon, and it is even more rare for it to become deadly in such a short period of time. So the question has always been- why Landon? Why did it develop and progress as quickly as it did for him? I've always had a hunch. I went in for an induction on June 28th. I was in labor for over 2 full days- the whole time I was receiving antibiotics every few hours. It is known that antibiotics kill all bacteria in their path- both good and bad. So basically, when you use antibiotics, you wipe out the flora (good bacteria) in your intestines. I believe that for those 2 days that I was in labor with IV antibiotics pumping through my system, Landon's flora was being completely eliminated. Then, once he turned into a birth position where he would no longer descend, Landon had to be delivered via caesarian- also known to disrupt/prevent establishing a balance of flora. And last, but not least, since Landon was too sick to eat from birth and I was never able to produce a single drop of milk from pumping (even with the help of 2 lactation consultants), even though Landon was technically "breastfed" in that he wasn't receiving formula, he actually did not eat anything for the first two weeks of his life. So there was another knock to the balance in his intestines.

Based on everything I've mentioned above, I believe that enterocolitis was practically welcomed into his intestines and had the perfect environment to thrive and quickly take over. In order to manage the enterocolitis, we have had to use the antibiotic Flagyl, to kill the bad bacteria that quickly takes over in Landon's intestines. The only problem with that, is that in the meantime, the Flagyl is also even further preventing Landon from developing flora in his intestines to balance and naturally fight off the chronic/recurrent infections. We already use a very strong, adult strength dose of probiotics to try to replinish the good bacteria in his intestines. Once we do the stool study, we will have specific information on what the levels of both good and bad bacteria are, what types, and what dosages of the specific strands that he needs in order to bring his intestines up to a "normal" point. Then, in May, once we attempt to take him off of the maintanence Flagyl once again, if he has an adequate amount of flora in his intestines, and if we are able to turn his intestines into a very unwelcoming environment for the bad bacteria to grow and take over, we could finally be on a path for a normal life for him without frequent rectal irrigations and antibiotics.

I could be completely wrong about everything, but I'm willing to risk the time and the money to possibly be closer to finding a solution. I will update next week with news about what Dr. Davis has to say. I hope everyone has a Happy Easter!

A Day in the Life

Hello everyone, I hope everything is going well for all of you. Things have been wonderful around here lately. Last week I was off work for Spring Break, and even with a few inconviences in our home (upper respiratory infections, pink eye) it went very well. I finally feel like our life is managable again, for the first time since Landon had his pull-thru surgery in December. Nothing has changed to make it easier, we are just finally getting settled into the routine for all the extra care that Landon requires.

So what exactly is the extra care that Landon requires? Well here is a breakdown:

4:45 AM: Alarm goes off. I get up and put a bottle in the bottle warmer, prepare his antibiotics, make sodium chloride solution for the irrigation, prepare the area, get all supplies ready

5:00 AM: John and Landon get up. John holds Landon while I do the rectal irrigation. It takes 30-45 minutes start to finish because we must continue until the solution runs clear.

5:45 AM: I give Landon his antibiotics and feed him his bottle. Sometimes he falls asleep and goes back to bed. Other times he plays in his bouncer for the rest of the morning.

6:15 AM: I get ready for work, very quickly! I shower the night before and let my hair air dry overnight because I don't have time for anything else. My routine for myself is very minimal. I also get Carson and Austin up and help them brush their teeth, get dressed, and take their vitamins.

7:00 AM: We leave the house. I drop Landon off at my mom's house first, and then Carson and Austin go to daycare.

8:00 AM: I get to work. Lately, Landon is in good enough health that I can focus on my job and the kids that I see. Sometimes Landon has to be brought to my work around 11:30 for an extra irrigation if things aren't going well for any reason.

2:00 PM: Landon takes his second dose of antibiotics for the day. My mom does this when I am at work.

3:15 PM: I get off work. I usually have at least one errand to run in Carbondale, the larger town that I work in. Sometimes I pick up produce from the natural foods store near the school where I work, sometimes I have to refill Landon's medicine, or sometimes I pick up milk from one of our wonderful Carbondale-Carterville-Herrin area milk donors. Next I pick up Carson and Austin from daycare.

4:15 PM: I arrive at my mom's house. Either my mom or my stepdad goes out to the car so I don't have to unload the boys. First I review what Landon has eaten for the day and how much. Then whoever stays inside helps me with our second rectal irrigation for the day. I'm spoiled because everything is usually already prepared and set up for me when I get there, saving around 15 minutes.

5:00 PM: I get home with the boys. John is usually gone to the gym for at least another hour, so I unload the boys and get some type of educational television show on for Carson and Austin to keep them out of trouble. Landon sits in his high chair and eats puffs while I prepare dinner.

6:00 PM: I mix Landon's two types of probiotics and his supplements he is taking in apple juice and give it to him. He also eats pureed food around this time.

6-7-ish PM: Somewhere in here Landon takes another bottle.

7:30 PM: Bath time for everyone! Yes, all 3 boys take a bath together in the tub. You would do this too if you saw how much easier it is.

8:00 PM: Carson and Austin get ready for bed and go to sleep. This marks the beginning of preparing to make it through the next day...

8:00-9:00 PM: Thaw donated milk (it is all kept frozen), wash and sterilize bottles, monitor amount of pureed food and prepare more if necessary

9:00 PM: Landon plays in his bouncer outside the bathroom doorway while I take a very quick shower. No time to even think about blow drying my hair, so I prepare it the best I can so that it dries halfway tame.

9:30 PM: Last rectal irrigation for the night. Once again, John holds Landon while I do the dirty work. Then John cleans up while I take Landon out to finish our day.

9:45-10:00 PM: Landon takes his HUGE bedtime bottle. Seriously, during the day he is unable to take more than 4 ounces at a time. But he eats 7-10 ounces for this last bottle of the day. Strange, I know.

10:15-11:00 PM: I hold Landon while he takes his pacifier and falls asleep anywhere within this time range. I usually watch television to stay occupied and whenever he falls asleep I put him in his crib and go to bed myself.

So there you have it. Our friends wonder why we are never able to go anywhere or do anything anymore- that's why. When we do venture out we are on a very strict schedule because a lot of these things just can't be done out in public places. Changing Landon is a great example. Most don't know this, but the stool that Landon passes is extremely acidic due to his intestintal issues. No matter how quickly we change him it always makes an acidic burn which is like diaper rash on steroids, and he has this all the time. The only way to keep this from happening is by completing a nearly 10 minute routine with several products that we have to order. As you can imagine, a public changing table is not the ideal spot for this routine to take place. The people that get in line behind us waiting for the changing table don't understand or appreciate why I am spending so much time changing Landon's diaper.

As you can imagine, weekends are much better than our weekday schedule that I described. For starters, no one in our house gets up until at least 6:30 on the weekends and we start our routine much later. We do have to plan our day around the schedule, but Carson and Austin take a nap during the afternoon anyway and so doing the afternoon irrigation and antibiotics that time just works out well since we are home at that time.

I am going to try to post some exciting updates later this week. Landon is doing some really awesome new things, and we have some exciting news about a new specialist that we are welcoming to the team for Landon's care that we will be traveling to see very soon. So keep checking back for an update hopefully sooner rather than later!

FAQ

Hello everyone! Everything is still going well with Landon's health. The maintenance Flagyl and frequent irrigations are keeping everything in check with his health and he has had a very good last few weeks developmentally. He is 8 months old now, and he is officially sitting up 100% independently, as in I can leave the room and go do something and when I return he is still sitting up. Not only that, but he has also learned to scoot in that position to get around. When he's on his stomach he is starting to push up onto his knees and elbows and rock, but he can only hold the position briefly before falling to the ground. He has shown interest in pulling up, but he still isn't strong enough to do it on his own. The best milestone he has hit so far, by far, is learning to clap. He understands the command "clap" or "yay" and starts clapping on his own. I'm concerned that he still isn't really making consonant sounds or starting to babble, but the fact that he is showing that he comprehends certain words is reassuring.

Since I don't have much to update, I thought it would be a good idea to address some of the questions we get frequently. Here are some of the questions we often get:

"What is the problem with Landon's heart?/ Does Landon have a problem with his heart?"

No, Landon does not have any problems with his heart. Although heart defects are the most common cause for going into cardiac arrest, his episode had nothing to do with his heart. Landon went into cardiac arrest from overwhelming infection which was the result of an infection known as enterocolitis related to Hirschsprung's disease. Once Landon was resuscitated, one of the first tests performed was an echocardiogram which showed that there are no problems with his heart.

"What exactly is Hirschsprung's disease?"

See my link above for a full description, but to summarize, it is the failure of ganglion cells- the nerve cells that allow the intestines to move waste material through- to migrate to any extent. The entire large and small intestines can both be affected, only the large, or only part of the large. Never the less, wherever the nerve cells are lacking stool cannot be passed through. This causes stool to back up in the intestines and can cause a variety of serious, life threatening complications. The most common complication is enterocolitis- an infection that only those with Hirschsprung's disease are able to get. Our surgeon, Dr. Levitt, has said that a typical person could go a week or more without passing stool, and although they would be very uncomfortable, they would be fine. A person with Hirschsprung's disease, however, is at risk for developing that infection if they aren't passing stool at least once a day. So it is crucial for patients with Hirschsprung's disease to be kept cleaned out, whether it be with laxatives or rectal irrigations, and then at some point the affected portion of the intestines must be removed.

"Does it affect Landon that he had 1/3 of his large intestine removed? Do you need your large intestine to live?"

This is a complicated question, but technically no, you do not need your large intestine to live. The role of the large intestine is to absorb water and certain nutrients from your stool. If you lose less than half, it generally does not make much of a difference in daily life, other than stool is expected to be looser and more acidic. Once you lose half or more, you run into problems with dehydration and certain measures must be taken. Your small intestine is a whole different ball game. Your small intestine absorbs nutrients. Once you start losing parts of your small intestine you risk short gut syndrome, which means that you are not able to take in adequate nutrition from food. IV nutrition known as TPN can be used to compensate for that problem,  but life expectancy and quality of life is affected. Fortunately, Landon still has 2/3 of his large intestine and all of his small intestine, so he really doesn't have it nearly as bad as it could be.

"What is the life expectancy for someone with Hirschsprung's disease?"

For someone only affected to the extent that Landon is, it is typical. Since Landon still has most of his intestines the thing most threatening to him is enterocolitis. He is at the greatest risk from enterocolitis for the next 2 years. The longest duration reported between corrective surgery for Hirschsprung's and a case of enterocolitis is 10 years. So basically, in 10 years he is pretty much out of the woods. He will always have to watch his diet and deal with several other nuisances from less than perfect intestines, but he should live a long life.

"...But doesn't Landon currently have enterocolitis?"

Yes. It is known that developing enterocolitis before having the corrective surgery for Hirschsprung's puts one at a much greater risk for developing it again. It is believed, although not proven that the severity plays a role. Before Landon was diagnosed with Hirschsprung's he developed entercolitis so severely that it caused his heart to stop. He barely survived- actually, it is a miracle and not able to be explained medically how he did survive after 35 minutes of cardiac arrest. Since then, he has never been able to shake it all the way. He is left with what is known as chronic/recurrent entercolitis. It is under control with a combination of frequent rectal irrigations and antibiotics, but it never goes away. As soon as we attempt to irrigate less frequently or stop the Flagyl he always develops symptoms within 4 days and has to resume treatment.

"When will Landon overcome enterocolitis?"

That's what I would love to know! We're attempting to stop the maintenance meds again in May, and we'll see how that goes. Any time we stop could be "the time" where he is finally able to stay healthy on his own. "The time" is most likely going to be sometime in the next 2 years. Like I mentioned above, the longest duration between the corrective surgery and an episode of enterocolitis is 10 years. So "the time" should definitely be in the next 10 years, excluding some record breaking misfortune. Right now we are mainly concerned with avoiding negative effects from long-term antibiotic use and the enterocolitis becoming resistant.

"Where does Landon receive treatment, you mention both St. Louis and Cincinnati?"

St. Louis is our nearest Children's hospital. That's where Landon was when he went into sudden cardiac arrest. Doctors on the 8 West floor of St. Louis Children's Hospital resuscitated Landon and saved his life. We stayed in St. Louis until Landon was 5 weeks old and he was finally released after his colostomy surgery. We still go to St. Louis for follow-ups with Neurology, and that would be our first choice in hospitals for just about anything. It is a wonderful hospital with great resources for families staying there long term. BUT...

St. Louis only sees around 10 cases of Hirschsprung's disease a year. They divide the cases among  surgeons, so each surgeon operates on only 2 cases a year at most. This is typical of most hospitals. Cincinnati Children's Hospital actually has a colorectal center with colorectal surgeons. All they do is operate on cases of Hirschsprung's disease and other colorectal malformations all day every day. Cincinnati is only a 5 hour drive, and so we decided to let them take over Landon's care for everything directly Hirschsprung's related. The top expert in the world on Hirschsprung's disease is Dr. Levitt, and we are fortunate enough to be able to call him Landon's surgeon. He did Landon's pull-thru surgery and determines Landon's plan of care. I thank God every day that we have the resources available to take Landon to the best of the best when it comes to this disease.

"Are Landon's developmental delays related to Hirschsprung's disease?"

No. This is probably the most common misconception surrounding Landon right now. Hirschsprung's disease is purely a disease affecting the intestines. Yes it can present as part of certain syndromes, but Hirschsprung's alone does not affect development. Landon has low muscle tone which is causing his delays. Is it a result of being critically ill at such a young age? Is it the result of spending around 2 months total in hospitals unable to get up and move around? Would he have had it even if none of those thing ever happened? Who knows. Honestly, I don't really care. Why spend time and energy worrying about "why?" Why doesn't change anything. I prefer to devote my energy towards "what do we do about it?" which is where we are right now. Landon sees a physical therapist and is doing very well. He is consistently staying about 2 months behind where we should be developmentally, but not falling further behind.


Hopefully that helps if anyone has had anything they are curious about. I am happy to answer any questions that anyone has or to cite my sources for any of the information I listed above. The reason that Landon ended up as sick as he did was due to lack of awareness. Hirschsprung's disease only affects 1 in 5,000, and so I understand that it's not going to be plastered all over billboards down the interstate, but no one should end up going through what Landon did. Something has to be done to raise awareness. So feel free to ask me anything, anytime, and I will be happy to answer :)

*EDIT*
I am going to add questions that I get. I just got my first :)

"Does Hirschsprung's run in your family or is Landon the first to get it?"

Landon is the first. The genetics of Hirschsprung's are not fully understood, but John Hopkins University is doing a lot of research right now on the topic. John and I, as well as all 3 of our boys have submitted DNA for their research. Anyway, there are several genes that are known to be involved, the most common being the RET gene. Since we are done having children there is no need to pursue genetic conseling or further testing to isolate the specific defect that Landon has, but if we were to have another child he/she would run a 12-14% chance of also having Hirschsprung's. Unfortunately, Landon has a 25% chance of passing it on to his children.

Quick Update

Not much to update, everything is going about the same.. but I do have some good news to share! Landon had his last check-up with his pediatrician at the end of January, and then a week and a half later went up to see his colorectal surgeon in the beginning of February- as some may remember. He had not gained weight between the appointments and so I was beginning to get concerned. On Tuesday, I took him to get the rest of his 6 month shots (he's still running behind from not being able to receive vacinations while he was so sick the first few months of life) and he has gained a whole pound in less than 3 weeks! Other than that, not much is new, so I thought I would add some new photos.

Maintenance

Well, the plan we made at our follow-up in Cincinnati was short-lived. As I mentioned before, we discontinued the Flagyl and dropped to 2 irrigations a day. By Monday, just 3 days later, Landon seemed off- excessively fussy and slightly poorer appetite- and so I began watching him closely. Then on Tuesday morning he refused to eat altogether and projective vomited at about 6 in the morning. Fortunately I kept what was left of his Flagyl and so I was able to get him back on it right away. I called Cincinnati and spoke with his nurse and she confirmed that I had done the right thing in starting him back on the Flagyl, and she recommended we do 4 irrigations a day until symptoms disappear and then go back up to 3 a day. She later called back and instructed us to fill a prescription for 25 mg of Flagyl per dose (his regular dose is 37.5 mg) and said that Dr. Levitt wanted us to continue the lower dose as a maintenance medication for the next 3 months.

In a way, I'm relieved that we don't have to worry about Landon getting sick again for a while. He will be 10.5 months old by the time we discontinue the maintenance meds. He will be much bigger and stronger, plus that will be more time that he has been on donated breast milk gaining antibodies which are making his immune system stronger. The downside is that I really hate him having to take Flagyl. Studies done on the safety of it have shown that it is carcinogenic in both mice and rats. It has been shown not to be carcinogenic in hamsters, which is good, but who knows which rodent reacted more closely to how a human baby would. Plus there are risks with long-term use of any antibiotic, such as developing c.difficile (c.diff) or creating resistant strains of bacteria. So hopefully he can successfully go off the Flagyl in May when we try again. I have done some more research and have decided to start Landon on grapefruit seed extract, to see if that will help. I also want to discuss trying sodium cromoglycate. There was a study done in Finland in 2001 on using sodium cromoglycate to aid with management of recurrent and chronic enterocolitis. Even though the sample size was small, only 5 participants, 3 of them responded favorably, and so it might be worth a try.

Last week we finished all 3 evaluations for Early Intervention. The physical therapist came first, which was the therapist I was the most interested in seeing since Landon's delays are mostly gross motor. She agreed that he has hypotonia, and classified it as being a moderate degree. When you pull him up from laying on his back he has what is known as "head lag" which means that he isn't strong enough to lift his head up with his body. His joints are also hypermobile, which means that they flex more than they should because he lacks the strength to form resistance. She showed me several exercises to do with him to help him make progress towards what he should be doing. She also explained that because of the hypotonia, when he attempts to make progress towards any gross motor milestone he is at a lower starting point than a typical baby. He has to work much harder at everything, which will result in greater fatigue. He also will need more time to meet his milestones, and so he probably won't crawl or walk on time. But she reassured us that when 5 year olds are playing on the playground they aren't going around comparing the age at which they walked, and so Landon will be fine long-term, he just may not be a star athlete.

The occupational therapist came next, and she was impressed with Landon's abilities. He is functioning pretty close to age appropriately with regard to his fine motor skills. The developmental therapist also thought that he looked good, with the exception of his gross motor skills. We are meeting on Thursday to discuss what the plan will be. Landon will either go ahead and start physical therapy, or the physical therapist will come back to re-evaluate him in 2 months. He was slightly below the criteria of a 30% delay, but the physical therapist was confident that in 2 months when there are more milestones to hit, that he will miss them and then meet the criteria. Either way we have exercises to do at home for the time-being, which is what I was hoping for. So here's to hoping for an uneventful few months full of lots of progress with Landon's gross motor skills!

Finding a balance

Thursday night we headed back to Cincinnati for Landon's follow-up with Dr. Levitt Friday morning. Just like last time, we went in and did an x-ray first, and then went up to discuss what it showed and what has changed since our last visit. The x-ray looked much better than his last. His small intestines are no longer dilated and there were no signs of enterocolitis. Dr. Levitt also did a rectal examination and Landon's sphincter is much better as well, which means that the dilations were successful.

We are continuing the dilations at least twice a day for maintenance, but also can do them as needed to help stimulate the area to move things along. We stopped the Flagyl all at once, but left with a presciption for half the dose just in case he doesn't tolerate the change. Dr. Levitt also set up a plan for tapering off the irrigations. He felt that the reason that Landon is not going on his own is because we are keeping him flushed out so often. The irrigations empty him out, and when we are doing it 3 times a day he isn't getting the chance to accumulate enough stool to feel the urge to push. The only problem with that is that Landon is still extremely prone to enterocolitis, and if things sit around for too long he gets an infection. So we have to find a balance between letting things accumulate enough that he learns to use those muscles and push, but not letting things sit around long enough to cause an infection. Dr. Levitt had us drop an irrigation, and so we are already down to two a day and will continue this for two weeks. At that point we will go down to once a day, and continue that for two weeks as well. If that goes well, then we will try to stop irrigating altogether. If at any point he starts showing signs of enterocolitis then we have to go back up to 3 irrigations a day, and then start the process of tapering off again. So we will see how that goes.

Landon is still doing well with starting solids. He has done so well that we have been able to introduce a new food every 3 or 4 days. So far all of the foods we have tried have been a success. Like I mentioned before, we only do foods from the Cincinnati "Laxative Diet" list. I make all of Landon's food which is a new adventure for me. Two of the most common culprits for causing constipation- bananas and apples- are in just about every baby food on the market. Every single combination food that I can find contains one or the other. Fruit and vegetable skins are also known to cause problems, and so when I make his food I'm able to strain everything and know that it does not contain skins. Those were the main reasons that I decided to make his food, so that he could eat a variety of foods and not be as limited by his diet restrictions. So far everything is going well with that, and I have a feeling it will get easier with time.

This afternoon Landon is having his first evaluation with Early Intervention. The physical therapist will be coming to our house to see what she thinks. On Thursday the developmental therapist will be coming, and we are still waiting on the occupational therapist to contact us, which I would imagine will be sometime this week. Landon has actually made a lot of progress over the past week with sitting up unsupported. He is sitting up without propping with his hands and holding it for 5-10 seconds, sometimes longer. He also does well sitting in his high chair at the table, but he isn't strong enough to eat in that position- he leans forward too much and food spills out of his mouth. I have to sit him in either his car seat or bouncer in order to feed him, which works at home, but it is yet another obstacle which keeps us from being able to leave the house often or for long periods of time.

Well, lunch is over for me. I will update soon on how the EI evaluations go. I hope everyone has a great week!

A little bit of everything

If you made it over here, then I see that you found that I moved Landon's Caring Bridge. Since we only have the internet on my phone, this makes it easier for me to update more often since Blogspot offers a mobile template. Also, I like the comment feature on the posts in case there is anything anyone would like to share.

Landon is doing okay lately, not really good or bad either one. He is still eating better, and probably gaining weight and so that part is good. We have introduced two different solids so far. Even though we will only be using foods selected from Cincinnati's 'Laxative Diet' list, I was still paranoid about food causing constipation. For that reason, we started with prunes. They may have had a little bit too much of a laxative effect, as they ran through him in less than 2 hours. I know that is good that he was able to pass them on his own, but I'm unsure if he was able to gain any nutrition or calories in that short of a transit period. Next was green beans, and last night was the first time that he tried them. He had an inconsolable period of crying from 9-10 last night, but that is something that happens from time to time anyway and so it is too soon to tell if it was from the food. I just fed him green beans again about 30 minutes ago and so by tonight I will have an answer.

Earlier this afternoon I met with the service coordinator from Early Intervention. We were assigned therapists for developmental, occupational, and physical therapy that will be contacting me to come seperately to evaluate Landon. If he exhibits a 30% or more delay in two areas then he will be eligible to receive therapy in our home for only $30 a month. If he doesn't qualify, then I will use his prescription for OT and PT and get him therapy privately, but that means more time off work to get him there and it will probably be much more expensive.

On Thursday night we are leaving to go back up to Cincinnati for another follow-up. They are doing a repeat abdominal x-ray and discussing what has happened since our last visit. We will be discontinuing the antibiotic that Landon has been taking for the last month, but I'm not sure if we will be stopping right away or doing it gradually. I also want to discuss why Landon is having so many issues with the affected portion of his intestines removed. It is normal to have some issues post-op, but not this many and to this extent. When he still had his ostomy he also struggled with enterocolitis, which is very rare. So I feel like there is something more going on. I have been doing a lot of research on chronic/recurrent enterocolitis and the outcomes. I found an old post from several years back from a parent that said that her daughter struggled with issues like that for 5 years before they discovered she had damaged connections from her small to large intestines. That got me thinking about an incidental finding in Landon's upper and lower GI studies done in July. We discovered that he had a mobile cecum. It isn't exactly rare, approximately 15% of us have a mobile cecum and have no idea. It isn't known to cause problems in the general population, but who knows if it causes problems when it exists along with Hirschsprung's. If anyone knows, it would be Dr. Levitt and so I plan on bringing it up. I'll try to update this weekend on what we find out in Cincinnati.

Big Day of Appointments

Today I'm home from work with a nasty stomach bug. Fortunately my mom is still watching Landon for me and my step-dad drove Carson and Austin to daycare and so I'm able to stay in solitary confinement to avoid passing this to everyone in the house. I've been out of sick days since December and so it is another unpaid day off, but at least it gives me a chance to update on what all happened yesterday in a timely manner.

First, Landon saw his pediatrician yesterday morning for his 7 month checkup. He is up to 20.5 lbs and 28.5 inches. Not surprisingly, with all of the issues we have had since his surgery he has dropped from the 90th percentile for weight down to the 70th. The good news is that his length has only dropped to the 88th percentile, down from 90th as well, and so he is still growing well despite the setbacks.

Next, we had to address Landon's development. I knew that he was starting to fall behind as he had recently missed all of his 6 month milestones. Already knowing that didn't make it much easier to hear though. When the nurse comes in she evaluates Landon using the scale for typical babies. Preemies have an adjusted scale, as do children with certain conditions such as Down syndrome, but there is no adjusted scale for babies that survived 35 minutes of cardiac arrest and have undergone many hours of general anesthesia and two major surgeries. Landon is still expected to fall on the "typically" developing scale. I had to answer "no" to all of the questions regarding his development.

Having had two other children go through well-baby checks, I can already tell that our pediatrician treats Landon differently. In addition to just looking him over, checking ears, throat, genitals, etc. he also does a much more thorough physical examination. He checks all of Landon's reflexes, puts him into different positions to check his strength, and does several tasks to see firsthand how he is doing. He was concerned with Landon's tone. He said that he is very "floppy" and said that he is going to have to add "developmental delay" and "hypotonia" to Landon's chart. Developmental delay is self explanatory, and I was expecting that, but the hypotonia caught me by surprise. Having worked with the populations that I have, the first thing that came to mind when I heard "hypotonia" is cerebral palsy. It means low muscle tone, and can be synonymous for cerebral palsy. Fortunately we were already heading to St. Louis for our afternoon appointment with neurology, which couldn't have been better timing.

After the unsettling checkup in the morning, the neurology appointment was very reassuring. The neurologist was much less concerned than our pediatrician. She said that he only has mild hypotonia in his extremities, but it is more severe in his trunk. She was encouraged by how alert and social he is. Landon successfully completed several tests with blocks. He struggled with passing them from hand to hand and lifting his arms above his shoulders. He also struggled with sitting up on his own, but for short periods of time he can balance hunched over and he is able to reach and pick up objects while doing so.

Overall, she said that Landon is functioning at a 5 month old level. She wasn't very concerned with his tone, she said that after what he has been through it would be much more concerning if he was hypertonic. She felt that it is likely a combination of everything- long hospital stays, 8+ hours of general anesthesia at a young age, being critically ill when he was a week old- that are causing his delays. Everything she said made sense. If most of his issues are in his trunk, that explains why he was keeping up with his milestones until recently. Sitting up was the first milestone that he missed, and so that goes right along with what she said.

So the next step- what do we do about it. A 2 month delay isn't exactly devastating, especially considering what Landon has been through. After all, this is the child that another member of the neurology team once called "Superman" because he could move his extremities and had his newborn reflexes back- even before they saw a perfect MRI. Just like our other boys, all I want for Landon is to be the best that he can be. So we are going to start physical and occupational therapy. I have contacted early intervention and we are meeting on Tuesday to go over financial and insurance information, and then soon after Landon will be evaluated. If he exhibits a 30% or more delay, he qualifies for services and they will come to our home to work with him. If he doesn't qualify, I have a prescription for OT and PT which I can use to get him therapy privately. The only reason that would not be ideal is due to the fact that we live in the middle of nowhere. The closest place that sees babies is 40 minutes away, and so that would be complicated with my work schedule. But we will do what we have to do. In July we will return to see his neurologist and re-evaluate how he is doing.

Last but not least, is the best part of going back to St. Louis. We were able to see the doctors/nurses/physician assistants/etc. that saved Landon's life. I had written and told them we were coming, with every intention of getting there at least half an hour early to go up and visit. But at the last minute John decided to go in to work, since his sick time is not exactly plentiful right now either, and so we were really rushed to get there by 4:30. We signed in in the waiting room at 4:30 exactly, and before I even sat down one of the doctors taking care of him the night that he coded on the floor was in the waiting room looking for us! I am thoroughly convinced that one of the main reasons that Landon survived was because he had so many people taking care of him that truly cared for him. CPR is very rarely successful after as long of a duration as Landon received it, but they didn't give up. And because they made the split second decision not to give up- even knowing how poor the prognosis surely was- Landon is still here having a wonderful life and making us so proud. Even though I send pictures every few months, it was great to be able to visit. Landon smiled and laughed at everyone. We went up to the 8th floor and saw many people that had been there "that night"- as it is referred to. Although we come to St. Louis often to take the boys for fun days, it is always on the weekend when many of them are off work. The best part of continuing to go in for neurology appointments during the week will be to visit! Since we will be going back in July after Landon's first birthday, we are going to work so hard so that maybe he will be able to walk in and actually say "hi." If early intervention asks what our goal for Landon is- that is it. For him to be able to walk in and greet everyone that saved his life, and to eventually be able to tell them "thank you."

Couldn't wait to share!

I know I usually go weeks without updates, but I couldn't wait to share. Since starting the donor milk at 5:00 am yesterday morning Landon started stooling on his own. My mom texted me a picture of a poopy diaper while I was at work, I'm sure my co-workers think I'm about the strangest person to ever live, but it was such a wonderful sight to see! His belly is the most flat it has ever been in his life. Even when I've thought that it looked good in the past he must have been distended becasue it has never looked this good.

Landon is so much happier and more alert. Struggling to digest his food must have been taking an even bigger toll than I realized. He hadn't rolled over since early December and he rolled 4 times last night. He also did a better job of sitting unsupported- although he is already 6.5 months old he is not yet sitting up on his own. When we did the irrigations yesterday there was hardly any stool that came out at all, he had passed it throughout the day. I know this is gross, but just to paint you a picture, on formula- picture cheese sauce mixed with cottage cheese. On breastmilk- picture water with yellow food coloring with a few tiny flecks.

This is a short update, but I had to share. Once again I'm on my lunch at work and I'm out of time. I hope everyone has a great rest of the week.

Repeating the same experiment over and over and expecting different results is the definition of insanity

Things haven't been great lately. In my last post when I said that the vomitting had resolved, I was wrong. He continued to vomit every 2 or 3 days. His appetite also went way down. Immediately following his surgery he had a huge appetite and ate 36+ ounces of formula a day for about a week. I was excited because he gained back all of the weight he had lost, but since then he has only been eating around 15-20 ounces a day. He was gradually losing weight instead of gaining and about a week before our follow-up appointment he started being unable to pass stool or gas on his own. We were irrigating him 4x per day and that wasn't even enough.

On the 11th we went back up to Cincinnati for his follow-up with Dr. Levitt. One look at his abdominal x-ray and he was sure that Landon had enterocolitis. This was very upsetting because we hadn't had an episode since we started going to Cincinnati when he was 2 months old. So now we're doing a month of antibiotics and continuing the frequent irrigations. On top of all that, Landon was also really tight where they reattached his remaining large intestine to his rectum and so we have to dilate. The smallest rod is the size of a sharpie marker, the largest one is the size of a magic marker. We have to insert them for one minute twice per day. He hates it and screams in pain. It takes one person to hold him still and another person has to do it. It's horrible, but it's what we have to do so there aren't any other options.

It is really bothering me that he has enterocolitis again. Since his first episode where we nearly lost him we have always been able to keep it under control when he gets it, but he had gone so long without getting it I thought maybe we were done. And we had been irrigating him several times a day which should have prevented it, so now I'm worried that we no longer are able to keep it under control. We go back for another follow-up on February 8th. He will still be on antibiotics so we expect to find out that the infection is gone, but hopefully it doesn't return.

Either way, once again I feel like we have lost a lot of control. We did all we could and we weren't able to manage it. It is definitely time for a change. I ditched the Culturelle and started Landon on a much stronger probiotic, VSL 3. As far as the poor appetite and weight loss go, there wasn't a lot that we could do. What makes babies gain weight? Fat and increased calories.. which are also constipating. You can't get a child constipated that is already battling dangerous intestinal infections. The stool gives the bacteria even more places to grow and take over. So it's risky to attempt to change formulas, and it might not even help. All I could do is beat myself up over not being able to breastfeed. While we were still in Carbondale I had two lactation consultants helping me with no success, they saw me pump for 30 minutes with nothing to show. The last thing I was told before we left was that due to my blood loss during the c-section (they had to give me 2 units) that my body just might be working on other things. On the 6th day of Landon's life when he went into cardiac arrest in my arms for such a long duration I quit pumping. Who knows what would have happened if I would have kept at it. Once he recovered, he did well on formula with his colostomy and so it wasn't incredibly urgent situation. I knew that breast milk would help him and so I tried the Yaz-Doperidone protocol to re-lactate for 2 months with no success. Once again, I was disappointed, but not a life or death situation because he did well on formula with the colostomy.

Unfortunately, since the pull-thru, his tummy just can't handle it. What options did I have, spend hundreds of dollars on the meds to try the protocol again which would probably have no success, or spend $5 an ounce to buy milk from the milk bank, knowing I could only afford to feed him about 4 ounces a day- and that is with cutting back on some major expenses. After battling with my pride I finally reached out and asked for help on my Facebook page. The response was overwhelming.. it has brought me to tears on several occasions. Enough wonderful ladies have already donated enough to give Landon 16 ounces a day for the next month. Many have commited to pumping the best they can to try to keep giving me more. If I can get 4 people with 4 ounces a day to spare I can continue giving Landon at least half breastmilk. Production can be inconsistent and you never know what is going to happen in life, but I think I've found enough. God bless all of those wonderful people. I know this is going to make such a big different for Landon. This could very well be the key to helping Landon become strong enough to finally kick enterocolitis for good. At the very least, it will be easier to digest and will give his poor intestines a break. It is amazing what people are willing to do to help others. I've given many of our donors a brief description of what happened with Landon and what issues he faces, but they really have no idea how great it is what they are doing for him. For our whole family! It has been incredibly stressful watching him get sick over and over and wondering if there was something I could have done differently to make this stop happening. This could very well be it. Thank you so much if any of you are reading this. There are no words to express my gratitude.
I'm at work right now and my lunch is over, but I will update in a few days. This is day 4 with no vomit, so keep your fingers crossed! I hope things will finally get better. As always, please keep Landon in your prayers.